Relative incidence of interstitial lung diseases in Brazil / Incidência relativa de doenças pulmonares intersticiais no Brasil

ABSTRACT Objective: To assess the relative frequency of incident cases of interstitial lung diseases (ILDs) in Brazil. Methods: This was a retrospective survey of new cases of ILD in six referral centers between January of 2013 and January of 2020. The diagnosis of ILD followed the criteria suggested by international bodies or was made through multidisciplinary discussion (MDD). The condition was characterized as unclassifiable ILD when there was no specific final diagnosis following MDD or when there was disagreement between clinical, radiological, or histological data. Results: The sample comprised 1,406 patients (mean age = 61 ± 14 years), and 764 (54%) were female. Of the 747 cases exposed to hypersensitivity pneumonitis (HP)-related antigens, 327 (44%) had a final diagnosis of HP. A family history of ILD was reported in 8% of cases. HRCT findings were indicative of fibrosis in 74% of cases, including honeycombing, in 21%. Relevant autoantibodies were detected in 33% of cases. Transbronchial biopsy was performed in 23% of patients, and surgical lung biopsy, in 17%. The final diagnoses were: connective tissue disease-associated ILD (in 27%), HP (in 23%), idiopathic pulmonary fibrosis (in 14%), unclassifiable ILD (in 10%), and sarcoidosis (in 6%). Diagnoses varied significantly among centers (c2 = 312.4; p < 0.001). Conclusions: Our findings show that connective tissue disease-associated ILD is the most common ILD in Brazil, followed by HP. These results highlight the need for close collaboration between pulmonologists and rheumatologists, the importance of detailed questioning of patients in regard with potential exposure to antigens, and the need for public health campaigns to stress the importance of avoiding such exposure.

RESUMO Objetivo: Avaliar a frequência relativa de casos incidentes de doenças pulmonares intersticiais (DPI) no Brasil. Métodos: Levantamento retrospectivo de casos novos de DPI em seis centros de referência entre janeiro de 2013 e janeiro de 2020. O diagnóstico de DPI seguiu os critérios sugeridos por órgãos internacionais ou foi feito por meio de discussão multidisciplinar (DMD). A condição foi caracterizada como DPI não classificável quando não houve um diagnóstico final específico após a DMD ou houve discordância entre dados clínicos, radiológicos ou histológicos. Resultados: A amostra foi composta por 1.406 pacientes (média de idade = 61 ± 14 anos), sendo 764 (54%) do sexo feminino. Dos 747 casos expostos a antígenos para pneumonite de hipersensibilidade (PH), 327 (44%) tiveram diagnóstico final de PH. Houve relato de história familiar de DPI em 8% dos casos. Os achados de TCAR foram indicativos de fibrose em 74% dos casos, incluindo faveolamento, em 21%. Autoanticorpos relevantes foram detectados em 33% dos casos. Biópsia transbrônquica foi realizada em 23% dos pacientes, e biópsia pulmonar cirúrgica, em 17%. Os diagnósticos finais foram: DPI associada à doença do tecido conjuntivo (em 27%), PH (em 23%), fibrose pulmonar idiopática (em 14%), DPI não classificável (em 10%) e sarcoidose (em 6%). Os diagnósticos variaram significativamente entre os centros (c2 = 312,4; p < 0,001). Conclusões: Nossos achados mostram que DPI associada à doença do tecido conjuntivo é a DPI mais comum no Brasil, seguida pela PH. Esses resultados destacam a necessidade de uma estreita colaboração entre pneumologistas e reumatologistas, a importância de fazer perguntas detalhadas aos pacientes a respeito da potencial exposição a antígenos e a necessidade de campanhas de saúde pública destinadas a enfatizar a importância de evitar essa exposição.

Connective tissue disease-associated interstitial lung disease / Doença pulmonar intersticial associada a doença do tecido conjuntivo

ABSTRACT Connective tissue disease-associated interstitial lung disease (CTD-ILD) represents a group of systemic autoimmune disorders characterized by immune-mediated organ dysfunction. Systemic sclerosis, rheumatoid arthritis, idiopathic inflammatory myositis, and Sjögren's syndrome are the most common CTDs that present with pulmonary involvement, as well as with interstitial pneumonia with autoimmune features. The frequency of CTD-ILD varies according to the type of CTD, but the overall incidence is 15%, causing an important impact on morbidity and mortality. The decision of which CTD patient should be investigated for ILD is unclear for many CTDs. Besides that, the clinical spectrum can range from asymptomatic findings on imaging to respiratory failure and death. A significant proportion of patients will present with a more severe and progressive disease, and, for those, immunosuppression with corticosteroids and cytotoxic medications are the mainstay of pharmacological treatment. In this review, we summarized the approach to diagnosis and treatment of CTD-ILD, highlighting recent advances in therapeutics for the various forms of CTD.

RESUMO Doença pulmonar intersticial associada à doença do tecido conjuntivo (DPI-DTC) representa um grupo de distúrbios autoimunes sistêmicos caracterizados por disfunção de órgãos imunomediada. Esclerose sistêmica, artrite reumatoide, miosite inflamatória idiopática e síndrome de Sjögren são as DTC mais comuns que apresentam acometimento pulmonar, bem como pneumonia intersticial com achados autoimunes. A frequência de DPI-DTC varia de acordo com o tipo de DTC, mas a incidência total é de 15%, causando um impacto importante na morbidade e mortalidade. A decisão sobre qual paciente com DTC deve ser investigado para DPI não é clara para muitas DTC. Além disso, o espectro clínico pode variar desde achados assintomáticos em exames de imagem até insuficiência respiratória e morte. Parte significativa dos pacientes apresentará doença mais grave e progressiva, e, para esses pacientes, imunossupressão com corticosteroides e medicamentos citotóxicos são a base do tratamento farmacológico. Nesta revisão, resumimos a abordagem do diagnóstico e tratamento de DPI-DTC, destacando os recentes avanços na terapêutica para as diversas formas de DTC.

Ecografía pulmonar en la evaluación del paciente crítico / Lung ultrasound in the evaluation of critically ill patients

Introducción: La ecografía es una técnica de imagen no invasiva que permite explorar diferentes órganos de manera inmediata, constituye un instrumento de alto valor diagnóstico al alcance del profesional de la salud, y es utilizada en todas las especialidades médicas. En los últimos años, la evolución tecnológica ha permitido que los aparatos de ecografía sean más pequeños, portátiles, y con una alta resolución, tal es el caso de la ecografía clínica o ecografía a pie de cama. La ecografía del paciente crítico ha cambiado la práctica médica; específicamente la ecografía pulmonar se debe realizar en todos los pacientes con enfermedad pulmonar aguda. Objetivo: Aportar el conocimiento teórico necesario para promover el uso de la ecografía pulmonar en la evaluación del paciente crítico, y contribuir, mediante su aplicación, a la disminución del riesgo de exposición a las radiografías. Métodos: Se efectuó una revisión de la literatura médica actualizada sobre el papel de la ecografía pulmonar en la evaluación del paciente crítico, en el período de julio a diciembre de 2021. Se utilizaron los siguientes motores de búsqueda: SciELO, Medigraphic y Google Académico. Conclusiones: En el contexto actual, la ecografía pulmonar ha adquirido un papel protagónico, pues su uso facilita una evaluación frecuente y no invasiva del paciente crítico con afección pleuropulmonar. Su aplicación garantiza la disminución del riesgo de exposición a las radiografías.

Introduction: ultrasound is a non-invasive imaging technique that allows us to explore different organs immediately; it constitutes an instrument of high diagnostic value within the reach of health professionals and used in all medical specialties. In recent years, technological evolution has allowed ultrasound devices to be smaller, portable and with high resolution, such is the case of clinical ultrasound or bedside ultrasound. Bedside ultrasound in critically ill patients has changed medical practice; specifically, lung ultrasound should be performed in all patients with acute lung disease. Objective: to provide the necessary theoretical knowledge in order to promote the use of lung ultrasound in the evaluation of critically ill patients, as well as to contribute, through its application, to reduce the risk of exposure to radiographs. Methods: a review of the updated medical literature on the role of lung ultrasound in the evaluation of the critically ill patients was performed from July to December 2021. SciELO, Medigraphic and Google Scholar were the search engines used. Conclusions: lung ultrasound has acquired a leading role in the current context, since its use facilitates a non-invasive and common evaluation of the criticall ill patients with pleuropulmonary disease. Its application guarantees the reduction of the risk of exposure to X-rays.

Sleep parameters in patients with chronic hypersensitivity pneumonitis: a case-control study / Parâmetros do sono em pacientes com pneumonite de hipersensibilidade crônica: estudo de caso-controle

ABSTRACT Objective: To compare patients with chronic hypersensitivity pneumonitis (cHP) and controls with normal spirometry in terms of their sleep characteristics, as well as to establish the prevalence of obstructive sleep apnea (OSA) and nocturnal hypoxemia. Secondary objectives were to identify factors associated with OSA and nocturnal hypoxemia; to correlate nocturnal hypoxemia with the apnea-hypopnea index (AHI) and lung function, as well as with resting SpO2, awake SpO2, and SpO2 during exercise; and to evaluate the discriminatory power of sleep questionnaires to predict OSA. Methods: A total of 40 patients with cHP (cases) were matched for sex, age, and BMI with 80 controls, the ratio of controls to cases therefore being = 2:1. The STOP-Bang questionnaire, the Epworth Sleepiness Scale (ESS), the Pittsburgh Sleep Quality Index, the Berlin questionnaire and the Neck circumference, obesity, Snoring, Age, and Sex (NoSAS) score were applied to all cases, and both groups underwent full-night polysomnography. Results: The patients with cHP had longer sleep latency, lower sleep efficiency, a lower AHI, a lower respiratory disturbance index, fewer central apneas, fewer mixed apneas, and fewer hypopneas than did the controls. The patients with cHP had significantly lower nocturnal SpO2 values, the percentage of total sleep time spent below an SpO2 of 90% being higher than in controls (median = 4.2; IQR, 0.4-32.1 vs. median = 1.0; IQR, 0.1-5.8; p = 0.01). There were no significant differences between cases with and without OSA regarding the STOP-Bang questionnaire, NoSAS, and ESS scores. Conclusions: The prevalence of OSA in cHP patients (cases) was high, although not higher than that in controls with normal spirometry. In addition, cases had more hypoxemia during sleep than did controls. Our results suggest that sleep questionnaires do not have sufficient discriminatory power to identify OSA in cHP patients.

RESUMO Objetivo: Comparar pacientes com pneumonite de hipersensibilidade crônica (PHc) e controles com espirometria normal quanto às características do sono, bem como estabelecer a prevalência de apneia obstrutiva do sono (AOS) e hipoxemia noturna. Os objetivos secundários foram identificar fatores associados à AOS e hipoxemia noturna; correlacionar a hipoxemia noturna com o índice de apneias e hipopneias (IAH), função pulmonar, SpO2 em repouso, SpO2 em vigília e SpO2 durante o exercício; e avaliar o poder discriminatório de questionários do sono para predizer AOS. Métodos: Um total de 40 pacientes com PHc (casos) foram emparelhados por sexo, idade e IMC com 80 controles (2:1). O questionário STOP-Bang, a Escala de Sonolência de Epworth (ESE), o Índice de Qualidade do Sono de Pittsburgh, o questionário de Berlim e o escore Neck circumference, obesity, Snoring, Age, and Sex (NoSAS, circunferência do pescoço, obesidade, ronco, idade e sexo) foram aplicados a todos os casos, e ambos os grupos foram submetidos a polissonografia de noite inteira. Resultados: Os pacientes com PHc apresentaram maior latência do sono, menor eficiência do sono, menor IAH, menor índice de distúrbio respiratório, menos apneias centrais, menos apneias mistas e menos hipopneias do que os controles. Os pacientes com PHc apresentaram SpO2 noturna significativamente menor; a porcentagem do tempo total de sono com SpO2 < 90% foi maior que nos controles (mediana = 4,2; IIQ: 0,4-32,1 vs. mediana = 1,0; IIQ: 0,1-5,8; p = 0,01). Não houve diferenças significativas entre os casos com e sem AOS quanto à pontuação no questionário STOP-Bang, no NoSAS e na ESE. Conclusões: A prevalência de AOS em pacientes com PHc (casos) foi alta, embora não tenha sido maior que a observada em controles com espirometria normal. Além disso, os casos apresentaram mais hipoxemia durante o sono do que os controles. Nossos resultados sugerem que os questionários do sono não têm poder discriminatório suficiente para identificar AOS em pacientes com PHc.

Risk factors for the prognosis of rheumatoid arthritis-associated interstitial lung disease: a multicenter retrospective clinical study / 中华风湿病学杂志

Objective:To explore the impact of clinical features, serological indicators, and pulmonary function test (PFT) on the prognosis of rheumatoid arthritis-associated interstitial lung disease (RA-ILD).Methods:Clinical data of RA-ILD patients who were diagnosed by HRCT and were followed up in Changhai Hospital or Yancheng First People's Hospital from 2011 to 2021 were collected Respiratory functional impairment of the patients was evaluated according to the changes of HRCT score and PFT, and the patients were divided into progressive group and stable group. COX survival analysis and ROC curve were used to determine the factors related to the progression of RA-ILD.Results:Finally 98 RA-ILD patients were included. The mean age of ILD onset was (62.9±12.1) years old, the median course of RA was 7.0 (1.0, 15.3)years, and the median follow-up time was 36.5 months (14.0, 79.5). There were 49 cases in the progressive group, and the clinical characteristics and laboratory tests of the two groups were compared. The results showed that: progressive time [(23(8.5,43.0)months vs 63(32.5,90.9) months, Z=-4.55, P=0.001)], HRCT score [(115(109,135) vs 111(105,116), Z=-2.70, P=0.007)], forced vital capacity(FVC) predicted [(70.1±15.7)% vs (80.8±19.7)%, t=2.12, P=0.039)], diffusing capacity of the lungs for CO(DLCO) predicted [(57.5±16.3)% vs (83.4±18.8)%, t=4.87, P=0.001)], male [(44.9% vs 18.4%, χ2=7.97, P=0.005)], UIP pattern [(36(73.5%) vs 9(18.4%), χ2=29.96, P<0.001)], RF>200 U/ml[(21(65.6%) vs 18(41.9%), χ2=4.15, P=0.042)], anti-CCP>75 U/ml [(42(91.3%) vs 35(71.4%), χ2=6.10, P=0.013], all had significantly different between the two groups. In multivariate analyses, UIP[ HR(95% CI)=3.25(1.62,6.50), P<0.001], anti-CCP antibody >75 U/ ml[ HR(95% CI)=3.85 (1.20,12.33), P=0.023] and smoking [ HR (95% CI): 5.74(1.10, 30.13), P=0.039] were significantly correlated with the progression of pulmonary fibrosis in RA-ILD patients. PFT was performed in only 44 patients with RA-ILD. The univariate analyses and ROC curve suggested that DLCO predicted [ HR (95% CI)=1.04 (1.02,1.06), P<0.001] was a significant risk factor for the progression of RA-ILD, and the area under curve (AUC) of DLCO was 0.845 [95% CI=(0.729,0.961)]. Conclusion:UIP pattern, high titer of anti-CCP antibody, smoking, and reduced DLCO predicted % may be potential predictors for poor prognosis of RA-ILD patients.

Study on the correlation of cyfra21-1 and modified ultrasound in connective tissue disease associated with interstitial lung disease / 中华风湿病学杂志

Objective:To investigate the correlation between cytokeratin 19 fragment(CYFRA21-1), modified ultrasound B-line and connective tissue disease associated with interstitial lung disease (CTD-ILD).Methods:The data of 112 patients with CTD hospitalized in the Department of Rheumatology and Immunology of the Second Hospital of Fujian Medical University from September 2019 to December 2021 were retrospectively collected. Sixty patients in the CTD-ILD group and 52 patients in the connective tissue disease without interstitial lung disease (CTD-noILD) group were included. The t-test and χ2 test were used to compare the demographic characteristics and tumor-associated antigens of the two groups of patients. Modified ultrasound score and HRCT Warrick score were evaluated by Pearson correlation analysis. In addition, the relationship between CYFRA21-1, modified ultrasound score and Warrick score were evaluated, and the diagnostic efficacy of CYFRA21-1 and modified ultrasound of CTD-ILD was evaluated and analyzed by binary logistic regression analysis. Results:Patients in the CTD-ILD group had higher CYFRA21-1 concentrations than the CTD-no-ILD group[5.74(4.25, 9.79) ng/ml vs. 2.79(2.21, 3.23) ng/ml, Z=45.94, P<0.001], patients in the CTD-ILD group had higher modified ultrasound scores than the CTD-no-ILD group [44.5(36.5, 60.0) vs. 5.0 (3.2, 6.8), P<0.001]. Modified ultrasound score was positively correlated with Warrick score ( r=0.93, P<0.001) and CYRFA21-1 was positively correlated with modified ultrasound score ( r=0.39, P=0.042). The sensitivity of CYFRA21-1 in determining CTD-ILD was 81.7% and the specificity was 92.3% [ AUC (95% CI)=0.88(0.81, 0.95), P<0.001], the sensitivity of modified ultrasound B-line to determine CTD-ILD was 96.4% and the specificity was 92.9% [ AUC (95% CI)=0.99 (0.97, 1.00), P<0.001]. History of smoking[ OR(95% CI)=9.26(1.11, 77.12), P=0.040] and elevated CYFRA21-1 concentration[ OR(95% CI)=19.40(4.89, 76.95), P<0.001] were risk factors for CTD-ILD. Conclusion:CYFRA21-1 is expected to be a serum marker indicating concomitant ILD in patients with CTD. Modified ultrasound B-line to determine concomitant ILD in CTD patients has good diagnostic utility and can reflect the severity of pulmonary fibrosis in CTD-ILD patients.

Risk factors analysis and prediction model establishment of anti-MDA5 antibody-positive dermatomyositis with rapid progression of interstitial lung disease / 中国医师杂志

Objective:To analyze the Risk factors for rapid progression of inpatients with anti-melanoma differentiation associated gene5 (MDA5) antibody-positive dermamyositis (DM) complicated with interstitial lung disease (ILD), and construct a clinical predictive model.Methods:A total of 63 hospitalized patients with anti MDA5 positive DM combined with ILD (MDA5+ DM-ILD) from January 1, 2016 to May 30, 2022 at the Second Affiliated Hospital of the Air Force Military Medical University were included in the study. They were divided into a control group (DM-ILD) and an observation group (DM-RPPILD) based on whether they had rapidly progressing interstitial lung disease (RPILD). Retrospective collection and organization of clinical case data from patients were conducted, and binary logistic regression was used to summarize the risk factors of DM-RPILD. R software was used to construct a clinical prediction model for RPILD occurrence using training set data, and validation set data was used to verify the predictive ability of the model.Results:The proportion of patients with SpO 2<90% at the initial diagnosis of ILD, the titers of anti MDA5 antibodies, immunoglobulin M (IgM), serum ferritin (FER) levels, and positive rates of anti Ro52 antibodies in the observation group were higher than those in the control group, the lymphocyte (LYM) count level was lower than that of the control group (all P<0.05). Binary logistic regression analysis showed SpO 2<90% at the initial diagnosis of ILD, FER level, LYM count, and anti Ro52 antibody were the influencing factors for the occurrence of RPILD (all P<0.05). The area under the curve (AUC) of the training set prediction model for predicting resistance to MDA5+ DM-RPILD was 0.922(95% CI: 0.887-0.957), with a sensitivity of 95.7% and a specificity of 72.5%; In the validation set, the prediction model predicted an AUC of 0.939(95% CI: 0.904-0.974) for resistance to MDA5+ DM-RPILD, with a sensitivity of 90.0% and a specificity of 88.9%; The calibration curves of the training and validation sets indicated that the predictive model had good calibration ability. Conclusions:SpO 2<90% at the initial diagnosis of ILD, FER levels increase, LYM count levels decrease, and anti Ro52 antibody positivity are risk factors for RPILD. The constructed clinical model has good predictive ability and has certain guiding significance for clinical work.

Taquipnea persistente como manifestación inicial de hiperplasia de células neuroendocrinas: reporte de un caso clínico / Persistent tachypnea as initial manifestation of hyperplasia of neuroendocrine cells: case report

Se realiza revisión de la literatura y presentación de un caso clínico de Hiperplasia de Células Neuroendocrinas en paciente lactante masculino que inicia su padecimiento a los 3 meses de vida con dificultad respiratoria caracterizada por retracciones subcostales y taquipnea persistente, posterior-mente a los 8 meses de edad se agrega hipoxemia respirando aire ambiente que requiere uso de oxígeno suplementario continuo. Tiene antecedente de tres hospitalizaciones, con diagnóstico de Bronquiolitis y Neumonía atípica, realizándose panel viral respiratorio con reporte negativo. El paciente persiste con sintomatología respiratoria a pesar de tratamientos médicos, por lo que se deriva a neumología pediátrica, unidad de enfermedad pulmonar intersticial del lactante, iniciando protocolo de estudio, se realiza tomografía tórax de alta resolución, que evidencia imágenes en vidrio despulido en lóbulo medio y región lingular, además de atrapamiento aéreo. Se concluye el diagnóstico de Hiperplasia de Células neuroendocrinas con base a la clínica y hallazgos tomográficos. La Hiperplasia de Células Neuroendocrinas es una patología pulmonar intersticial poco frecuente, cuyo diagnóstico es clínico y radiológico, en la minoría de los casos se requiere biopsia pulmonar para confirmación. Puede ser fácilmente confundida con otras enfermedades respiratorias comunes, por lo que es importante sospecharla para realizar un diagnóstico precoz. La mayor parte de los casos evolucionan con declinación de los síntomas, mejorando espontáneamente en los primeros años de vida.

A review of the literature and presentation of a clinical case of Neuroendocrine Cell Hyperplasia in a male infant patient who begins his condition at 3 months of age with respiratory distress characterized by subcostal retractions and persistent tachypnea is presented. After 8 months of age hypoxemia is added requiring continuous oxygen therapy. He has a history of three hospitalizations, with a diagnosis of bronchiolitis and atypical pneumonia, respiratory viral panel has a negative report. The patient persists with respiratory symptoms despite medical treatments, so it is referred to pediatric pulmonology, initiating study protocol for interstitial lung disease of the infant. A high resolution chest tomography is performed, which evidences images in polished glass in the middle lobe and lingular region, in addition to air entrapment. The diagnosis of neuroendocrine cell hyperplasia is concluded based on clinical and tomographic findings. Neuroendocrine Cell Hyperplasia is a rare interstitial pulmonary pathology, whose diagnosis is clinical and radiological. Lung biopsy is required only in the minority of cases for confirming diagnosis. It can be easily confused with other common respiratory diseases, so it is important to suspect it to make an early diagnosis. Most cases evolve with decline in symptoms, improving spontaneously in the first years of life.

Early diagnosis of dermatomyositis and early recognition of visceral injury / 中华皮肤科杂志

Dermatomyositis, a common autoimmune disease in clinical practice, often involves muscles and lungs, and can be complicated by malignant tumors, and the lung involvement can be fatal. Therefore, early diagnosis and treatment of dermatomyositis is of great benefit for the reduction of muscle and lung injury, early recognition and management of malignant tumors, and improvement of prognosis and survival rate of patients. However, the heterogeneity and various clinical manifestations of dermatomyositis pose challenges to early diagnosis. This article describes risk factors for dermatomyositis complicated by rapidly progressive pulmonary interstitial fibrosis, dysphagia or malignant tumors, and proposes a mode of "rashes + nailfold capillary abnormalities + myositis antibodies" for the early diagnosis of dermatomyositis, early recognition of important visceral injury and tumors, and early management, in order to improve overall survival rate of patients.

Analysis of incidence and influencing factors of interstitial pneumonia in diffuse large B-cell lymphoma after treatment of R-CHOP-like regimens / 白血病·淋巴瘤

Objective:To investigate the incidence of interstitial pneumonia (IP) and its risk factors in newly-diagnosed diffuse large B-cell lymphoma (DLBCL) after treatment of R-CHOP regimen (rituximab combined with cyclophosphamide + doxorubicin + vincristine + prednisone) and R-CDOP regimen (rituximab combined with cyclophosphamide + vincristine + liposomal doxorubicin + prednisone).Methods:The clinical data of 54 newly-diagnosed DLBCL patients who were admitted to the Central Hospital Affiliated to Shandong First Medical University from January 2015 to August 2020 were retrospectively analyzed, of which 25 cases were treated with R-CDOP regimen, and 29 cases were treated with R-CHOP regimen. The incidence of IP was compared in patients stratified according to different clinically factors, and the risk factors of IP were analyzed by multivariate logistic regression.Results:The patients with R-CDOP regimen [compared with R-CHOP regimen: 32.0% (8/25) vs. 3.4% (1/29)], normal lactate dehydrogenase level before treatment [compared with high level: 29.0% (9/31) vs. 0 (0/23)], eosinophilic count>0.1×10 9/L [compared with ≤0.1×10 9/L: 28.0% (7/25) vs. 6.9% (2/29)] and Ki-67 positive index<80% [compared with ≥80%: 23.1% (9/39) vs. 0 (0/15)] had a higher incidence of IP (all P<0.05), there were no statistical differences in the incidence of IP among patients stratified with age, gender, smoking history, underlying disease, stage, international prognostic index score, Eastern Cooperative Oncology Group score, type, B symptoms, β 2-microglobulin, and lymphocyte count (all P>0.05). Multivariate logistic regression analysis showed that the application of R-CDOP regimen was the independent risk factor for the incidence of IP (compared with R-CHOP regimen: OR = 2.898, 95% CI 1.358-6.176, P = 0.008). Conclusions:The application of chemotherapy with R-CDOP regimen in DLBCL patients increases the incidence risk of IP, which needs to be closely monitored and prevented during treatment.

Tomographic pleuropulmonary manifestations in rheumatoid arthritis: a pictorial essay / Manifestações pleuropulmonares tomográficas na artrite reumatoide: ensaio pictórico

ABSTRACT Rheumatoid arthritis (RA) is an autoimmune inflammatory and heterogeneous disease that affects several systems, especially the joints. Among the extra-articular manifestations of RA, pleuropulmonary involvement occurs frequently, with different presentations, potentially in all anatomic thoracic compartments, and may determine high morbidity and mortality. The most common pleuropulmonary manifestations in patients with RA include interstitial lung disease (ILD), pleural disease, pulmonary arterial hypertension, rheumatoid lung nodules, airway disease (bronchiectasis and bronchiolitis), and lymphadenopathy. Pulmonary hypertension and ILD are the manifestations with the greatest negative impact in prognosis. HRCT of the chest is essential in the evaluation of patients with RA with respiratory symptoms, especially those with higher risk factors for ILD, such as male gender, smoking, older age, high levels of rheumatoid factor, or positive anti-cyclic citrullinated peptide antibody results. Additionally, other etiologies that may determine tomographic pleuropulmonary manifestations in patients with RA are infections, neoplasms, and drug-induced lung disease. In these scenarios, clinical presentation is heterogeneous, varying from being asymptomatic to having progressive respiratory failure. Knowledge on the potential etiologies causing tomographic pleuropulmonary manifestations in patients with RA coupled with proper clinical reasoning is crucial to diagnose and treat these patients.

RESUMO A artrite reumatoide (AR) é uma doença inflamatória autoimune e heterogênea que afeta vários sistemas, principalmente as articulações. Dentre as manifestações extra-articulares da AR, o acometimento pleuropulmonar ocorre com frequência, com diferentes apresentações, potencialmente em todos os compartimentos anatômicos do tórax e pode determinar alta morbidade e mortalidade. As manifestações pleuropulmonares mais comuns em pacientes com AR incluem doença pulmonar intersticial (DPI), doença pleural, hipertensão arterial pulmonar, nódulos pulmonares reumatoides, doença das vias aéreas (bronquiectasia e bronquiolite) e linfadenopatia. A hipertensão pulmonar e a DPI são as manifestações com maior impacto negativo no prognóstico. A TCAR de tórax é essencial na avaliação de pacientes com AR sintomáticos respiratórios, principalmente aqueles com fatores de risco maiores para DPI, como sexo masculino, tabagismo, idade mais avançada, níveis elevados de fator reumatoide ou anticorpos antipeptídeos citrulinados cíclicos positivos. Além disso, outras etiologias que podem determinar manifestações pleuropulmonares tomográficas em pacientes com AR são infecções, neoplasias e doença pulmonar induzida por drogas. Nesses cenários, a apresentação clínica é heterogênea, variando de ausência de sintomas a insuficiência respiratória progressiva. O conhecimento das possíveis etiologias causadoras de manifestações pleuropulmonares tomográficas em pacientes com AR, aliado a um raciocínio clínico adequado, é fundamental para o diagnóstico e tratamento desses pacientes.

Progressive fibrotic interstitial lung disease / Doença pulmonar intersticial fibrosante progressiva

ABSTRACT Many interstitial lung diseases (ILDs) share mechanisms that result in a progressive fibrosing phenotype. In Brazil, the most common progressive fibrosing interstitial lung diseases (PF-ILDs) are chronic hypersensitivity pneumonitis, idiopathic pulmonary fibrosis, unclassified ILD, and connective tissue diseases. PF-ILD is seen in approximately 30% of patients with ILD. Because PF-ILD is characterized by disease progression after initiation of appropriate treatment, a diagnosis of the disease resulting in fibrosis is critical. Different criteria have been proposed to define progressive disease, including worsening respiratory symptoms, lung function decline, and radiological evidence of disease progression. Although the time elapsed between diagnosis and progression varies, progression can occur at any time after diagnosis. Several factors indicate an increased risk of progression and death. In the last few years, antifibrotic drugs used in patients with idiopathic pulmonary fibrosis have been tested in patients with PF-ILD. The effects of nintedanib and placebo have been compared in patients with PF-ILD, a mean difference of 107.0 mL/year being observed, favoring nintedanib. The U.S. Food and Drug Administration and the Brazilian Health Regulatory Agency have approved the use of nintedanib in such patients on the basis of this finding. Pirfenidone has been evaluated in patients with unclassified ILD and in patients with other ILDs, the results being similar to those for nintedanib. More studies are needed in order to identify markers of increased risk of progression in patients with ILD and determine the likelihood of response to treatment with standard or new drugs.

RESUMO Muitas doenças pulmonares intersticiais (DPI) compartilham mecanismos que resultam em um fenótipo fibrosante progressivo. No Brasil, as doenças pulmonares intersticiais fibrosantes progressivas (DPI-FP) mais comuns são a pneumonite de hipersensibilidade crônica, a fibrose pulmonar idiopática, a DPI não classificada e as doenças do tecido conjuntivo. A DPI-FP é observada em aproximadamente 30% dos pacientes com DPI. Como a DPI-FP é caracterizada pela progressão da doença após o início do tratamento adequado, é fundamental diagnosticar a doença que resulta em fibrose. Diferentes critérios foram propostos para definir doença progressiva, incluindo piora dos sintomas respiratórios, declínio da função pulmonar e evidências radiológicas de progressão da doença. Embora o tempo decorrido entre o diagnóstico e a progressão varie, a progressão pode ocorrer a qualquer momento após o diagnóstico. Vários fatores indicam risco aumentado de progressão e morte. Nos últimos anos, antifibróticos usados em pacientes com fibrose pulmonar idiopática foram testados em pacientes com DPI-FP. Os efeitos do nintedanibe e placebo foram comparados em pacientes com DPI-FP, com diferença média de 107,0 mL/ano a favor do nintedanibe. A Food and Drug Administration (EUA) e a Agência Nacional de Vigilância Sanitária aprovaram o uso do nintedanibe em tais pacientes com base nesse achado. A pirfenidona foi avaliada em pacientes com DPI não classificada e em pacientes com outras DPI, e os resultados foram semelhantes aos do nintedanibe. Mais estudos são necessários para identificar marcadores de risco aumentado de progressão em pacientes com DPI e determinar a probabilidade de resposta ao tratamento com medicamentos-padrão ou novos.

Openreuma consensus on the role of nursing in the care of patients with rheumatoid arthritis and diffuse interstitial lung disease / Consenso de Openreuma sobre el papel de la enfermería en el cuidado de pacientes con artritis reumatoide y enfermedad pulmonar intersticial difusa / Consenso Openreuma sobre o papel da enfermagem no cuidado de pacientes com artrite reumatoide e doença pulmonar intersticial difusa

Objective.To develop practical recommendations, based on the best available evidence and experience, on the nursing management of patients with rheumatoid arthritis (RA) and interstitial lung disease (ILD). Methods. The usual consensus methodology was used, with a nominal group, systematic reviews (SRs), and Delphi survey. The expert panel, consisting of rheumatology nurses, rheumatologists, a psychologist, a physiotherapist, and a patient, defined the scope, the users, the topics on which to explore the evidence and on which to issue recommendations. Results.Three PICO questions evaluated the efficacy and safety of pulmonary rehabilitation and non-pharmacological measures for the treatment of chronic cough and gastroesophageal reflux by means of SR of the literature. With the results of the reviews, 15 recommendations were established for which the degree of agreement was obtained with a Delphi survey. Three recommendations were rejected in the second round. The 12 recommendations were in patient assessment (n=4); patient education (n=4); and risk management (n=4). Only one recommendation was based on available evidence, while the remaining were based on expert opinion. The degree of agreement ranged from 77% to 100%. Conclusion.This document presents a series of recommendations with the aim of improving the prognosis and quality of life of patients with RA-ILD. Nursing knowledge and implementation of these recommendations can improve the follow-up and prognosis of patients with RA who present with ILD.

Objetivo.Desarrollar recomendaciones prácticas, basadas en la mejor evidencia y experiencia disponible, sobre el manejo de enfermería de los pacientes con artritis reumatoide (AR) y enfermedad pulmonar intersticial (EPI). Métodos. Se utilizó la metodología de consenso en la que un panel de expertos (formado por enfermeras de reumatología, reumatólogos, una psicóloga, una fisioterapeuta y una paciente) definió el ámbito, los usuarios, los temas sobre los que explorar la evidencia y sobre los que emitir recomendaciones. Tres preguntas PICO evaluaron la eficacia y seguridad de la rehabilitación pulmonar y las medidas no farmacológicas para el tratamiento de la tos crónica y el reflujo gastroesofágico mediante la búsqueda de revisiones sistemáticas, excluyendo aquellas cuya calidad era baja, muy baja o críticamente baja, según la herramienta AMSTAR-2. Posteriormente, se hizo una reunión para la formulación de recomendaciones que se presentaron con un resumen de la evidencia a la encuesta Delphi. Resultados.Con los resultados de las revisiones se establecieron 15 recomendaciones cuyo grado de acuerdo osciló entre el 77% y el 100% en la una encuesta Delphi. Tres recomendaciones fueron rechazadas en la segunda ronda: una por la evidencia disponible y los dos restantes se basaron en la opinión de expertos. Las 12 recomendaciones restantes aprobadas se referían a la evaluación del paciente (n=4), a la educación del paciente (n=4) y a la gestión del riesgo (n=4). Conclusión. El conocimiento del consenso Openreumapor parte de enfermería y la aplicación sus 12 recomendaciones basadas en la mejor evidencia y experiencia puede mejorar el seguimiento y el pronóstico de los pacientes con AR que presentan EPI.

Objetivo.Desenvolver recomendações práticas, baseadas na melhor evidência e experiência disponíveis, sobre o manejo de enfermagem de pacientes com artrite reumatoide (AR) e doença pulmonar intersticial (DPI). Métodos.Foi utilizada a metodologia de consenso, com grupo nominal, revisões sistemáticas e levantamento Delphi. O painel de especialistas, formado por enfermeiros reumatologistas, reumatologistas, psicólogo, fisioterapeuta e paciente, definiu o escopo, os usuários, os tópicos sobre os quais explorar as evidências e sobre os quais emitir recomendações. Três questões do PICO avaliaram a eficácia e segurança da reabilitação pulmonar e medidas não farmacológicas para o tratamento da tosse crônica e refluxo gastroesofágico por meio de RS. Aqueles cuja qualidade era baixa, muito baixa ou criticamente baixa, de acordo com a ferramenta AMSTAR-2, foram excluídos. Posteriormente, realizou-se uma reunião para formular recomendações que foram apresentadas com um resumo das evidências ao inquérito Delphi. Resultados. Com os resultados das revisões, foram estabelecidas 15 recomendações cujo grau de concordância entre 77% e 100% foi obtido com uma pesquisa Delphi. Três recomendações foram rejeitadas na segunda rodada. As 12 recomendações referiam-se à avaliação do paciente (n=4); à educação do paciente (n=4); e ao gerenciamento de risco (n=4). Apenas uma recomendação foi baseada nas evidências disponíveis, enquanto as demais foram baseadas na opinião de especialistas. Conclusão. Este documento apresenta uma série de recomendações com o objetivo de melhorar o prognóstico e a qualidade de vida dos pacientes com AR-ILD. O conhecimento da enfermagem e a aplicação dessas recomendações podem melhorar o acompanhamento e o prognóstico de pacientes com AR com DPI.

Pneumonite de hipersensibilidade na infância / Hypersensitivity pneumonitis in childhood

Neste relato descrevemos as características clínicas, epidemiológicas e radiológicas da pneumonite de hipersensibilidade, uma causa rara de insuficiência respiratória em pediatria. Paciente masculino, com 8 anos de idade, proveniente da zona rural, admitido em serviço terciário por quadro de febre, vômitos, tosse seca, dispneia progressiva, anorexia e perda de peso há 15 dias, associado a taquipneia, esforço respiratório, hipóxia e estertores finos em base direita. Tomografia computadoriza de tórax demonstrou opacidades com atenuações em vidro fosco, com comprometimento difuso e distribuição predominantemente centrolobular e acinar, característicos de pneumonite por hipersensibilidade. Na revisão das condições e hábitos de vida, foi relatado pela responsável do paciente a presença de um aviário e convívio com aves de várias espécies na residência, reforçando a hipótese diagnóstica, após descartadas outras causas de insuficiência respiratória. Iniciado corticoterapia com metilprednisolona 1 mg/ kg/dia por 7 dias, seguido de redução progressiva nas semanas posteriores. Paciente evoluiu com melhora do quadro e alta hospitalar, após orientações sobre controle ambiental e importância do afastamento dos antígenos desencadeantes. A pneumonite por hipersensibilidade é uma síndrome incomum na população pediátrica, que pode levar à insuficiência respiratória e fibrose pulmonar, devendo ser considerada nos pacientes com epidemiologia positiva. Pela sua raridade e semelhança com outras infecções respiratórias, ressalta-se ainda a importância da coleta de dados sobre os hábitos de vida dos pacientes, destacando sua importância para a elucidação diagnóstica.

We report the clinical, epidemiological, and radiological features of hypersensitivity pneumonitis, a rare cause of respiratory failure in pediatrics. An 8-year-old male patient, from a rural area, was admitted to a tertiary care facility for fever, vomiting, dry cough, progressive dyspnea, anorexia, and weight loss for 15 days, associated with tachypnea, respiratory effort, hypoxia, and fine rales at the right base. Chest computed tomography showed ground-glass opacities, diffuse involvement, and predominantly centrilobular and acinar distribution, characteristic of hypersensitivity pneumonitis. In the review of living conditions and habits, the patient's guardian reported the presence of an aviary and interaction with birds of various species in the residence, supporting the presumptive diagnosis of hypersensitivity pneumonitis, after ruling out other causes of respiratory failure. Corticosteroid therapy was started with methylprednisolone 1 mg/kg/day for 7 days, followed by tapering over subsequent weeks. The patient's condition improved, and he was discharged home after receiving guidance on environmental control and the importance of removing the triggering antigens. Hypersensitivity pneumonitis is an uncommon syndrome in the pediatric population. It can lead to respiratory failure and pulmonary fibrosis and should therefore be considered in patients with a positive epidemiological history. Due to its rarity and similarity to other respiratory diseases, collecting data on patients' lifestyle habits is highlighted as an important diagnostic tool.

Silicosis crónica complicada, hallazgo en un paciente asintomático / Complicated Chronic Silicosis: Findings in Asymptomatic Patient

La silicosis es producida por la inhalación mantenida de dióxido de silicio (SiO2). Los trabajos en canteras e industrias de roca ornamental, granito y pizarra, y marmolerías son las exposiciones clásicamente descriptas, junto al pulido con chorro de arena. El arenado de jeans, producción de mesadas de cocinas y baños han surgido más recientemente. Las presentaciones clínicas pueden clasificarse en silicosis crónica (sim ple, complicada y fibrosis pulmonar), silicosis acelerada y silicosis aguda, según los años de exposición y factores individuales. Se presenta un caso clínico de un paciente masculino de 78 años con silicosis crónica complicada, asintomático, diagnosticado en contexto de un examen prequirúrgico.

Silicosis is caused by sustained inhalation of silicon dioxide (SiO2). Work in quarries and industries of ornamental rock, granite and slate, and marble work, are the typi cally described types of exposure, together with sandblasting. Denim sandblasting and the production of kitchen and bathroom countertops have emerged more recently. Clinical presentations can be classified into chronic silicosis (simple, complicated, and pulmonary fibrosis), accelerated silicosis, and acute silicosis, depending on years of exposure and individual factors. We present a clinical case of a 78-year-old male pa tient with complicated chronic silicosis, asymptomatic, diagnosed in the context of a pre-surgical examination.

Manifestaciones pulmonares al diagnóstico de 170 pacientes con vasculitis asociadas a anticuerpos anticitoplasma de neutrófilos: cohorte retrospectiva de un centro universitario / Pulmonary manifestations at the diagnosis of 170 patients with vasculitis associated with anticytoplasmic neutrophil antibodies: retrospective cohort of a university center

INTRODUCCIÓN: Las Vasculitis Asociadas a Anticuerpos Anticitoplasma de Neutrófilos (VAA) son infrecuentes, pero de amplio espectro de presentación. Si bien el consenso de clasificación de Chapel Hill del año 2012, sigue vigente, la tendencia actual es clasificarlas de acuerdo al marcador inmunológico: anti-Proteinasa 3 (PR3) o anti-mieloperoxidasa (MPO). Las manifestaciones pulmonares clásicas son la hemorragia alveolar y los nódulos pulmonares. En los últimos 10 años se ha descrito la enfermedad pulmonar difusa (EPD). Los estudios epidemiológicos son escasos, y suelen representar en su mayoría poblaciones norteamericanas o europeas. El objetivo es describir las características del compromiso pulmonar al debut en VAA en un centro universitario. PACIENTES Y MÉTODO: De un total de 190 pacientes con diagnóstico de VAA se incluyeron 170 en seguimiento en nuestro centro. Se revisaron aspectos clínicos, demográficos, laboratorio e imagenológicos de los pacientes incluidos. RESULTADOS: De los 170 pacientes, 112 (65,88%) presentaron compromiso pulmonar. 106 (94,64%) de los pacientes fueron anticuerpos anti citoplasma de neutrófilos (ANCA) positivos; de estos, 56 (53,27%) MPO (+) y 39 (36,45%) PR-3 (+). Un tercio de los pacientes de ambos grupos presentó hemorragia alveolar. En los pacientes MPO (+) predomina la EPD (53,5%) y en PR-3 (+) los nódulos pulmonares (69,23%). Destaca la baja frecuencia de patología obstructiva asociada. CONCLUSIONES: El compromiso pulmonar en las VAA es prevalente y heterogéneo. En nuestra serie, destaca la frecuencia de EPD en VAA MPO (+), lo que releva la importancia del estudio con ANCA en paciente con diagnóstico y seguimiento por EPD.

INTRODUCTION: Antineutrophil Cytoplasmic Antibodies (ANCA) associated vasculitis (AAV) are uncommon, but of broad spectrum of presentation. Although the 2012 Chapel Hill classification consensus remains valid, the current trend is to classify them according to the immunological marker: anti-Proteinase 3 antibody (PR-3) or anti-Myeloperoxidase antibody (MPO). The classic pulmonary manifestations are alveolar hemorrhage and pulmonary nodules. Interstitial lung disease (ILD) has been described in the last 10 years. Epidemiological studies are scarce, and they usually represent mostly North American or European populations. The objective is to describe the characteristics of lung involvement upon debut in AAV in a university center. PATIENTS AND METHODS: Of a total of 190 patients diagnosed with AAV, 170 were included in follow-up at our center. Clinical, demographic, laboratory and imaging aspects of the included patients were reviewed. RESULTS: Of the 170 patients, 112 (65.88%) had lung involvement. 106 (94.64%) of the patients were ANCA (+); of these, 56 (53.27%) MPO (+) and 39 (36.45%) PR-3 (+). One third of the patients in both groups had alveolar hemorrhage. In MPO (+) patients, ILD predominates (53.5%) and in PR-3 (+) pulmonary nodules (69.23%). The low frequency of associated obstructive pathology stands out. CONCLUSIONS: Pulmonary manifestations in AAVs are frequent and heterogeneous. Locally, the association of ILD and AAV MPO (+) stands out, which highlights the importance of ANCA study in patients with diagnosis and follow-up by ILD.

Radiological and histological characteristics of patients with interstitial lung disease undergoing lung biopsy in a reference institution from eastern Colombia / Características radiológicas e histológicas de pacientes con enfermedad pulmonar intersticial llevados a biopsia pulmonar en una institución del oriente colombiano

Abstract Introduction: Interstitial lung disease (ILD) diagnosis requires a mukidisciplinary approach and, in some cases, lung biopsy. Objective: To describe the sociodemographic and clinical characteristics, as well as the radiological and histological findings, of patients with ILD who required lung biopsy after a mukidisciplinary board (pneumology, radiology, and pathology) of a reference center for respiratory diseases in Bucaramanga, Colombia, failed to reach the ILD diagnosis. Materials and methods: Cross-sectional study. The medical records of 56 patients treated at the Instituto Neumológico del Oriente who underwent lung biopsy between 2015 and 2019 were reviewed. Measures of central tendency and dispersion were calculated for demographic and clinical variables, respectively, to characterize them. A bivariate analysis was performed using Fisher's exact test to determine whether there were differences in the distribution of the sociodemographic and clinical variables according to the radiological patterns and the final histological diagnosis. Results: Participants' median age was 67 years (IQR: 59-72) and 55.35% were men. 43 patients had a radiological pattern inconsistent with usual interstitial pneumonia (UIP); 4 had a pattern consistent with possible UIP; and 9 had a pattern consistent with UIP. The most common histologic diagnoses were hypersensitivity pneumonitis (HP) (32.14%), nonspecific interstitial pneumonia (NSIP) (17.86%), and UIP (19.64%). Conclusion: In the study population, the primary reason for performing a lung biopsy was the presence of a radiologic pattern inconsistent with UIP, with HP being the predominant histopathological diagnosis. This is the first study to characterize patients with ILD who underwent lung biopsy in eastern Colombia, making a significant contribution to our understanding of the disease's epidemiology in the country.

Resumen Introducción. El diagnóstico de la enfermedad pulmonar intersticial (EPI) requiere un enfoque multidisciplinar y, en ocasiones, de una biopsia pulmonar. Objetivo. Describir las características sociodemográficas y clínicas, y los hallazgos radiológicos e histológicos de pacientes con EPI que requirieron biopsia pulmonar luego de no lograrse un diagnóstico de esta enfermedad por la junta médica multidisciplinar (neumología, radiología y patología) de un centro de referencia en enfermedades respiratorias de Bucaramanga, Colombia. Materiales y métodos. Estudio transversal. Se revisaron las historias clínicas de 56 pacientes atendidos en el Instituto Neumológico del Oriente y que fueron remitidos a biopsia pulmonar entre 2015 y 2019. Se analizaron variables demográficas y clínicas, calculando medidas de tendencia central y de dispersión para su respectiva caracterización. Se realizó un análisis bivariado mediante test exacto de Fisher para determinar si existían diferencias en la distribución de las variables sociodemográficas y clínicas de acuerdo con los patrones radiológicos y el diagnóstico histológico definitivo. Resultados. La mediana de edad fue 67 años (RIC: 59-72), 55.35% fueron hombres. 43 pacientes presentaron patrón radiológico inconsistente con neumonía intersticial usual (NIU); 4, patrón de posible NIU y, 9, patrón de NIU. Los diagnósticos histológicos más frecuentes fueron neumonitis por hipersensibilidad (NH) (32.14%), neumonía intersticial no específica (17.86%) y NIU (19.64%). Conclusión. La principal razón para realizar biopsia pulmonar en la población de estudio fue la presencia de un patrón radiológico inconsistente con NIU, siendo la NH el principal diagnóstico histopatológico. Este es el primer trabajo que caracteriza a pacientes con EPI del oriente colombiano llevados a biopsia pulmonar, lo que representa un importante aporte al conocimiento de la epidemiología de esta enfermedad en Colombia.

Prevalencia y letalidad de enfermedades pulmonares intersticiales en la Región de Los Ríos, Chile / Prevalence and lethality of interstitial pulmonary diseases in the Region of Los Ríos, Chile

BACKGROUND: Idiopathic Pulmonary Fibrosis (IPF) is a subtype of interstitial lung disease (ILD) that significantly affects people's quality of life, whose prevalence and mortality has increased. AIM: To determine the prevalence and lethality of ILD in the Los Ríos Region between the years 2018 and 2019. MATERIAL AND METHODS: Review of a database of all patients attended at an outpatient clinic of the regional hospital. Those patients with the diagnosis of ILD and seen between 2018 and 2019 were selected. Mortality and its causes were verified with death certificates. RESULTS: In the study period, 339 cases with ILD, aged 71 ± 10 years (64% women) were identified. The calculated ILD prevalence was 84 cases per 100,000 inhabitants, with a higher predominancy in Futrono and Paillaco communes. IPF and Connective Tissue Diseases were the predominant subtypes. Overall lethality was 18%, with more deaths among patients with IPF (n = 31). CONCLUSIONS: In the Los Ríos Region, ILDs have a relevant prevalence and lethality.

Association between serum albumin level and prognosis in patients with scleroderma-associated interstitial lung disease / 中华全科医师杂志

Objective:To examine the association between baseline serum albumin level and short-term, long-term outcomes in patients with systemic sclerosis associated interstitial lung disease (SSc-ILD).Methods:A total of 259 patients with SSc-ILD who were hospitalized in West China Hospital of Sichuan University from January 2008 to December 2018 were enrolled. The data of serum albumin, demographic characteristics, peripheral blood hemoglobin at admission, and treatment plan were obtained from the hospital information management system, and the survival of the patients were followed up to June 1, 2019. The mean baseline serum albumin of 259 patients was 37.67 g/L, there were 118 patients with serum albumin ≤37.67 g/L (low protein group) and 141 patients with serum albumin>37.67 g/L (high protein group).Results:There were 64 males and 195 females with a mean age of 50.0(41.0, 61.0) years. The follow-up time was 627(61, 1 426) days. Compared to high protein group, the low protein group had higher proportion of male patients [30.5%(36/118) and 19.9%(28/141),χ 2=3.92, P=0.048], and higher levels of the erythrocyte sedimentation rate [45.0(27.0,69.0) vs. 29.0 (19.0,46.0)mm/1 h,χ 2=4.07, P<0.001], neutrophil percentage [71.50(63.35,77.13) vs. 65.60(59.50,72.50)%,χ 2=3.65, P<0.001], platelet [196(140,273) vs. 172(126,240)×10 9/L,χ 2=1.99, P=0.046], nutrophil/lymphocyte ratio [33.85(2.53,5.28) vs. 2.61(1.97,3.83),χ 2=4.57, P<0.001], platelet/lymphocyte ratio [149.0(112.0,216.8) vs. 113.5(72.76,158.8),χ 2=4.98, P<0.001], aspartate aminotransferase [27.0(21.0,39.0) vs. 23.0 (19.5,30.0) IU/L,χ 2=2.93, P=0.003], globulin [31.20(26.90,36.83) vs. 29.50(25.65,32.80) g/L,χ 2=2.28, P=0.023], serum cystatin C[1.14(0.98,1.33) vs. 1.02(0.88,1.16) mg/L,χ 2=3.80, P<0.001], IgA[2 710 (1 965,3 505) vs. 2 460 (1 862,3 105) mg/L,χ 2=2.13, P=0.033], IgG[15.05(12.83,21.08) vs. 13.60(11.53,17.23)g/L,χ 2=3.24, P=0.001], IgE[60.44(24.92,197.99) vs. 34.82(14.72,85.04) kIU/L,χ 2=3.33, P=0.001] and circulating immune complex [0.13(0.08,0.19) vs. 0.10(0.08,0.13)O.D,χ 2=2.60, P=0.009]; and lower levels of hemoglobin [121.5(101.8,132.0) vs. 129.0(119.0,142.0) g/L,χ 2=5.05, P<0.001], albumin [(33.28±3.49) vs.(41.34±2.95) g/L,χ 2=20.17, P<0.001] and IgM[1 320 (932,1 745) vs.1 560(1 170,2 030) mg/L, χ 2=2.63, P=0.009]. The utilization rate of antibiotics was higher in the low protein group (60 vs. 43 cases, χ 2=11.10, P=0.001). The number of patients followed up to 1, 5, and 10 years were 248, 245, and 244, respectively. The 1-year, 5-year, and 10-year cumulative survival rates of patients in low protein group and high protein group were(91.0% vs. 98.4%, χ 2=6.23, P=0.013;87.0% vs. 97.1%, χ 2=6.15, P=0.013; 81.6% vs. 97.1%, χ 2=7.00, P=0.008) respectively. Conclusions:Patients with scleroderma-associated interstitial lung disease have an increased risk of poorer prognosis when serum albumin ≤37.67 g/L.

Clinical characteristics of patients with antisynthetase syndrome and positive anti-Ro52 antibody / 中华内科杂志

To analyze the clinical characteristics of patients with antisynthetase syndrome (ASS) and positive anti-Ro52 antibody. The clinical data of 203 ASS patients admitted to the First Affiliated Hospital of Zhengzhou University from 2017 to 2020 were analyzed retrospectively. Demographics, clinical manifestations, laboratory results, treatment and outcome were collected including data of 18 patients with rapidly progressive interstitial lung disease (RP-ILD). In total, the majority were women (148,72.9%). The average onset age was (51.9±13.3) years. There were 163 (80.3%) patients with positive anti-Ro52 antibody. The positivity in women (77.3% vs. 55.0%, P=0.004) was higher, and the median time from disease onset to diagnosis [4.5 (2.0, 24.0) months vs. 2.0 (1.0, 12.0) months, P=0.024] was longer in patients with positive anti-Ro52 antibody than those negative. Compared with negative patients, patients with positive anti-Ro52 antibody had a higher incidence of interstitial lung disease (ILD) (96.9% vs. 65.0%, P<0.001), arthritis (33.7% vs. 17.5%, P=0.046), and arthralgia (39.3% vs. 20.0%, P=0.022). Higher rate of positve antinuclear antibody (ANA) (85.3% vs. 55.0%, P<0.001), lower rate of positive anti-Jo-1 antibody (32.5% vs. 50.0%, P=0.039), lower albumin level [(34.6±5.2) g/L vs. (37.3±4.7) g/L, P=0.004] and lower lymphocyte counts [(1.4±0.8) ×10 9/L vs. (1.8±0.8) ×10 9/L, P=0.014] were more common in patients with positive anti-Ro52 antibody. The presence of anti-Ro52 antibody is associated with a particular phenotype of ASS, leading to common ILD, involvement of joints, high ANA positivity, low albumin and low lymphocyte counts.