The impact of hospitalization on mortality in patients with connective tissue disease-associated interstitial lung disease: a medical records review study

Abstract Background Interstitial lung disease (ILD) remains one of the most important causes of morbidity and mortality in patients with Connective Tissue Diseases (CTD). This study evaluated the impact of hospitalization on mortality in an ethnically and racially diverse cohort of CTD-ILD patients. Methods We conducted a medical records review study at Montefiore Medical Center, Bronx, NY. We included 96 patients and collected data on demographic characteristics, reasons for hospitalization, length of stay, immunosuppressant therapy use, and mortality. We stratified our patients into two cohorts: hospitalized and nonhospitalized. The hospitalized cohort was further subdivided into cardiopulmonary and non-cardiopulmonary admissions. Two-sample tests or Wilcoxon's rank sum tests for continuous variables and Chi-square or Fisher's exact tests for categorical variables were used for analyses as deemed appropriate. Results We identified 213 patients with CTD-ILD. Out of them, 96 patients met the study's inclusion criteria. The majority of patients were females (79%), and self-identified as Hispanic (54%) and Black (40%). The most common CTDs were rheumatoid arthritis (RA) (29%), inflammatory myositis (22%), and systemic sclerosis (15%). The majority (76%) of patients required at least one hospitalization. In the non-hospitalized group, no deaths were observed, however we noted significant increase of mortality risk in hospitalized group (p = 0.02). We also observed that prolonged hospital stay (> 7 days) as well as older age and male sex were associated with increased mortality. Conclusion Prolonged (> 7 days) hospital stay and hospitalization for cardiopulmonary causes, as well as older age and male sex were associated with an increased mortality risk in our cohort of CTD-ILD patients.

A Challenging Case Of Pulmonary Arterial Hypertension: Never Judge By Appearances

Abstract We report the case of a patient with symptomatic pulmonary hypertension (PH) associated with diffuse systemic sclerosis (SSc) whose initial assessment suggested a group 3 (clinical classification) PH. The patient had a history of drugs/toxins consumption, which contributed to the development of intrinsic pulmonary vascular disease. This changed the panorama towards the diagnosis of pulmonary arterial hypertension (PAH), with important therapeutic and prognostic implications. In fact, the excellent clinical, laboratory and hemodynamic response to therapy confirmed the hypothesis of a case of drug-associated PAH (DPAH) in a patient with diffuse SSc and lung disease. Considering the presence of DPAH, it was deemed necessary to assess acute vasoreactivity during right heart catheterization (RHC). If criteria were met, the clinical scenario may change towards a favorable and sustained clinical and hemodynamic response with oral calcium channel blockers. However, the response to inhaled nitric oxide was negative in our patient and the therapeutic strategy with dual oral combination therapy with tadalafil and ambrisentan was continued. After six-months of therapy the patient significantly improved, from a high to a low risk of one-year mortality.

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.

Mechanism of action and clinical research progress of iguratimod in connective tissue diseases / 中国药房

As a new type of immunosuppressant，iguratimod can mediate the anti-inflammatory signaling pathway by inhibiting the proliferation of inflammatory cells and reducing the release of inflammatory cytokines， and play the role of anti-inflammatory. It can affect the proliferation of immune cells and the expression of immune factors，reduce the production and deposition of immune complexes in the body，and play the role of immune regulation. It can regulate bone metabolism by mediating signaling pathways such as Wnt/β-catenin，Toll-like receptor 4/nuclear factor-κB and osteoprotegerin/nuclear factor-κB receptor activating factor ligand， and play a role in bone protection. It can inhibit pulmonary fibrosis by inhibiting the expression of transforming growth factor β1/ Smad2/3 signaling pathway，tumor necrosis factor-α，interleukin-1，interleukin-6，matrix metalloproteinase-9 and other inflammatory cytokines in lung tissue，and inhibiting the expression of collagen and fibronectin. Its efficacy and safety have been confirmed in the clinical application of rheumatoid arthritis and primary Sjogren syndrome and included in the diagnosis and treatment of the disease. It has also shown good efficacy in the clinical application of other connective tissue diseases such as systemic lupus erythematosus and ankylosing spondylitis，and no obvious safety risks have been found.

A modified tunnel technique to treat multiple gingival recessions: Case Series

Abstract This case series reports a modified tunnel technique with connective tissue graft for the root coverage of multiple Miller Class I, II, and III gingival recessions. The modified approach presents an innovative suture technique to improve the stability and position of the graft. Ten patients with multiple gingival recessions (n=85 teeth) received surgical root coverage treatment. The gingival recession height and width were measured and presented as median, minimum, and maximum values. The percentage of the root coverage after at least 12 months expressed the treatment effectiveness. The Shapiro-Wilk test evaluated the normality; pared Wilcoxon test determined the exact P-value for the differences in the height of the gingival recession before and after surgical treatment (α = 0.05). An average of 97.9% (± 5.6%, p < 0.0001) root coverage after treatment occurred, and 73 out of 85 recessions presented complete root coverage after 12 months. Treatment of Miller class I and II gingival recessions resulted in root coverage higher than 99 and class III higher than 95% (p < 0.0001). The presented case series report the efficacy of a modified surgical technique promoting more than 95% of root coverage after 12 months in multiple Miller Class I, II, and III gingival recessions. Well-designed blind randomized controlled trials are needed to validate the proposed technique.

Resumo Esta série de casos relata uma técnica de túnel modificada com enxerto de tecido conjuntivo para o recobrimento radicular de múltiplas recessões gengivais Classe I, II e III de Miller. A abordagem modificada apresenta uma técnica de sutura inovadora para melhorar a estabilidade e a posição do enxerto. Dez pacientes com múltiplas recessões gengivais (n=85 dentes) receberam tratamento cirúrgico de recobrimento radicular. A altura e a largura da recessão gengival foram mensuradas e apresentadas como valores medianos, mínimos e máximos. A eficácia do tratamento foi expressa como uma porcentagem da cobertura radicular após pelo menos 12 meses. O teste de Shapiro-Wilk avaliou a normalidade; o teste de Wilcoxon pared determinou o valor P exato para as diferenças entre a altura da recessão gengival antes e após o tratamento cirúrgico (α = 0,05). Uma média de 97,9% (± 5,6%, p < 0,0001) de cobertura radicular após o tratamento ocorreu, e 73 das 85 recessões apresentaram cobertura radicular completa após 12 meses. O tratamento das recessões gengivais classe I e II de Miller resultou em recobrimento radicular superior a 99 e classe III superior a 95% (p < 0,0001). A série de casos apresentada relata a eficácia de uma técnica cirúrgica modificada promovendo mais de 95% de cobertura radicular após 12 meses em múltiplas recessões gengivais Classe I, II e III de Miller. Ensaios controlados randomizados cegos bem desenhados são necessários para validar a técnica proposta.

Evaluation of Connective Tissue Changes in Different Histological Grades of Oral Squamous Cell Carcinoma: A Histochemical Study

Introduction: In squamous cell carcinoma, cells invade the stroma in the form of islands, strands or sheets, which are surrounded by an extracellular matrix, thus producing reactive changes in the stroma. These reactive changes in the stroma may alter the biological behavior of oral cancer which convey some diagnostic and prognostic significance. Objective: This study was to compare staining intensity of various components of connective tissue such as collagen, elastin and glycoprotein among three histological grades of oral squamous cell carcinoma and normal oral mucosa. Materials and Methods: A total sample of 48 in which 36 cases of histologically diagnosed oral squamous cell carcinoma, 12 each of well, moderate and poorly differentiated squamous cell carcinomas and 12 sections of normal mucosa as the control group were selected for the present study. The sections of tissue blocks were stained with connective tissue specific stains such as Verhoeff’s -VanGieson stain and PAS for collagen, elastin and glycoprotein respectively. Results: Staining intensity of collagen, elastin and glycoprotein around tumor island among different grades of OSCC and normal mucosa revealed statistically significant changes (P value <0.001). Collagen and glycoprotein degradation and elastosis are more prominent in poorly differentiated squamous cell carcinomas. Conclusion: Observable changes were seen in the stroma, in all the three grades of OSCC’s compared to normal mucosa. There was an increased stromal response in poorly differentiated carcinomas, when compared to the other grades. Role of the stroma is like a double-edged sword, at times helping in tumor invasion and otherwise warding off the tumor cells.

Update in Imaging Evaluation of Bone and Soft Tissue Sarcomas / Atualização na avaliação por imagens dos sarcomas ósseos e das partes moles

Abstract The evolution in imaging evaluation of musculoskeletal sarcomas contributed to a significant improvement in the prognosis and survival of patients with these neoplasms. The precise characterization of these lesions, using the most appropriate imaging modalities to each clinical condition presented, is of paramount importance in the design of the therapeutic approach to be instituted, with a direct impact on clinical outcomes. The present article seeks to update the reader regarding imaging methodologies in the context of local and systemic evaluation of bone sarcomas and soft tissues.

Resumo A evolução na avaliação por imagens dos sarcomas musculoesqueléticos contribuiu para melhora significativa no prognóstico e na sobrevida dos portadores destas neoplasias. A caracterização precisa destas lesões, mediante utilização das modalidades de imagem mais adequadas a cada condição clínica apresentada, é de suma importância no delineamento da abordagem terapêutica a ser instituída, com impacto direto sobre os desfechos clínicos. O presente artigo busca atualizar o leitor a propósito das metodologias de imagem no contexto da avaliação local e sistêmica dos sarcomas ósseos e das partes moles.

Clinical Profile of Sharp Syndrome: Is it rare in India?

Background: In 1972, Dr Sharp and colleagues described a new connective tissue disease, characterized by overlapping features of systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and polymyositis/ dermatomyositis (PM/DM) and by the presence of antibodies against the U1 small nuclear ribonucleoprotein autoantigen (U1 snRNP). This condition was termed mixed connective tissue disease (MCTD) and proposed as a distinct disease. Later, after observing the clinical evolution of MCTD patients, Sharp himself agreed that the original concept of MCTD had to be modified and that Internal organs were at risk for serious complications; patients were not always steroid responsive; prognosis was not always benign. Materials and methods: Patients in the age group of 15-50 years diagnosed to have connective tissue diseases were included. 8 patients in the age group of 15-50 admitted in Medicine department were taken and they were evaluated for the clinical profile of sharp syndrome by thorough clinical examination, routine laboratory tests and special investigations depending on the clinical profile. Results: 8 patients with connective tissue disease attending the medicine OPD were studied. Of these 8 patients were female patients. The median age of onset was 36 years, 8 patients met criteria by sharp and Alarcon-Segovia. The clinical features of patients at presentation are Raynaud抯 phenomena, Puffy fingers, esophagus dysmotility, skin rash, interstitial lung disease, arthritis, pulmonary hypertension, myositis, anemia. Conclusion: SHARP syndrome is a rare condition, as evidenced by the small series of cases reported to date. Diagnosis is based on clinical and paraclinical criteria. The evolution can be interspersed with various complications that can affect the short, medium and long-term prognosis

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.

Caracterización clínico-radiológica de pacientes con enfermedad pulmonar intersticial y panel de miositis positivo en hospital Santiago Oriente / Clinical-radiological characterization of patients with interstitial lung disease and positive myositis panel at hospital Santiago Oriente

Introducción: Las Enfermedades Pulmonares Intersticiales (EPI) afectan principalmente al intersticio pulmonar, con importante morbimortalidad asociada. Tienen un espectro de posibles etiologías que es cada vez más amplio. Hay una importante causalidad a partir de Enfermedades del Tejido Conectivo (ETC), describiéndose cada vez más casos asociados a Síndrome Antisintetasa, y con diversos patrones radiológicos según serología obtenida, agrupada en "Panel de Miositis" (PaM). El presente estudio de cohorte retrospectiva reúne PaMs realizados en el Hospital Santiago Oriente, correlacionando resultados con manifestaciones clínicas e imagenológicas. Material y Métodos: Se recuperaron 33 PaMs realizados entre 2017 y 2022, y a través de revisión de fichas de los pacientes de quienes provenían las PaMs se consignaron las principales manifestaciones clínicas, imagenológicas y de la serología reumatológica complementaria, estableciendo correlaciones entre múltiples variables. Resultados: Hubo 15 pacientes PaM positivos (45,4%), 8 de ellos (53%) ya contaban con alguna miopatía inflamatoria diagnosticada. Los principales hallazgos clínicos consignados fueron pápulas de Gottron, artritis, eritema heliotropo, Fenómeno de Raynaud y fiebre. El anticuerpo positivo más frecuente fue Ro-52. Se pudo objetivar ANA positivo en 10 casos (66,7%). Se identificó EPI en 66,7% de aquellos con PaM positivo, siendo la Neumonía Intersticial no específica fibrótica con Neumonía en Organización la manifestación más frecuente. No hubo asociación significativa entre manifestaciones imagenológicas y anticuerpos específicos. Se encontró ANA 1/80 en 66,7% de los casos, lo cual no se asoció a mayor riesgo de EPI. Conclusiones: Existe asociación entre varias ETC y las EPI. Destaca la importancia de los hallazgos clínicos para establecer un adecuado índice de sospecha, para dirigir oportunamente el estudio complementario (ej: PaM), y la eventual terapia específica.

Introduction: Interstitial Lung Diseases (ILD) mainly affect the pulmonary interstitium, with significant associated morbidity and mortality. They have a spectrum of possible etiologies that is increasingly broad. There is an important causality from Connective Tissue Diseases (CTD), describing more and more cases associated with Antisynthetase Syndrome, and with different radiological patterns according to the serology obtained, enclosed into "Panel of Myositis" (PaM). This retrospective cohort study gathers PaMs performed at Hospital Santiago Oriente, PaM results are correlated with clinical and imaging manifestations. Material and Methods: 33 PaMs performed between 2017 and 2022 were saved up and by reviewing the clinical records of the patients from whom the PaMs came, their clinical and radiological manifestations and the results of their complementary rheumatological serology were recorded to establish correlations between multiple variables. Results: There were 15 positive PaMs (45.4%), 8 (53%) of them already had some diagnosed inflammatory myopathy. The main clinical findings reported were Gottron's papules, arthritis, heliotrope erythema, Raynaud's phenomenon, and fever. The most frequent positive antibody detected was Ro-52. Positive ANA could be found in 10 cases (66.7%). PID was identified in 66.7% of those with a positive PaM, being non-specific fibrotic Interstitial Pneumonia with Organizing Pneumonia being the most frequent manifestation. There was no significant association between imaging manifestations and specific antibodies. ANA 1/80 was found in 6.7% of the cases, which was not associated with an increased risk of PID. Conclusions: There is association between several CTEs and EPIs. It is necessary to highlight the importance of the clinical findings to establish an adequate index of suspicion, in order to timely direct the complementary study (eg: PaM), and the eventual specific therapy.

Hospital Dermatology: analysis of dermatological consultations in a tertiary teaching hospital

Abstract Background In hospital settings, dermatology can offer substantial clinical support for the diagnosis and management of skin conditions, reducing morbidity and mortality. Thus, the study aimed to analyze the profile of referrals and consultations performed by the Dermatology Service of the Santa Casa de Misericordia de Porto Alegre, from August 2018 to January 2020. Methods This study is descriptive, quantitative, and retrospective, conducted through data collection and review of medical records and referrals. The variables included were clinical data of referrals, in-patients profiles, dermatological diagnoses, complementary exams, therapeutic conduct, and recommended follow-ups. Results A total of 1020 referrals were analyzed, which resulted in 641 consultations (328 men, 313 women). The most prevalent skin disease groups were 'Dermatitis and Eczema' (33.1%) and 'Other infectious skin diseases (21.8%), while the most frequent ICD-10 were 'Drug eruptions - L27' (9.9%) and 'Other and unspecified dermatitis - L30' (6.6%). Corticoids were the most recommended treatments (27.7%), followed by antifungals (13.1%). 'Consultation Discharge' (44%) and 'Outpatient' Dermatology follow-up (27%) were the most frequent causes for ending consultation. Study limitations Among the study limitations, the authors highlight its retrospective nature, with data analysis based on referrals and medical records, which may present inaccurate or incomplete information. In addition to this, the study may demonstrate a certain degree of subjectivity due to the review and interpretation process conducted by the researchers. However, the definition of objective criteria based on previous studies attenuates such possible bias. Furthermore, considering that the Dermatology teams are composed of a preceptor dermatologist and residents, the established diagnoses were not submitted to third-party verification, except in the cases of skin biopsies and cultures. Thus, the professional's experience and skills may have influenced the dermatological diagnoses. Conclusions These findings underlie the importance of Dermatology in hospital assistance, contributing to the management of a wide range of skin conditions.

Research progress on the tunnel technique for root coverage / 口腔疾病防治

@#Gingival recessions (GRs) result in root hypersensitivity, root surface caries, and esthetic problems. Various root coverage surgeries are being developed for periodontal plastic therapy. The tunnel technique (TUN) is one of the most widely applied surgeries due to its features of being minimally invasive, practical, excellent outcomes and long-term stability; however, there are still some limitations of this technique. The history and evolution from the envelope flap to TUN, including its efficiency when compared with coronally advanced flaps with a connective tissue graft (CTG), are reviewed in this paper. The limitations of TUN are discussed in consideration of our clinical experience; for example, there is high technique sensitivity when TUN is applied in GR>5 mm because of the great difficulty in covering the grafts. The advantages of surgical access, including vertical incisions in the vestibule, “W” type and pinhole access, are discussed for different situations. Mattress sutures and sling sutures in a single tooth or multiple teeth are applied in TUN. The different types of grafts, such as CTG, platelet-rich plasma, articular dermal matrix and xenogeneic collagen matrix, are described. Mechanical, chemical and biological conditioning of the root surface are recommended during surgery. Protecting the surgical area and taking antibiotics postoperatively are also very important. Finally, the modifications when TUN is applied with other kinds of techniques are discussed, including lateral closed TUN, laterally positioned flaps, double papilla flaps and frenuloplasty. Minimally invasive, esthetic, long-term stability and simplified techniques are the development trends of TUN in the future.

Two consecutive pregnancies of a woman with severe pulmonary arterial hypertension associated with connective tissue disease / 中华围产医学杂志

Pulmonary arterial hypertension (PAH) is an abnormal remodeling of the pulmonary vascular wall due to various causes, resulting in severe cardiovascular disease characterized by increased pulmonary vascular resistance and pressure. The mortality and morbidity of pregnant women with PAH are extremely high. This article reports a woman with severe PAH associated with connective tissue disease who developed cardiac arrest, PAH crisis, and right heart failure during her two consecutive pregnancies without regular prenatal examination. After multidisciplinary consultation and extracorporeal membrane oxygenation, effective cardiopulmonary support was timely, and the patient was finally discharged from the hospital in stable condition. After ten months of follow-up, the mother and child both had good outcomes. Although the mother and her child were survived, severe PAH is a contraindication for pregnancy due to its severely harmful effect on endangering maternal and fetal health.

Subacute combined degeneration of spinal cord with autoimmune disease in children: a case report / 中华神经科杂志

The clinical manifestations of subacute combined degeneration of spinal cord (SCD) in children are complex and vary greatly. Due to the fact that some patients with SCD may be complicated with autoimmune diseases, the high early misdiagnosis and missed diagnosis rates are observed. One case of 13-year old female with severe anemia, multiple joint swelling and pain in left limbs and paralysis of bilateral lower limbs with the extremely low level of serum vitamin B12 and poly-glandular involvement as well as a variety of positive auto-antibodies (anti-intrinsic factor antibody, anti-parietal cell antibody, thyroid peroxidase antibody, thyroid globulin antibody and perinuclear anti-neutrophil cytoplasmic antibody) was retrospectively analyzed. The patient was diagnosed as SCD with autoimmune disease (undifferentiated connective tissue disease and autoimmune polyglandular syndrome). The patient′s condition gradually alleviated after high-dose intravenous methylprednisolone, immunoglobulin, naproxen (then changed to hydroxychloroquine 1 month later), vitamin B12 and levothyroxine sodium tablets supplementation, blood transfusion and rehabilitation. SCD with autoimmune diseases is rare in children, and the clinical manifestations vary greatly. Early recognition and early treatment can improve the prognosis of SCD. The clinical data of this child were retrospectively analyzed, so as to improve the understanding of the disease by clinicians.

Analysis of clinical and pathological features of 54 adult inpatients with erythema nodosum / 中华皮肤科杂志

Objective:To investigate clinical and histopathological features of adult erythema nodosum (EN) .Methods:Clinical data were collected from 54 adult inpatients with histopathologically confirmed EN in Department of Dermatology and Venereology, the First Affiliated Hospital of Wannan Medical College from November 2019 to July 2022, and analyzed retrospectively.Results:Among the 54 EN patients, there were 6 males and 48 females, their ages were 42.50 ± 11.68 years (range, 18 - 73 years), and their disease course ranged from 1 day to 10 years; 30 patients (55.56%) were diagnosed with idiopathic EN, and 24 (44.44%) with secondary EN. The most common etiological factor in secondary EN was infection (17 cases), including respiratory tract infection (9 cases), tuberculosis infection (6 cases), upper respiratory tract infection comorbid with active hepatitis B virus infection (2 cases) ; the following common etiological factor was connective tissue disease (7 cases), including Behcet′s syndrome (4 cases), Sj?gren′s syndrome (1 case), and undifferentiated connective tissue diseases (2 cases). The patients′ ages were significantly younger in the secondary EN group (38.33 ± 12.15 years) than in the idiopathic EN group (46.17 ± 10.20 years, t = 2.58, P = 0.013). All patients had skin lesions on their lower limbs, lesions were limited to both lower limbs in 24 patients with idiopathic EN and 12 with secondary EN, and the proportion of patients with lesions limited to both lower limbs was significantly lower in the secondary EN group than in the idiopathic EN group ( χ2 = 5.44, P = 0.020). Compared with the idiopathic EN group, the secondary EN group showed significantly increased white blood cell counts ([7.56 ± 2.46] × 10 9/L vs. [6.04 ± 1.60] × 10 9/L, t = 2.62, P < 0.05) and C-reaction protein levels (34.34 ± 46.48 mg/L vs. 11.45 ± 18.13 mg/L, t = 2.28, P < 0.05). In the idiopathic EN group, 23 patients mainly showed histopathological features of septal panniculitis, while 17 patients in the secondary EN group mainly showed histopathological features of mixed panniculitis or lobular panniculitis, and the proportion of patients with histopathological features of mixed panniculitis or lobular panniculitis was significantly higher in the secondary EN group than in the idiopathic EN group ( χ2 = 12.18, P < 0.001) . Conclusion:EN was more common in female adults; idiopathic EN was the most common type, and secondary EN may be a cutaneous sign of systemic diseases; for EN patients at a relatively young age, with lesions involving both lower limbs or more sites, higher white blood cell counts and C-reaction protein levels, and histopathological manifestations of lobular panniculitis, systemic examinations were required to rule out underlying causes.

Clinical classification and histopathological features of connective tissue nevi / 中华皮肤科杂志

Connective tissue nevi (CTN) , a kind of benign skin hamartomas, can be classified into 3 types according to the excessive components predominating in skin lesions, including collagen type, elastin type and proteoglycan type, and each type of CTN includes various inherited and acquired diseases. Therefore, genetic, clinical, and histopathological features should be considered for the confirmation of diagnosis of CTN and its subtypes. According to the latest Chinese and international literature, this review elaborates clinical classification and histopathological characteristics of CTN, aiming to further strengthen the understanding of this disease.

Clinical features of patients with connective tissue disease combined with sarcoidosis / 中华风湿病学杂志

Objective:To investigate the clinical features of patients with coexisting connective tissue disease (CTD) and sarcoisosis and to avoid misdiagnosis and mistreatment.Methods:To analyze the clinical manifestations, laboratory data, imaging and pathological features of patients with CTD combined with sarcoidosis in Peking Union Medical College Hospital from January 1985 to December 2021.Results:There were 17 patients with CTD(including 10 SS, 2 DM, 2 PBC, 1 SLE, 1 RA and 1 UCTD), combined with sarcoidosis, with a mean age of (55±10) years old and the ratio of male-to-female was 1:16. Eight patients were diagnosed as CTD before sarcoidosis, while 3 patients after sarcoidosis. The other 6 patients were diagnosed with the two diseases almost simultaneously. Lymphadenopathy(12/17), pulmonary nodules (8/17), subcutaneous nodules (4/17), rash (4/17) and blurred vision (1/17) were the main manifestations of patients with the onset of nodular disease. Nine patients were treated based on the presentation of sardoisis and 5 patients for CTD; 3 patients were treated for both diseases at the same time. All 17 patients discharged with improvement after treatment.Conclusion:When sarcoidosis do coexists with CTD, occult CTD might occur. It is important to investigate specific manifestations including pathological features of sarcoidosis and differentiate it from CTD.

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.

Expression of serum connective tissue growth factor, glyoxalase Ⅰ, pyruvate kinase M2 in endometrial carcinoma and its relationship with clinicopathological features / 中国医师进修杂志

Objective:To explore the expression of serum connective tissue growth factor (CTGF), glyoxalase Ⅰ (GLO-I) and pyruvate kinase M2 (PKM2) in endometrial cancer and their relationship with clinicopathological characteristics.Methods:A total of 96 endometrial cancer patients in Yuechi County People's Hospital from February 2015 to February 2017 were selected as the research group, 48 patients with endometrial hyperplasia during the same period were selected as the benign control group, and 48 patients with healthy physical examination during the same period were selected as the healthy control group. The serum levels of CTGF, GLO-Ⅰ, and PKM2 in the three groups were analyzed. The correlation between serum levels of CTGF, GLO-Ⅰ and PKM2 in the research group was analyzed, and the relationship between each serum index and clinicopathological characteristics was analyzed.Results:The levels of serum CTGF, GLO-Ⅰ and PKM2 in the research group were higher than those in the benign control group and healthy control group: (184.31 ± 37.14) μg/L vs. (110.45 ± 20.59), (17.28 ± 0.42) μg/L; (95.17 ± 16.56) pmol/L vs. (56.29 ± 10.14), (9.08 ± 0.66) pmol/L; (20.25 ± 6.13) μg/L vs. (13.11 ± 4.58), (9.05 ± 2.74) μg/L; and the levels of serum CTGF, GLO-Ⅰ and PKM2 in the benign control group were higher than those in the healthy control group, there were statistical differences ( P<0.05). The results of Pearson correlation analysis showed that the level of CTGF had positive correlation with GLO-Ⅰ and PKM2 ( r = 0.713, 0.741, P<0.05), and the level of GLO-Ⅰ had positive correlation with PKM2 ( r = 0.823, P<0.05). The results of Spearman correlation analysis showed that the levels of CTGF, GLO-Ⅰ, PKM2 had positive correlation with FIGO stage ( r = 0.609, 0.704, 0.721; P<0.05), myometrial invasion depth ( r = 0.753, 0.695, 0.719; P<0.05), lymph node metastasis ( r = 0.776, 0.744, 0.640; P<0.05); had negative correlation with the degree of differentiation ( r = - 0.711, - 0.720, - 0.668; P<0.05). Conclusions:Serum CTGF, GLO-I, PKM2 expression levels are abnormally elevated in patients with endometrial cancer, which are significantly related to multiple clinicopathological characteristics.

Analysis of pregnancy outcomes, disease progression, and risk factors in patients with undifferentiated connective tissue disease / 北京大学学报(医学版)

OBJECTIVE@#To investigate the fetal and maternal outcomes, risk factors of disease progression and adverse pregnancy outcomes (APOs) in patients with undifferentiated connective tissue disease (UCTD).@*METHODS@#This retrospective study described the outcomes of 106 pregnancies in patients with UCTD. The patients were divided into APOs group (n=53) and non-APOs group (n=53). The APOs were defined as miscarriage, premature birth, pre-eclampsia, premature rupture of membranes (PROM), intrauterine growth restriction (IUGR), postpartum hemorrhage (PPH), and stillbirth, small for gestational age infant (SGA), low birth weight infant (LBW) and birth defects. The differences in clinical manifestations, laboratory data and pregnancy outcomes between the two groups were compared. Logistic regression analysis was performed to analyze the risk factors for APOs and the progression of UCTD to definitive CTD.@*RESULTS@#There were 99 (93.39%) live births, 4 (3.77%) stillbirths and 3 (2.83%) miscarriage, 20 (18.86%) preterm delivery, 6 (5.66%) SGA, 17 (16.03%) LBW, 11 (10.37%) pre-eclampsia, 7 (6.60%) cases IUGR, 19 (17.92%) cases PROM, 10 (9.43%) cases PPH. Compared with the patients without APOs, the patients with APOs had a higher positive rate of anti-SSA antibodies (73.58% vs. 54.71%, P=0.036), higher rate of leukopenia (15.09% vs. 3.77%, P=0.046), lower haemoglobin level [109.00 (99.50, 118.00) g/L vs. 124.00 (111.50, 132.00) g/L, P < 0.001].Multivariate Logistic regression analysis showed that leucopenia (OR=0.82, 95%CI: 0.688-0.994) was an independent risk factors for APOs in UCTD (P=0.042). Within a mean follow-up time of 5.00 (3.00, 7.00) years, the rate of disease progression to a definite CTD was 14.15%, including 8 (7.54%) Sjögren's syndrome, 4 (3.77%) systemic lupus erythematosus (SLE), 4 (3.77%) rheumatoid arthritis and 1 (0.94%) mixed connective tissue disease. Multivariate Cox proportional risk regression analysis showed that Raynaud phenomenon (HR=40.157, 95%CI: 3.172-508.326) was an independent risk factor for progression to SLE.@*CONCLUSION@#Leukopenia is an independent risk factor for the development of APOs in patients with UCTD. Raynaud's phenmon is a risk factor for the progression of SLE. Tight disease monitoring and regular follow-up are the key measures to prevent adverse pregnancy outcomes and predict disease progression in UCTD patients with pregnancy.