Patrones tomográficos de neumonía en organización. Serie de casos y revisión de la literatura / Tomographic patterns of organizing pneumonia. Case series and review

Resumen La neumonía en organización es un tipo de enfermedad pulmonar intersticial difusa que puede ser idiopática (criptogénica) o secundaria a numerosas etiologías, y se asocia con hallazgos clínicos y de laboratorio inespecíficos. Su diagnóstico y tratamiento exigen un equipo interdisciplinario, en el que las imágenes desempeñan un papel indispensable. Se presenta una serie de nueve casos, haciendo énfasis en las características clínicas y profundizando en los diversos patrones imagenológicos identificados. También se aporta una revisión de las variantes recientemente descritas.

Abstract Organizing pneumonia is a type of diffuse interstitial lung disease that can be idiopathic (cryptogenic) or secondary to numerous etiologies, and is an entity associated with nonspecific clinical and laboratory findings. Its diagnosis and treatment require an interdisciplinary team in which images play an indispensable role. The presentation of a series of nine cases is made, emphasizing the clinical characteristics and delving into the various identified imaging patterns. A review of the recently described variants is also provided.

Oxigenoterapia domiciliaria y capacidad aeróbica en pacientes con enfermedad pulmonar intersticial difusa. Estudio transversal / Home Oxygen Therapy And Aerobic Capacity In Patients With Diffuse Interstitial Lung Disease. Cross-Sectional Study

Introducción: La enfermedad pulmonar intersticial difusa es un grupo de enfermedades que causan un trastorno de la capacidad aeróbica y calidad de vida, además, ocasionan una gran tasa de morbimortalidad para esta población. El uso de oxigenoterapia domiciliaria mayor a 15 horas diarias tiene beneficios en pacientes hipoxémicos crónicos, sin embargo, poco se ha comparado su uso con pacientes que no lo reciben. Objetivo: Describir las características clínicas, capacidad aeróbica funcional y calidad de vida relacionada con la salud de dos grupos de pacientes con enfermedad pulmonar intersticial difusa, uno con indicación de oxigenoterapia domiciliaria y otro grupo sin indicación. Métodos: Estudio descriptivo transversal, que incluyó 41 pacientes con enfermedad pulmonar intersticial difusa que firmaron consentimiento informado. En ambos grupos, características demográficas y clínicas, ansiedad/depresión, calidad de vida relacionada con la salud con el cuestionario Saint George y la capacidad aeróbica funcional con la prueba de marcha de seis minutos fueron medidas. Se compararon los grupos con la prueba t de student para muestras independientes. Resultados: El grupo enfermedad pulmonar intersticial difusa con oxigenoterapia domiciliaria presentó mayor porcentaje de antecedente de tabaquismo (p = 0,041), menor distancia caminada en la prueba de marcha de seis minutos (304,1 ± 108,7 vs. 390,3 ± 95,6 p = 0,01), y menor porcentaje de la distancia predicha (58,37 ± 20,45 vs. 73,34 ± 22,90, p = 0,034) frente al grupo enfermedad pulmonar intersticial difusa sin oxigenoterapia domiciliaria. Conclusiones: Los pacientes con enfermedad pulmonar intersticial difusa con indicación de oxigenoterapia domiciliaria presentan menor capacidad aeróbica funcional comparada con pacientes sin indicación(AU)

Introduction: Diffuse interstitial lung disease is a group of diseases that cause a disorder of aerobic capacity and quality of life; in addition, they cause a high rate of morbidity and mortality for this population. The use of home oxygen therapy greater than 15 hours a day has benefits in chronic hypoxemic patients, however, little has been compared to patients who do not receive it. Objective: Describe the clinical characteristics, functional aerobic capacity and health-related quality of life of two groups of patients with diffuse interstitial lung disease, one with indication for home oxygen therapy and another group without indication. Methods: A cross-sectional descriptive study included 41 patients with diffuse interstitial lung disease who signed informed consent. In both groups, demographic and clinical characteristics, anxiety/depression, health quality of life related with the Saint George questionnaire, and functional aerobic capacity with the six-minute gait test were measured. The groups were compared with the student's t-test for independent samples. Results: The diffuse interstitial lung disease group with home oxygen therapy presented a higher percentage of smoking history (p = 0.041), a shorter distance walked in the six-minute gait test (304.1 ± 108.7 vs. 390.3 ± 95.6 p = 0.01), and a lower percentage of the predicted distance (58.37 ± 20.45 vs. 73.34 ± 22.90, p = 0.034) compared to the diffuse interstitial lung disease group without home oxygen therapy. Conclusions: Patients with diffuse interstitial lung disease with indication of home oxygen therapy have lower functional aerobic capacity compared to patients without indication(AU)

Prevalence of latent tuberculosis infection among patients with interstitial lung disease requiring immunosuppression / Prevalência de infecção latente por tuberculose em pacientes com doença pulmonar intersticial com necessidade de imunossupressão

ABSTRACT Objective To characterize the prevalence of latent tuberculosis infection (LTBI) in patients with interstitial lung diseases (ILDs) requiring immunosuppression. Only 5 to 10% of individuals infected with Mycobacterium tuberculosis develop tuberculosis, and certain groups of patients have an increased risk of illness, such as the immunocompromised. Patients with ILDs are frequently treated with immunosuppressants and, therefore, might have a higher risk of developing the disease. Methods Prospective study conducted at the ILD reference center of the Federal University of Paraná from January 2019 to December 2020. The screening of LTBI was performed with the use of the tuberculin skin test (TST). Results The sample consisted of 88 patients, of whom 64.8% were women, with a mean age of 61.4 years. The most frequent diagnoses were autoimmune rheumatic disease ILD (38.6%) and hypersensitivity pneumonitis (35.2%). The most common immunosuppressant in use at the time of the TST was prednisone, either in combination with mycophenolate (19.3%) or alone (17.1%). The majority of participants had fibrotic lung disease, characterized by a reticular interstitial pattern on chest computed tomography (79.5%) and moderate to severe functional impairment (mean FVC 69.2%). A prevalence of LTBI of 9.1% (CI 95%, 2.1%-15.1%) was found, with a TST median of 13. Conclusion Patients with ILD who are treated with immunosuppressants are not commonly screened for LTBI, despite being under a greater risk of progression to active disease. This study suggests the need for a more cautious approach to these patients.

RESUMO Objetivo Caracterizar a prevalência de Infecção Latente por Tuberculose (ILTB) em pacientes com Doenças Pulmonares Intersticiais (DPIs) que necessitam de imunossupressão. Apenas 5 a 10% dos indivíduos infectados pelo Mycobacterium tuberculosis desenvolvem tuberculose, sendo que certos grupos de pacientes apresentam maior risco de doença, tais como os imunocomprometidos. Pacientes com DPIs são frequentemente tratados com imunossupressores, portanto, podem apresentar maior risco de desenvolver a doença. Métodos Estudo prospectivo conduzido no Centro de Referência para DPI da Universidade Federal do Paraná (UFPR), entre Janeiro de 2019 e Dezembro de 2020. O rastreio de ILTB foi realizado por meio da Prova Tuberculínica (PT). Resultados A amostra foi composta por 88 pacientes, dos quais 64,8% eram mulheres, com, em média, 61,4 anos de idade. Os diagnósticos mais frequentes foram DPI associada a doença reumática autoimune (DRAI) (38,6%) e pneumonite de hipersensibilidade (35,2%). Prednisona foi o imunossupressor mais comumente utilizado à época da PT, em combinação com micofenolato (19,3%) ou isoladamente (17,1%). A maioria dos participantes tinha doença pulmonar fibrótica, caracterizada por infiltrado reticular em tomografia computadorizada de tórax (79,5%), bem como comprometimento funcional moderado a grave (Capacidade Vital Forçada (CVF) média de 69,2%). Observou-se uma prevalência de ILTB de 9,1% (Intervalo de Confiança (IC) 95%, 2,1%-15,1%), com mediana da PT de 13. Conclusão Não é comum que pacientes com DPI tratados com imunossupressores sejam avaliados quanto à presença de ILTB, apesar de estarem sob um maior risco de progressão para doença ativa. Este estudo sugeriu a necessidade de uma abordagem mais cuidadosa em relação a esses pacientes.

Enfermedad intersticial pulmonar secundaria a infección por SARS-CoV-2 en paciente con artritis reumatoide

RESUMEN La presentación clínica de COVID-19 es heterogénea, desde casos asintomáticos, hasta síndrome de dificultad respiratoria aguda y muerte. La enfermedad grave puede ocurrir en pacientes con comorbilidades, incluidos pacientes con enfermedades reumáticas sistémicas. El impacto de la artritis reumatoide en pacientes que cursan con COVID-19 aún no está claro. Presentamos el caso de un paciente con antecedente de AR, quien cursó con COVID-19, y reingresó con insuficiencia respiratoria aguda y hallazgos tomográficos de enfermedad intersticial pulmonar de rápida evolución.

ABSTRACT The clinical presentation of COVID-19 is heterogeneous. It ranges from asymptomatic cases to acute respiratory distress syndrome and death. Severe disease can occur in patients with comorbidities, including patients with systemic rheumatic diseases. The impact of rheumatoid arthritis in patients with COVID-19 is not yet clear. We present the case of a patient with a history of rheumatoid arthritis who had COVID19 and was readmitted with respiratory difficulties and tomographic findings that showed rapidly evolving interstitial lung disease.

Diagnosis of Diffuse Interstitial Lung Diseases: BAL Study - Its Utility for the Diagnosis

Abstract Introduction: Diffuse interstitial lung diseases are a hard-to-diagnose heterogeneous group of respiratory diseases. The study of bronchoalveolar lavage through flow cytometry may define typical cell patterns in different diseases and so help confirm the differential diagnosis. The purpose of this study was to retrospectively analyze the clinical utility of cell and lymphocyte subpopulations detected in the bronchoalveolar lavage by flow cytometry in order to define typical cell patterns that allow for making a differential diagnosis of granulomatous lung diseases. Materials and methods: The retrospective study included 44 patients. The subjects were diagnosed with sarcoidosis or hypersen sitivity pneumonitis during a period of 3 years. We performed the cellular analysis of bronchoalveolar lavage through flow cytometry and histological and imaging testing (HRCAT, High Resolution Computed Axial Tomography) as part of the diagnosis. The percentages of T cells, B cells, NK cells, CD4, CD8 and CD4/CD8 were analyzed by flow cytometry for the following markers: CD3 +, CD19 + CD4 +, CD8 +, CD3 + CD4-CD8- and CD3 + CD16-CD56-. Results: We conclude that the most important parameters were lymphocytosis and especially the CD4/CD8 quotient. This quotient was high for diseases such as sarcoidosis and low for hypersensitivity pneumonitis, in comparison with the values found in the peripheral blood. Conclusions: The BAL (Bronchoalveolar Lavage) study is useful for differentiating between granulomatous interstitial lung diseases and other DILDs (diffuse interstitial lung diseases).

Interstitial lung disease due to anti-TNF use in the treatment of psoriasis

Abstract Psoriasis is a chronic inflammatory disease that affects the skin variably, according to genetic and environmental factors. Some patients may benefit from systemic treatment with immunobiological agents, drugs that can be accompanied by several adverse effects. A case of a 58-year-old patient undergoing treatment for psoriasis with adalimumab for five years is reported. Alterations compatible with interstitial pneumonia were detected with important regression after adalimumab discontinuation. This case is relevant due to the scarcity of reports on late pulmonary adverse effect of anti-TNF treatment of psoriasis.

Clinical characteristics and related factors of systemic lupus erythematosus with interstitial pneumonia / 北京大学学报(医学版)

OBJECTIVE@#To investigate the clinical features, radiologic scores and clinically relevant risk factors prognosis of secondary interstitial lung disease (ILD) in patients with systemic lupus erythematosus (SLE).@*METHODS@#In this study, 60 SLE patients in Department of Rheumatology of the First Affiliated Hospital of Baotou Medical College and Taizhou First People's Hospital from January 2015 to March 2019 were retrospectively analyzed. All of those 60 patients with SLE underwent lung high resolution computed tomography (HRCT) examination. We used a 1 ∶1 case-control study. There was a matching of age and gender between the two groups. Thirty patients with SLE related ILD (SLE-ILD) were in the case group, and 30 patients with SLE without ILE (SLE non-ILD) were in the control group. The clinical features, pulmonary function test, radiologic characteristic of SLE patients were collected and were used to analyze SLE-ILD.@*RESULTS@#In this study, we reached the following conclusions: First, there were statistically significant differences in chest tightness/shortness of breath, Raynaud's phenomenon, and Velcro rale between SLE-ILD and SLE non-ILD patients (both P < 0.05); Second, hemoglobin (Hb) and albumin (ALB) in the patients of SLE-ILD had a significant decrease compared with the patients of SLE non-ILD. Blood urea nitrogen (BUN), erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) increased in SLE-ILD patients compared with SLE non-ILD patients, the difference had statistical significance (P < 0.05); Third, for SLE-ILD patients, the most common type was non-specific interstitial pneumonia (NSIP), followed by usual interstitial pneumonia and lymphocytic interstitial pneumonia; Fourth, there was no significant difference in clinical-radiology-physiology scores between the different ILD types (P>0.05), similarly, the lung HRCT score and lung function between different ILD types had no significant difference (P>0.05); Fifth, multivariate Logistic regression analysis showed that decreased albumin and chest tightness/shortness of breath might be the risk factor for SLE-ILD.@*CONCLUSION@#There are statistically significant differences between the SLE-ILD group and SLE non-ILD group in terms of chest tightness/shortness of breath, Velcro rale and Raynaud's phenomenon. Decreased albumin and chest tightness/shortness of breath in SLE patients should be alerted to the occurrence of ILD. NSIP is the most common manifestation of SLE-ILD.

Síndrome de Caplan: la paradoja del pulmón como origen de la artritis reumatoide / Caplan syndrome: the paradox of the lung as the origin of rheumatoid arthritis

Resumen Históricamente, los estudios de campo observacionales han sido el punto de partida para el desarrollo de grandes avances en el entendimiento de las enfermedades autoinmunes. En el caso de la artritis reumatoide (AR), se han descrito varias asociaciones clínicas hasta la actualidad que tienen en común modelos inmunológicos transversales a la historia natural de la misma, lo que ha permitido avanzar en el desarrollo de nuevos objetivos terapéuticos. Se pretende hacer una breve descripción del compromiso nodular en AR, partiendo de las observaciones clínicas del Dr. Anthony Caplan en pacientes con riesgo de neumoconiosis, con el fin de reconocer el valor de este tipo de asociaciones en el ejercicio médico profesional.

Historically, observational field studies have been the starting point for the development of great advances in the understanding of autoimmune diseases. In the case of rheumatoid arthritis (RA), several clinical associations have been described to date which have in common immunological models transverse to its natural history, which has allowed progress in the development of new therapeutic objectives. Our aim is to make a brief description of nodular involvement in RA, based on the clinical observations of Dr. Anthony Caplan in patients at risk of pneumoconiosis, in order to recognize the value of this type of association in professional medical practice.

Compromiso pulmonar en síndrome de Sjögren / Pulmonary involvement in Sjögren's syndrome

RESUMEN El síndrome de Sjögren es una enfermedad autoinmunitaria sistémica con un alto impacto individual y social. El compromiso pulmonar presenta múltiples manifestaciones, con impacto en calidad de vida y riesgo de mortalidad. El abordaje dinámico integrado mediante un grupo de diagnóstico multidisciplinario que incluya expertos en neumología, reumatología, radiología y patología tiene el potencial de impactar en la identificación, las estrategias de manejo y los desenlaces. Aunque es necesario reconocer tempranamente a los pacientes con mayor riesgo, en la actualidad no se cuenta con biomarcadores confiables. Las estrategias de manejo farmacológico se basan en la inmunomodulación, pero la evidencia para su uso es de baja calidad. Promover el entrenamiento y la sensibilización del personal de salud podría reducir los retrasos en el acceso a una evaluación especializada.

ABSTRACT Sjögren's syndrome is a systemic autoimmune disease with a high burden for the individual, as well as society. Pulmonary compromise presents with a myriad of manifestations that influence patient quality of life and mortality risk. An integrated dynamic approach by a multidisciplinary diagnostic discussion team that includes experts in chest diseases, rheumatology, radiology, and pathology has the potential to improve the identification, management strategies, and outcomes. Although early recognition of patients at high risk is essential, there is currently a lack of reliable biomarkers. Pharmacological therapies are based on immunomodulation, although the evidence to support their use is of low quality. Increasing awareness and training among healthcare professionals may reduce a delayed access to specialized assessment.

Diagnosis and treatment of interstitial lung disease: focusing on idiopathic pulmonary fibrosis

Interstitial lung disease (ILD) is a rare condition characterized by extensive inflammation and fibrosis mainly involving the pulmonary interstitium or alveoli. Usually, patients with ILD clinically present with chronic cough and exertional dyspnea. ILD is classified into subtypes based on clinical characteristics, detailed history obtained from patients, and radiological, and/or histopathological features. The most common type of idiopathic interstitial pneumonia is idiopathic pulmonary fibrosis (IPF). IPF is a chronic progressive fibrosing ILD and is associated with poor prognosis. An exclusive diagnosis of IPF requires no known condition causing ILD and typical radiological and/or histopathological features of lung fibrosis. Fibrosis observed in this condition is attributable to repetitive epithelial injury with consequent abnormal wound healing in genetically susceptible and elderly individuals. Currently, pirfenidone and nintedanib are useful disease-modifying agents available to treat IPF. In this article, we review the concept, diagnosis, clinical course, and treatment of ILD.

Pharmacological treatment of pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a condition that has been described as alveolar collapse and thickening, which correlate with dysregulated surfactant production and injury to type 2 alveolar cells. As resolution of chest computed tomography has improved, especially with the development of high-resolution computed tomography (HRCT), the diagnostic measures adopted for pulmonary fibrosis has gradually shifted from biopsy to HRCT. This shift towards HRCT has aided in diagnostic evaluation and detection of the therapeutic and adverse effects of drugs for pulmonary fibrosis. Further, after the endpoint was changed to forced vital capacity, significant improvements are being observed in clinical trial outcomes. Currently active clinical trials are replacing lung biopsy with HRCT. In 2014, pirfenidone and nintedanib gained approval for tandem use in patients with IPF. These drugs were found to not only reduce the progression of pulmonary fibrosis, but also the acute exacerbation and mortality associated with the condition. These drugs showed consistent benefits regardless of the severity of patients' symptoms. Additionally, both nintedanib and pirfenidone were found to be effective in patients with advanced pulmonary fibrosis that was not classified as IPF. Nintedanib has been shown to reduce forced vital capacity in interstitial lung diseases associated with systemic sclerosis. In the next three to five years, many changes in treatment are expected, not only for IPF, but also for the entire spectrum of pulmonary fibrotic diseases. Pirfenidone and nintedanib are now considered standard treatments for IPF and few other fibrotic lung diseases. Clinicians treating patients with pulmonary fibrosis should keep themselves updated with the results of clinical trials that are currently underway.

Neumonitis por docetaxel, discusión de un caso / Interstitial pneumonitis due to docetaxel

Resumen La neumonitis intersticial por docetaxel es un evento infrecuente, pero con alto potencial de mortalidad y puede ser potencialmente reversible con el uso de esteroides sistémicos; se considera importante en el diagnóstico diferencial del paciente bajo quimioterapia con síntomas respiratorios. A continuación, se presenta un caso de neumonitis fatal por docetaxel en un paciente con carcinoma de próstata avanzado. Paciente de 80 años de edad con historia de cáncer de próstata de 15 años de evolución, en progresión ósea y ganglionar, y que inició manejo con docetaxel. Después de la aplicación del tercer ciclo presenta disnea de esfuerzos, fiebre, tos seca y deterioro general. Se realizó diagnóstico de neumonitis por docetaxel y se descartaron otras entidades. La neumonitis intersticial por docetaxel es una entidad rara. Los pilares del tratamiento son la suspensión del medicamento, el soporte ventilatorio y el uso de glucocorticoides sistémicos.

Abstract Interstitial pneumonitis due to docetaxel is an infrequent event but with a high mortality potential; it can be potentially reversible with the use of systemic steroids and is considered important in the differential diagnosis of the patient undergoing chemotherapy with respiratory symptoms. We present a case of fatal pneumonitis due to docetaxel in a patient with advanced prostate carcinoma. An 80-year-old patient with a history of prostate cancer of15 years of evolution, with disease progression in bones and lymph nodes, in whom treatment with Docetaxel was started, after the application of the third cycle, he presented with dyspnea on exertion, fever, dry cough and general deterioration. A diagnosis of pneumonitis due to docetaxel was made, discarding other entities. Interstitial pneumonitis due to docetaxel is a rare entity. The pillars of the treatment are the suspension of the medication, the ventilatory support and the use of systemic glucocorticoids.

Inhibidor de Janus quinasas como opción terapéutica en artritis reumatoide y enfermedad pulmonar intersticial asociada: reporte de 4 casos / Janus kinase inhibitors as a therapeutic option in rheumatoid arthritis and associated interstitial lung disease. Report of four cases

RESUMEN La artritis reumatoide (AR) es una enfermedad sistémica que en las últimas décadas ha tenido múltiples opciones terapéuticas. Con la información disponible en la literatura no se recomienda el uso de terapia biológica en pacientes con enfermedad pulmonar difusa, dado que estos medicamentos pueden exacerbar el compromiso pulmonar. Un medicamento de más reciente aparición es el inhibidor de la enzima Janus quinasa, el cual es una opción terapéutica en monoterapia o combinado para pacientes con AR en moderada y alta actividad de la enfermedad, con contraindicación al uso de FARME sintéticos o biológicos, y en pacientes con fallo terapéutico, no obstante, su seguridad a nivel pulmonar no ha sido evaluada en ensayos clínicos y la información disponible es escasa. Describimos el tratamiento, seguimiento y resultados del uso de tofacitinib en 4 pacientes con enfermedad pulmonar secundaria a AR en una serie de casos.

ABSTRACT Rheumatoid arthritis (RA) is a systemic disease for which multiple therapeutic options have been developed in the last decades. Based on the information available in the literature, the use of biological therapy in patients with diffuse lung disease is not recommended because these medications can exacerbate the lung disease. A newer drug is the Janus kinase enzyme inhibitor, which can be used as monotherapy or in combination in patients with moderate to high activity RA, in whom the use of synthetic or biological DMARDs is contraindicated, as well as in patients with therapeutic failure. However, the pulmonary safety of the drug has not been evaluated in clinical trials and the information available is limited. This article discusses the treatment, follow-up, and outcomes of the use of tofacitinib in a series of 4 patients with lung disease secondary to RA.

Spectrum of HRCT findings in interstitial lung diseases and their correlation with mediastinal lymphadenopathy

Background: Interstitial lung disease (ILD) represents a heterogeneous non-infectious group of acute and chronic diseases affecting the lung parenchyma. ILDs are usually associated with significant morbidity and mortality, particularly when fibrosis occurs. ILD is usually associated with mediastinal lymph node enlargement, the extent of lymph node enlargement may correlate to disease activity or progression of fibrosis. In the present study, authors have correlated the spectrum of high-resolution CT findings in ILDs with mediastinal lymph node enlargements.Methods: One hundred and four cases of ILDs confirmed by HRCT findings and pulmonary function tests were included in this study. HRCT was performed using a GE 128 SLICE CT OPTIMA 660 scanner, USA with 1.5 mm collimation at full inspiration. The findings were classified into three groups-fibrotic, ground glassing and nodular pattern. Authors assessed the presence, number and sites of enlarged lymph nodes (short axis ≥10 mm in diameter).Results: The largest subsets of patients were found in the 51-60 age groups. Fibrotic pattern was the most common pattern in this study (50%) followed by ground glassing (44%). Nodular pattern was the least predominant pattern (6%). Lymphadenopathy was seen in 84% of UIP and 63% cases of NSIP. P value was 0.049 which indicates a significant relation between lymphadenopathy and various ILDs. A strong relationship between lymphadenopathy and the predominant fibrotic pattern followed by ground glassing was observed.Conclusions: A significant association was established between lymphadenopathy and the type of ILD where fibrotic pattern had the maximum association.

Role of HRCT in Smoking Related Interstitial Lung Diseases

Background: Smoking induced lung diseases constitute a complex group of disorders, varying from the well-known entity of Chronic Obstructive Pulmonary Disease (COPD) to the more recently described interstitial lung diseases. Aim and objectives: The aim of the study was to delineate the relation between cigarette smoking and the development of interstitial lung disease with declaration of the different types of the interstitial lung associated with smoking. Materials and methods: This was prospective study consisting of patients with smoking history referred to Department of Radiodiagnosis of NRIGH for HRCT with symptoms of progressive dyspnea. Inclusion criteria were smokers with progressive dyspnea, (age range 30-90 years, mean age 54 years ± 8). HRCT was done to all subjects using GE Light Speed Multislice 4 channels. CT scan examination was performed using GE Light Speed Multislice 4 channels present in our radiology department. The examination was done in supine position. A scout was taken with kV 120 and mA 120, then helical scanning was done in caudo-cranial direction to minimize respiration artifacts, using detector row 4, helical thickness 1.25, pitch 1.5:1, speed (mm/rot) 7.5, Detector configuration 4 · 1.25, beam collimation 5.00 mm, interval 1.00, gantry tilt 0.0, FOV depends on the patients’ body build, but is about 35 cm, kV 120–140, mA 120–160, total exposure time about 16 s during breath hold in inspiration. The images acquired were sent to a separate workstation to be processed, manipulated and reconstructed. Reconstruction of the images was done using reconstruction software available at the workstation to attain HRCT axial, coronal and sagittal images. Also mediastinal window images were done for each case. Results: In the present study, the most common seen HRCT pattern was Idiopathic Pulmonary Fibrosis, most affected gender were males and most affected age group was between 60-70 years. Sreedevi Meka, D Ankamma Rao. Role of HRCT in Smoking Related Interstitial Lung Diseases. IAIM, 2019; 6(8): 78-94. Page 79 Conclusion: In the appropriate clinical evaluation and in the presence of typical findings, high - resolution CT plays an essential role in evaluation and definite diagnosis of Smoking Related – Interstitial Lung Disease, and this may obviate further testing. However, lung biopsy may be needed when the high-resolution CT are relatively non-specific or when a confident definitive diagnosis is needed.

Imaging in idiopathic pulmonary fibrosis: diagnosis and mimics

Idiopathic pulmonary fibrosis is a chronic disease of unknown etiology that usually has a progressive course and is commonly associated with a poor prognosis. The main symptoms of idiopathic pulmonary fibrosis, including progressive dyspnea and dry cough, are often nonspecific. Chest high-resolution computed tomography is the primary modality used in the initial assessment of patients with suspected idiopathic pulmonary fibrosis and may have considerable influence on subsequent management decisions. The main role of computed tomography is to distinguish chronic fibrosing lung diseases with a usual interstitial pneumonia pattern from those presenting with a non-usual interstitial pneumonia pattern, suggesting an alternative diagnosis when possible. A usual interstitial pneumonia pattern on chest tomography is characterized by the presence subpleural and basal predominance, reticular abnormality honeycombing with or without traction bronchiectasis, and the absence of features suggestive of an alternative diagnosis. Idiopathic pulmonary fibrosis can be diagnosed according to clinical and radiological criteria in approximately 66.6% of cases. Confirmation of an idiopathic pulmonary fibrosis diagnosis is challenging, requiring the exclusion of pulmonary fibroses with known causes, such as asbestosis, connective tissue diseases, drug exposure, chronic hypersensitivity pneumonitis, and other forms of idiopathic interstitial pneumonitis. The histopathological hallmark of usual interstitial pneumonia is a heterogeneous appearance, characterized by areas of fibrosis with scarring and honeycombing alternating with areas of less affected or normal parenchyma. The aim of this article was to review the clinical, radiological, and pathological features of idiopathic pulmonary fibrosis and of diseases that might mimic idiopathic pulmonary fibrosis presentation.

Pathological interpretation of connective tissue disease-associated lung diseases / 영남의대학술지

Connective tissue diseases (CTDs) can affect all compartments of the lungs, including airways, alveoli, interstitium, vessels, and pleura. CTD-associated lung diseases (CTD-LDs) may present as diffuse lung disease or as focal lesions, and there is significant heterogeneity between the individual CTDs in their clinical and pathological manifestations. CTD-LDs may presage the clinical diagnosis a primary CTD, or it may develop in the context of an established CTD diagnosis. CTD-LDs reveal acute, chronic or mixed pattern of lung and pleural manifestations. Histopathological findings of diverse morphological changes can be present in CTD-LDs airway lesions (chronic bronchitis/bronchiolitis, follicular bronchiolitis, etc.), interstitial lung diseases (nonspecific interstitial pneumonia/fibrosis, usual interstitial pneumonia, lymphocytic interstitial pneumonia, diffuse alveolar damage, and organizing pneumonia), pleural changes (acute fibrinous or chronic fibrous pleuritis), and vascular changes (vasculitis, capillaritis, pulmonary hemorrhage, etc.). CTD patients can be exposed to various infectious diseases when taking immunosuppressive drugs. Histopathological patterns of CTD-LDs are generally nonspecific, and other diseases that can cause similar lesions in the lungs must be considered before the diagnosis of CTD-LDs. A multidisciplinary team involving pathologists, clinicians, and radiologists can adequately make a proper diagnosis of CTD-LDs.

Enfermedad pulmonar intersticial difusa: evaluación clínica y funcional previa a un programa de rehabilitación pulmonar / Interstitial Lung Disease: clinical and functional evaluation prior to a pulmonary rehabilitation program

La presencia de enfermedades intersticiales se ha vuelto más frecuente en nuestro medio. La rehabilitación pulmonar es una opción beneficiosa, sin embargo, el comportamiento y estrategias de intervención siguen escasas, justificando su investigación en nuestro país. El objetivo es describir estos pacientes al ingreso de un programa de rehabilitación pulmonar desde la parte sociodemográfica y calidad de vida relacionada con la salud, hasta su parte clínica y funcional. Método: Estudio descriptivo en 41 pacientes que ingresaron a rehabilitación pulmonar, se realizó recolección de datos sociodemográficos, clínicos y farmacológicos; se implementó el test de la marcha de los 6 minutos para valorar la capacidad funcional; la calidad de vida relacionada con la salud fue valorada con St. George's Respiratory Questionnaire y ansiedad y depresión con Hospital Anxiety and Depression Scale. Resultados: Hombres 56,1%. Edad media 57.6±12.9. El hábito de fumar estuvo presente en 39% de los casos con media de 18.97 ± 17.2 paquetes/año. El 66.7% de los pacientes que visitaron urgencias por complicaciones respiratorias fueron hospitalizados con una media de 9.7±14.4 días. La distancia recorrida fue de 348.2±109 metros. Los hombres recorren en promedio 108.8 metros más que las mujeres. Discusión: Se discuten los resultados a la luz del contexto local y regional teniendo en cuenta la disponibilidad y uso de pruebas diagnósticas. Los resultados contrastan con la literatura respecto al sexo de los pacientes en los que predomina con EPID. Los hallazgos son coherentes con el curso de las enfermedades en cuanto a visita a urgencias y hospitalizaciones.

The presence of interstitial diseases has become more frequent in our environment. Pulmonary rehabilitation is a beneficial option. However, the behavior and intervention strategies are still scarce. This justifies the research on these kind of diseases in our country. The objective is to describe the patients' condition upon admission in a pulmonary rehabilitation program considering the socio-demographic and quality of life characteristics related to health as well as the clinical and functional characteristics. Method: Descriptive study made with 41 patients admitted to a pulmonary rehabilitation program. Socio-demographic, clinical and pharmacological data was collected. The 6-Minute Walk Test was used to evaluate the functional capacity. The quality of life related to health was evaluated with St. George's Respiratory Questionnaire and the anxiety, and its relation with depression was evaluated with the Hospital Anxiety and Depression Scale. Results: Men 56.1%. Mean age 57.6 ± 12.9. The habit of smoking was present in 39% of the cases with a mean of 18.97 ± 17.2 packs / year. Hospitalization was made on 66.7% of patients who visited the emergency department due to respiratory complications, with a mean of 9.7 ± 14.4 days. The distance traveled was 348.2 ± 109 meters. Men walked an average of 108.8 meters more than women. Discussion: The results are discussed within the local and regional context, considering the availability and use of diagnostic tests. The results contrast with the literature regarding the sex of the patients where ILDs predominate. The findings are consistent with the diseases' course in terms of emergency room visits and hospitalizations.

Interstitial Lung Disease and Lung Cancer Development: A 5-Year Nationwide Population-Based Study / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: In this nationwide 5-year longitudinal population-based study, we aimed at investigating the incidence of lung cancer among patients with interstitial lung disease. MATERIALS AND METHODS: Data was collected from the Korean National Health Insurance Research Database from 49,773,195 Korean residents in 2009. Thirteen thousand six hundred and sixty-six patients with interstitial lung disease diagnosed January-December 2009. The end of follow-up was June 30, 2014. Up to four matching chronic obstructive pulmonary disease controls were selected to compare the lung cancer high-risk group based on age, sex, diagnosis date (within 30 days), and hospital size. The number of patients with newly developed lung cancer was determined. RESULTS: The incidences of lung cancer were 126.98, 156.62, and 370.38 cases per 10,000 person-years (2,732, 809, and 967 cases of cancer, respectively) in the chronic obstructive pulmonary disease, interstitial lung disease, and chronic obstructive pulmonary disease with interstitial lung disease groups, respectively. Of the 879 patients with idiopathic pulmonary fibrosis, 112 developed lung cancer (incidence, 381.00 cases per 10,000 person-years). CONCLUSION: Incidence of lung cancer among patients with interstitial lung disease was high. Interstitial lung diseases have a high potential for developing into lung cancer, even when concurrent with chronic obstructive pulmonary disease.

Dispneia expiratória restritiva em um gato com fibrose pulmonar idiopática: relato de caso / Restrictive expiratory dyspnea in a cat with idiopathic pulmonary fibrosis: case report

As doenças pulmonares intersticiais constituem um grupo de doenças difusas do parênquima pulmonar, no qual a fibrose pulmonar intersticial está incluída. Histologicamente, esta se caracteriza por hiperplasia de pneumócitos tipo II, hiperplasia ou hipertrofia de músculo liso e fibrose. Embora a patogenia da fibrose pulmonar intersticial não esteja bem elucidada, devido às semelhanças microscópicas encontradas nos pneumócitos tipo II em felinos e na forma familiar da doença em humanos, acredita-se que haja caráter genético para o seu desenvolvimento. Os sinais clínicos frequentemente relatados incluem desconforto respiratório, cianose, letargia e perda de peso. Devido ao caráter progressivo e à ausência de tratamento específico, a doença apresenta prognóstico desfavorável. Foi atendida uma gata de 12 anos de idade, com histórico de dispneia há 20 dias. Ao exame clínico, o animal apresentou dispneia expiratória restritiva, crepitação à ausculta torácica e foi visualizado padrão intersticial ao exame radiográfico do tórax. A paciente foi submetida à punção com agulha fina de tecido pulmonar e veio a óbito algumas horas após o procedimento, apresentando insuficiência respiratória aguda. No exame histológico do tecido pulmonar, foi verificada a ocorrência de fibrose pulmonar idiopática. O objetivo do presente trabalho é relatar um caso de dispneia expiratória restritiva em um felino doméstico devido à fibrose pulmonar idiopática, já que, segundo o conhecimento dos autores, não há nenhum relato da ocorrência da doença no país.(AU)

Interstitial lung diseases are a group of diffuse parenchymal lung diseases in which interstitial lung fibrosis is included. Histologically, it is characterized by type II pneumocyte hyperplasia, hypertrophy or hyperplasia of smooth tissue and fibrosis. Although the pathogenesis of interstitial lung fibrosis has not been elucidated, due to the microscopic similarities found in type II pneumocytes in cats and familial form of the disease in humans, it is believed that there is a genetic trait for development. The frequently reported clinical signs include respiratory distress, cyanosis, lethargy, and weight loss. Due to the progressive nature and the absence of specific treatment, the disease has a poor prognosis. A 12-year-old cat with dyspnea for 20 days was assisted. The animal underwent fine needle aspiration of lung tissue and died few hours after the procedure, with acute respiratory failure. Upon histological examination of lung tissue, the occurrence of idiopathic pulmonary fibrosis was found. The aim of this study is to report a case of restrictive expiratory dyspnea in a domestic feline due to idiopathic pulmonary fibrosis, because, according to our knowledge, there is no report on the occurrence of the disease in our country.(AU)