The three smoking-related interstitial lung diseases (ILD) include desquamative interstitial pneumonia (DIP), respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), and pulmonary Langerhans cell histiocytosis (PLCH). They are considered discrete entities, yet it is not unusual to find a mixture of pathologic features rendering the histopathologic diagnosis difficult. It is uncommon to have overlap in the different radiologic findings between these diseases. We present a unique case, in that the extent of DIP and PLCH-like changes were manifested both histologically and on high resolution computed tomography (HRCT) with ground-glass attenuation and upper lobe cystic changes suggestive of both diseases.
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http://dx.doi.org/10.1097/SMJ.0b013e318187cced | DOI Listing |
J Clin Rheumatol
November 2024
Servicio de Reumatología Hospital JM Cullen, Santa Fe, Argentina.
Objectives: The aims of this study were to describe the frequency of pleuropulmonary computed tomography (CT) findings in patients with IgG4-related disease (IgG4-RD) and to compare clinical and laboratory characteristics between patients with and without pleuropulmonary involvement in chest CT.
Methods: This is a study conducted within the IgG4-RD study group of the Argentine Society of Rheumatology (GESAR IgG4) cohort of patients with IgG4-RD. Member centers of the group were requested to submit pulmonary CT scans of the patients.
Eur Clin Respir J
January 2025
Department of Respiratory Medicine, Odense University Hospital, Odense, Denmark.
Background: Birt-Hogg-Dubé syndrome (BHD), a rare genetic disease characterized by multiple pulmonary cysts, can lead to spontaneous pneumothorax, cutaneous hamartomas, renal cysts, and renal cell cancer. The overall aim of this study was to assess clinical characteristics of patients with BHD-emphasizing on trends in pulmonary function patterns.
Methods: By use of data from electronic patient journals, we conducted a retrospective cohort study on clinical characteristics and pulmonary function tests (PFT) from patients with BHD, who were clinically followed-up in a Danish tertiary referral center for rare and interstitial lung diseases.
Ulcerative colitis can present with extra-intestinal manifestations, including interstitial lung disease and primary sclerosing cholangitis. When pulmonary symptoms precede gastrointestinal, diagnosis can be challenging. Consideration of Ulcerative colitis in patients with unexplained lung and hepatic pathology is crucial, as a failure of timely intervention can lead to multiorgan complications.
View Article and Find Full Text PDFCurr Treatm Opt Rheumatol
December 2024
Division of Rheumatology, Inflammation, and Immunity, Brigham and Women's Hospital, Boston, MA USA.
Purpose Of Review: To summarize the current treatment landscape of rheumatoid arthritis-associated interstitial lung disease (RA-ILD) in the context of the recent 2023 American College of Rheumatology/American College of Chest Physicians guideline for ILD treatment in systemic autoimmune rheumatic diseases.
Recent Findings: The guideline conditionally recommends mycophenolate, azathioprine, and rituximab for first-line RA-ILD therapy, with cyclophosphamide and short-term glucocorticoids as additional options. For RA-ILD progression after first line, mycophenolate, rituximab, nintedanib, tocilizumab, cyclophosphamide, and pirfenidone are conditionally recommended, while long-term glucocorticoids are conditionally recommended against.
Cureus
December 2024
Internal Medicine, Unidade Local de Saúde de São José, Lisbon, PRT.
Anti-melanoma differentiation-associated protein 5 (anti-MDA5) clinically linked amyopathic dermatomyositis (CADM) is a rare autoimmune condition strongly linked to rapidly progressive interstitial lung disease (RP-ILD), a life-threatening complication. We present a 63-year-old female patient with anti-MDA5-positive CADM, who developed RP-ILD with an imaging pattern consistent with organizing pneumonia. She presented with Gottron's papules, periungual erythema, progressive dyspnea, and anorexia.
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