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Updated: Jul 16, 2025
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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
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Pulmonary fibrosis in sarcoidosis
Huda Asif1, Manuel Ribeiro Neto2, Daniel Culver3
1University of South Florida, FL, USA . hasif@usf.edu.
Summary
Sarcoidosis can lead to pulmonary fibrosis in 5% of patients, increasing mortality. Early diagnosis and treatment with anti-fibrotic agents can slow disease progression.
Area of Science:
- Pulmonology
- Pathology
- Radiology
Background:
- Sarcoidosis can progress to pulmonary fibrosis in 5% of cases, significantly increasing mortality.
- Histopathology reveals fibrosis in a lymphangitic pattern around granulomas, with a Th1 to Th2 shift and angiogenesis implicated in fibrosis.
- Clinical presentation includes dyspnea and cough, often with pulmonary function tests showing a mixed ventilatory defect and decreased diffusion capacity.
Purpose of the Study:
- To summarize the key aspects of sarcoidosis-associated pulmonary fibrosis.
- To highlight diagnostic markers and imaging findings.
- To outline current and emerging treatment strategies.
Main Methods:
- Review of histopathological findings in sarcoidosis-associated pulmonary fibrosis.
- Analysis of clinical features and pulmonary function test results.
- Evaluation of serologic markers and CT imaging characteristics.
- Assessment of treatment outcomes with glucocorticoids, steroid-sparing agents, and anti-fibrotic therapies.
- Review of complications and transplant eligibility criteria.
Main Results:
- Elevated serologic markers (soluble interleukin 2 receptor, chitotriosidase, KL-6, chemokine ligand 18) are associated with disease progression.
- CT imaging demonstrates characteristic fibrosis patterns, predominantly in the upper and central lung zones.
- Complications include sarcoidosis-associated pulmonary hypertension (SAPH) and chronic pulmonary aspergillosis.
- Anti-fibrotic agents like pirfenidone and nintedanib have demonstrated efficacy in slowing pulmonary function decline.
- Lung transplantation is a viable option for advanced disease (NYHA class III or IV).
Conclusions:
- Sarcoidosis-associated pulmonary fibrosis is a serious complication with distinct clinical, imaging, and serologic features.
- Prompt diagnosis and management, including anti-fibrotic therapy, are crucial for improving outcomes.
- Multidisciplinary care and consideration for lung transplantation are essential for eligible patients.
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