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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Recent updates in advanced pulmonary sarcoidosis
James Tadjkarimi1, Evelyn Lynn1, Sujal Desai2
1Interstitial Lung Disease Unit.
Current Opinion in Pulmonary Medicine
|July 19, 2026
Summary
Recent advancements in advanced pulmonary sarcoidosis (APS) focus on high-resolution CT phenotyping and functional imaging. Treatment now emphasizes steroid-sparing agents, improving outcomes for this severe lung disease.
Area of Science:
- Pulmonology
- Radiology
- Rheumatology
Background:
- Advanced pulmonary sarcoidosis (APS) represents end-stage lung disease.
- It is characterized by fibrotic lung disease and/or active inflammation.
- Sarcoidosis-associated pulmonary hypertension (SAPH) is a critical complication.
Purpose of the Study:
- To review recent developments in phenotyping, monitoring, and treatment of APS.
- To highlight progress in recognizing and managing this severe condition.
- To inform clinical practice regarding APS management.
Main Methods:
- Utilizing high-resolution chest computed tomography (HRCT)-based phenotyping.
- Employing pulmonary function tests (PFTs) and fibrosis extent for prognosis.
- Incorporating early echocardiography and FDG-PET CT for inflammatory assessment.
Main Results:
- HRCT phenotyping and fibrosis extent are key prognostic tools.
- FDG-PET CT serves as a functional marker of inflammatory burden.
- Treatment shifts towards steroid-sparing agents, reducing toxicity.
- SAPH management requires identifying underlying mechanisms and targeted therapies.
Conclusions:
- APS encompasses fibrotic lung disease and SAPH, associated with high morbidity and mortality.
- Early assessment and multidisciplinary care are crucial.
- Ongoing vigilance is necessary for effective management of advanced disease.