Fast Five Quiz: Idiopathic Pulmonary Fibrosis Differential Diagnosis

Guy W. Soo Hoo, MD, MPH; Zab Mosenifar, MD


April 04, 2022

Stage 4 sarcoidosis can clinically mimic IPF. Sarcoidosis is a multisystem granulomatous disease of unknown etiology that primarily affects the lungs. Although many patients with pulmonary sarcoidosis experience periods of remission and favorable long-term outcomes, approximately 10%-30% develop pulmonary fibrosis and progressive pulmonary disease (S4).

Clinical differences between sarcoidosis and IPF can help in the differential diagnosis. The typical histologic honeycombing pattern and fibroblast foci associated with IPF are not typically seen in sarcoidosis. In addition, the nonproductive cough associated with IPF is typically not a symptom in patients with sarcoidosis.

Positron emission tomography (PET) scan may be more helpful than HRCT scans to assess patients with sarcoidosis because it can detect inflammatory activity which guides treatment. The treatment plan for S4 includes anti-inflammatory and immunosuppressive agents including azathioprine, corticosteroids, leflunomide, and methotrexate.

Learn more about sarcoidosis.


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