Fast Five Quiz: Idiopathic Pulmonary Fibrosis Differential Diagnosis

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


January 26, 2023

Because presenting signs and symptoms and HRCT findings are similar for many interstitial lung diseases, the definitive diagnosis is made with a surgical lung biopsy or bronchoscope cryobiopsy. The histologic pattern of NSIP has varying degrees of inflammation and fibrosis in a temporally uniform pattern with preserved architecture of the underlying alveoli. This is unlike the temporal heterogeneity seen in patients with usual interstitial pneumonias such as IPF.

Erythrocyte sedimentation rate, C-reactive protein, and chest x-ray are all components of the NSIP workup but are typically not required for definitive diagnosis.

Thyroid-stimulating hormone, free triiodothyronine, and free thyroxine studies are typically not necessary to confirm the diagnose of NSIP, although abnormalities in these hormones may be present in patients with NSIP because of the link between autoimmune disease and NSIP.

NSIP may respond to anti-inflammatory medications; however, a treatment trial response does not provide a definitive diagnosis.

Learn more about the differential diagnosis of NSIP.


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