Interstitial pneumonia with autoimmune features (IPAF): multidisciplinary diagnosis in pulmonology and rheumatology

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The diagnostic group of idiopathic interstitial pneumonia with autoimmune features (IPAF) includes patients with the idiopathic interstitial pneumonia and several clinical, serological and/or morphological signs, indicating the presence of the systemic autoimmune process, however, not meeting the current classificational criteria for a definite connective tissue disease. The data from clinical studies showed marked heterogeneity within this classification group. The authors review the current IPAF criteria as well as the perspective of their revision and subsequent application to improve the diagnosis and treatment of this disease.

Key words

Interstitial pneumonia with autoimmune features (IPAF), interstitial pneumonia (IP), high-resolution computed tomography (HRCT), connective tissue diseases.