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Updated: Jul 5, 2025
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
The Pattern and Progression of "Usual" Interstitial Pneumonia with Autoimmune Features: Comparison with Patients with
Alessandro Libra1, Michele Colaci2, Lucia Spicuzza1
1Department of Clinical and Experimental Medicine, Regional Referral Center for Rare Lung Disease, Policlinico "G. Rodolico-San Marco", University of Catania, 95123 Catania, Italy.
Background:
We proposed the term "UIPAF" to define patients with Usual Interstitial Pneumonia (UIP) associated with only one domain of the classification called "Interstitial Pneumonia with Autoimmune Features" (IPAF). The objective of this study was to evaluate the clinical presentation and prognosis of UIPAF patients, compared with two cohorts, composed of IPAF and idiopathic pulmonary fibrosis (IPF) patients, respectively.
Methods:
The patients were enrolled as IPAF, UIPAF, or IPF based on clinical, serological, and radiological data and evaluated by a multidisciplinary team.
Results:
We enrolled 110 patients with IPF, 69 UIPAF, and 123 IPAF subjects. UIPAF patients were similar to IPAF regarding autoimmune features, except for the prevalence of Rheumatoid Factor in UIPAF and anti-SSA in IPAF. A similar proportion of the two cohorts progressed toward a specific autoimmune disease (SAD), with differences in the kind of SAD developed. The real-life management and prognosis of UIPAF patients proved to be almost identical to IPF.
Conclusions:
UIPAF shared with IPAF similar autoimmune features, suggesting the opportunity to be considered IPAF, excluding the morphological domain by the classification. However, the real-life management and prognosis of UIPAF are similar to IPF. These data suggest a possible modification in the therapeutic management of UIPAF.
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