Abstract
Recent evidence suggests that Idiopathic Nonspecific Interstitial Pneumonia (iNSIP) is a distinct clinical entity amongst other idiopathic interstitial pneumonias, and few data seem to suggest a likely pathogenetic role of autoimmunity. The aim of the present study was to assess if iNSIP might represent an early lung manifestation of an autoimmune disease.
After initial review of cases found in the medical records database by searching for the term “NSIP” (n=63), thirty-seven iNSIP were identified, and were re-evaluated on a dynamic integrated multidisciplinary approach.
Twenty-seven cases with iNSIP were selected for the study. Mean±SD age at first respiratory symptom was 54.2±8 years, 70% were females, and 59% were never-smokers. At follow-up (mean months±SD: 59.7±29, range: 12–138), autoimmune diseases occurred in fourteen patients (51.8%), with 7 autoimmune thyroiditis (26%), 6 undifferentiated connective disease (UCTD) (22%), and 3 connective tissue disease (CTD) (11%). Patients developing autoimmune diseases were older and more frequently never-smoking females.
In more than 50% of patients diagnosed with iNSIP evidence of autoimmune diseases develop within 2 years, suggesting a likely link between the clinical entity of iNSIP and autoimmune disorders.
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