Prevalence of myositis specific and associated antibodies in a cohort of patients affected by idiopathic NSIP and no hint of inflammatory myopathies

The presence of interstitial lung disease (ILD) is a common and fearsome feature of idiopathic inflammatory myopathies (IIM). Such patients show radiological pattern of non-specific interstitial pneumonia (NSIP). The present study aimed to assess the prevalence of myositis-specific and myositis-associated antibodies (MSA and MAA) in a cohort of patients with a previous diagnosis of NSIP and no sign or symptom of IIM. Secondly, it will be assessed whether patients displaying MSA and/or MAA positivity have a worse or a better outcome than idiopathic NSIP. All patients affected by idiopathic NSIP were enrolled. MSA and MAA were detected using EUROLINE Autoimmune Inflammatory Myopathies 20 Ag (Euroimmun Lubeck, Germany), line immunoassay. A total of 16 patients (mean age 72 +/- 6.1 years old) were enrolled. Six out of 16 patients (37.5%) had significant MSA and/or MAA positivity: one displayed positivity of anti-PL-7 (+ +), one of anti-Zo (+ +), anti-TIF1 gamma (+ + +) and anti-Pm-Scl 75 (+ + +), one of anti-Ro52 (+ +), one of anti-Mi2 beta (+ + +), one of anti-Pm-Scl 75 (+ + +) and the latter of both anti-EJ (+ + +) and anti-Ro52 (+ + +).Two out of 7 seropositive patients showed a significant impairment of FVC (relative risk 4.8, 95% CI 0.78-29.5; p = 0.0350). Accordingly, among the 5 patients that started antifibrotic treatment during the observation time, 4 were seronegative. Our findings highlighted a potential autoimmune or inflammatory in idiopathic NSIP patients and also in those without significant rheumatological symptoms. A more accurate diagnostic assessment may ameliorate diagnostic accuracy as well as may provide new therapeutic strategy (antifibrotic + immunosuppressive). A cautious assessment of NSIP patients with a progressive and non-responsive to glucocorticoids disease course should therefore include an autoimmunity panel comprising MSA and MAA.

Automated summary

The study aimed to assess the prevalence of myositis-specific and myositis-associated antibodies (MSA and MAA) in patients previously diagnosed with non-specific interstitial pneumonia (NSIP) and without symptoms of idiopathic inflammatory myopathies (IIM). The results showed that 37.5% of the patients displayed significant MSA and/or MAA positivity, and two seropositive patients showed a significant impairment of FVC. A cautious assessment of NSIP patients, especially those with progressive and non-responsive disease, should include an evaluation of MSA and MAA.