Pulmonary cystic destruction in systemic sclerosis and anti-synthetase syndrome associated non-specific interstitial pneumonitis: a case series of a novel radiological phenotype

Abstract Objectives To describe a novel radiographic phenotype of Systemic Autoimmune Rheumatic Disease associated Interstitial Lung Disease (SARD-ILD) observed within a regional specialist multidisciplinary (MDT) service. Methods This was a retrospective case series of patients with SARD-ILD complicated by radiographic pulmonary cystic destruction. Cases were identified through review of multidisciplinary team (MDT) records from the North Bristol SARD-ILD service. Cases with MDT reported ‘cystic changes’ were identified and clinico-radio-pathological features reviewed. Results Five cases of systemic sclerosis-associated interstitial lung disease (SSc-ILD) and two cases of anti-synthetase syndrome-associated interstitial lung disease (ASyS-ILD) with cystic changes were identified amongst a total of one-hundred and eight SARD-ILD patients. All seven cases presented with radiological patterns of cellular non-specific interstitial pneumonia (NSIP). Median age at diagnosis with SARD was 33 years, six cases had ILD identified within six months of SARD diagnosis. The cohort was ethnically diverse with one ex-smoker. Microcystic destructive changes appeared and progressed within areas of ground glass despite standard-of-care immunomodulation. Changes are radiographically and histologically distinct from traction bronchiolectasis, honeycombing and smoking related lung disease. Histological specimens were available for 2 cases confirming fibrotic NSIP, with one including affected tissue demonstrating intimal thickening of pulmonary vasculature. Over a median follow up of 49 months, all cases remain alive and transplant free; five fulfilled criteria for progressive disease and six required ambulatory oxygen. Conclusions This represents the first western cohort describing microcystic destructive pulmonary changes in SSc-ILD and the first report in ASyS-ILD. Systematic case identification is required to determine demographic associations and prognostic implications.