ArticleRheumatology advances in practice2026
Pulmonary cystic destruction in systemic sclerosis and antisynthetase syndrome associated non-specific interstitial pneumonitis: a case series of a novel radiological phenotype.
Article in Rheumatology advances in practice, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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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 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 SSc-associated ILD (SSc-ILD) and two cases of antisynthetase syndrome-associated ILD (ASyS-ILD) with cystic changes were identified among a total of 108 SARD-ILD patients. All seven cases presented with radiological patterns of cellular non-specific interstitial pneumonia (NSIP). The median age at diagnosis with SARD was 33 years and six cases had ILD identified within 6 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 two cases confirming fibrotic NSIP, with one including affected tissue demonstrating intimal thickening of the 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.
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