Milder ILD with preserved lung function significantly contributes to respiratory caused mortality in SSc

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Background: While there is excess mortality in systemic sclerosis (SSc) associated ILD with FVC% predicted <70%, risk in less severe ILD is less clear. Objective: To evaluate respiratory-caused mortality in SSc-patients with FVC ≥70%. Methods: We assessed characteristics associated with respiratory and non-respiratory causes of death in a prospective SSc-ILD cohort including 323 Norwegian patients. The severity of ILD was defined as FVC% predicted ≥/<70%, extent of ILD on HRCT</≥10%, and by respiratory symptoms (functional class ≤/> 2). Results: Respiratory causes of death were observed in 24/99 deceased patients. Of 24 patients dying of respiratory cause, 67% died of respiratory tract infections and 33% from respiratory failure. Mean 1.1 year prior to death, these 24 SSc-ILD patients had 12 (50%) FVC ≥70%, 57% had ≤10% extent of ILD, and 44% had functional class ≤ 2. Compared to 104 surviving patients with FVC ≥70% were more often older, male, had progressive ILD, presence of respiratory symptoms, reduced six-minute walking distance, and pulmonary hypertension. Conclusion: Respiratory causes of death were common among SSc-ILD patients with preserved lung function. This finding highlights the importance of up-front measures to halt disease progression, even in cases with apparently mild ILD phenotypes. erj;64/suppl_68/PA4271/F1 F1 F1

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