Atypical presentation and progression of scleroderma associated interstitial lung disease

Abstract

A known complication associated with scleroderma or systemic sclerosis (SSc) is the development of interstitial lung disease (ILD). It is occasional that symptoms of connective tissue diseases (CTDs) emerge in patients with ILD that have previously ruled out rheumatologic etiology. Presented here is a unique case of scleroderma associated interstitial lung disease (SSc-ILD) where the diagnosis of scleroderma was made over 5 years after the diagnosis of ILD. A 52-year-old female presented with dyspnea on exertion and chronic cough with a history of ILD dating back 5 years. Initial consultation revealed negative autoimmune serology and no symptoms or signs associated with scleroderma. When new rheumatologic features presented months later, serology was repeated and showed positive antinuclear antibody with nucleolar pattern suggestive of scleroderma. This case shows a potential importance of thoroughly monitoring rheumatologic signs and symptoms with patients already diagnosed with ILD, even with no initial suggestions of CTDs.