ANCA-associated renal vasculitis and acute exacerbation of scleroderma-associated interstitial lung disease: A case report

Abstract

In this report, we describe a patient with the unusual concomitant presentation of an acute exacerbation of systemic sclerosis-associated interstitial lung disease with the development of anti-neutrophil cytoplasmic antibody-associated active renal vasculitis. Systemic sclerosis is a connective tissue disease characterized by systemic autoimmunity and organ fibrosis and vasculopathy. Interstitial lung disease is identified in the majority of patients with systemic sclerosis and may have varying degrees of severity. Patients with systemic sclerosis-associated interstitial lung disease rarely develop acute exacerbations of interstitial lung disease.  An overlap of systemic vasculitis has been reported to occur in patients with systemic sclerosis – yet this too is a rare occurrence. This case is unique in that it demonstrates the concomitant presentation of these rare clinical scenarios and serves to highlight the importance of a comprehensive and multidiscciplinary approach to the evaluation of patients in general, and systemic autoimmune patients in particular.