A case of pulmonary fibrosis associated with rheumatoid arthritis, scleroderma sine scleroderma and ANCA associated vasculitis

Abstract

Introduction: Diffuse parenchymal lung disease (DPLD) may be idiopathic or may be due to known associations such as autoimmune diseases. The prognosis in cases associated with autoimmune diseases depends on many factors such as histopathology, baseline lung function, auto-antibody level, et cetera. DPLD and its prognosis is worse in patients with overlap syndromes. Case description: We present a rare case of a 71 year old Caucasian lady with gradually worsening pulmonary fibrosis secondary to an overlap syndrome with rheumatoid arthritis (RA), scleroderma sine scleroderma (SSS) and anti neutrophil cytoplasmic antibody (ANCA) associated vasculitis. Discussion and Evaluation: In this paper, we share information from review of literature regarding DPLD associated with RA, systemic sclerosis (SSc) and ANCA associated vasculitis. Details of our discussion include prognostic factors, histology and radiographic features of these individual disease entities. Conclusion: Since pulmonary fibrosis in overlap syndromes has a poor prognosis, extensive work up should be performed even when clinical evidence of only one autoimmune disease is present.