Interstitial Lung Disease with ANCA-associated Vasculitis

Abstract

The association between interstitial lung disease (ILD) and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), particularly microscopic polyangiitis (MPA), has been described in a number of case reports and case series reports in the last 2 decades. In addition, patients with pulmonary fibrosis and ANCA positivity but without other manifestations of systemic vasculitis have also been reported. Pulmonary fibrosis was clinically manifested at the time of diagnosis in the majority of AAV patients that developed this condition. Moreover, ANCA-positive conversion occurs in patients initially diagnosed with idiopathic pulmonary fibrosis, and as a result, other manifestations of systemic vasculitis develop in some of these patients. There is significant predominance of myeloperoxidase (MPO)-ANCA and MPA in patients with AAV and ILD. Radiological and pathological findings generally demonstrate usual interstitial pneumonia (pattern) in the lungs of these patients. In most studies, AAV patients with ILD have a worse prognosis than those without it.

Keywords: anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis; interstitial lung disease; microscopic polyangiitis.

Figure 1

Representative high-resolution computed tomography scan in a patient with interstitial lung disease and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. This patient was initially diagnosed with idiopathic pulmonary fibrosis, and myeloperoxidase-ANCA-positive granulomatosis with polyangiitis developed later.