Mikroskopische Polyangiitis als ätiologischer Faktor der Lungenfibrose – eine bekannte, doch häufig spät diagnostizierte Ursache

Abstract

Lung fibrosis as first symptomatic manifestation of microscopic polyangiitis (MPA) is rare. It is characterized by progressive exertional dyspnea, radiologic detection of reticular shadowing, and increased titers of MPA-associated autoantibodies. This case study shall remind of the rare treatable cause of lung fibrosis. A 78-year-old male patient with long-standing exertional dyspnea and typical chest radiograph appearances of advanced lung fibrosis is presented. The finding of MPA autoantibodies in laboratory tests was decisive to confirm the diagnosis of MPA, and a therapy adapted to the disease severity could be initiated. An early diagnosis of MPA and a fast onset of therapy are important, because the influence of an already existent lung fibrosis is uncertain.