Development of microscopic polyangiitis following idiopathic pleuroparenchymal l fibroelastosis: A case report

Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare type of idiopathic interstitial pneumonia, which is characterised by pleural fibrosis and subjacent parenchymal fibroelastosis of the upper lobes. Herein, we present a case of microscopic polyangiitis (MPA) following PPFE. The patient had abnormal shadows on chest radiographs 15 years before the onset of MPA, and the patient was diagnosed with PPFE. Four years after the PPFE diagnosis, the patient was diagnosed with MPA based on persistent fever, purpura, mononeuritis multiplex, myeloperoxidase-antineutrophil cytoplasmic antibody positivity, and pathological findings of peritubular capillaritis on kidney biopsy. The patient was treated with glucocorticoids, including methylprednisolone pulse therapy and rituximab, followed by maintenance therapy with rituximab. One year after treatment, the PPFE had not worsened. PPFE occasionally occurs secondary to connective tissue disease, including MPA; however, to the best of our knowledge, this is the first report of PPFE preceding MPA. Our case suggests that PPFE, as other interstitial lung diseases, may be associated with MPA and precede the onset of MPA. The accumulation of more cases is needed to clarify the characteristics of MPA-associated PPFE.