Open AccessMyeloperoxidase Anti-Neutrophil Cytoplasmic Antibody-Positive Interstitial Pneumonia Associated with Granulomatosis with Polyangiitis Diagnosed by Surgical Lung Biopsy
Author(s) -
Haruo Chino,
Eri Hagiwara,
Hideya Kitamura,
Tomohisa Baba,
Hideaki Yamakawa,
Tamiko Takemura,
Takashi Ogura
Publication year - 2016
Publication title -
respiration
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.264
H-Index - 81
eISSN - 1423-0356
pISSN - 0025-7931
DOI - 10.1159/000449529
Subject(s) - medicine , pathology , lung biopsy , proteinase 3 , anti neutrophil cytoplasmic antibody , lung , microscopic polyangiitis , granulomatosis with polyangiitis , vasculitis , myeloperoxidase , biopsy , inflammation , disease
Granulomatosis with polyangiitis (GPA) is a rare systemic vasculitis that often involves the lung. However, interstitial pneumonia (IP) is rarely seen in GPA patients. We herein report 3 cases of IP associated with GPA diagnosed by surgical lung biopsy. High-resolution CT showed uniform subpleural reticular opacity with traction bronchiectasis. Biopsies from all 3 patients revealed neutrophilic capillaritis, microabscesses with giant cells, and coexisting histological findings of usual IP pattern or fibrosing nonspecific IP pattern. All 3 patients had elevated levels of serum myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA), but not proteinase 3-ANCA. We diagnosed GPA and treated with corticosteroid and cyclophosphamide. Follow-up CT showed improvement of the lesions in all patients. Surgical lung biopsy specimens which revealed GPA enabled us to conduct the most suitable therapy. This report indicates the importance of surgical lung biopsy for differentiating idiopathic IPs from GPA-associated IP and suggests a relationship between MPO-ANCA and IP in GPA.