Chiaki Hosoda1, Tomohisa Baba1, Eri Hagiwara1, Hiroyuki Ito1, Norikazu Matsuo1, Hideya Kitamura1, Tae Iwasawa2, Koji Okudela3, Tamiko Takemura4, Takashi Ogura1. 1. Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan. 2. Department of Radiology, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan. 3. Department of Pathology, Graduate School of Medicine, Yokohama City University, Yokohama, Japan. 4. Department of Pathology, Japan Red Cross Medical Center, Tokyo, Japan.
Abstract
BACKGROUND AND OBJECTIVE: Myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) is occasionally positive in patients with usual interstitial pneumonia (UIP). However, the differences from idiopathic pulmonary fibrosis (IPF/UIP) have not been well documented. We aimed to clarify the clinical, radiological and pathological features of UIP associated with MPO-ANCA (ANCA/UIP). METHODS: We retrospectively reviewed the medical records of 12 consecutive ANCA/UIP patients not manifesting microscopic polyangiitis and 108 IPF/UIP patients with no autoantibodies, both diagnosed by surgical lung biopsy. RESULTS: There was no significant difference in clinical background, laboratory results and pulmonary function tests between ANCA/UIP patients and IPF/UIP patients except for the percentage of bronchoalveolar lavage neutrophils. HRCT showed subpleural reticulation in both groups. Increased attenuation around honeycombing and cysts was significantly observed in ANCA/UIP. Pathologically, ANCA/UIP had more prominent inflammatory cell infiltration, lymphoid follicles with germinal centres and cellular bronchiolitis. During the disease course, three of 12 patients (25%) developed microscopic polyangiitis. Immunosuppressive treatment tended to be more effective in ANCA/UIP patients, and the survival time in ANCA/UIP patients tended to be longer than those with IPF/UIP. CONCLUSION: ANCA/UIP may be distinguishable from IPF/UIP with a combination of HRCT findings of increased attenuation around honeycombing and cysts and some of the characteristic pathological findings. In contrast to IPF/UIP, immunosuppressive treatment could be a therapeutic option for ANCA/UIP.
BACKGROUND AND OBJECTIVE: Myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) is occasionally positive in patients with usual interstitial pneumonia (UIP). However, the differences from idiopathic pulmonary fibrosis (IPF/UIP) have not been well documented. We aimed to clarify the clinical, radiological and pathological features of UIP associated with MPO-ANCA (ANCA/UIP). METHODS: We retrospectively reviewed the medical records of 12 consecutive ANCA/UIP patients not manifesting microscopic polyangiitis and 108 IPF/UIP patients with no autoantibodies, both diagnosed by surgical lung biopsy. RESULTS: There was no significant difference in clinical background, laboratory results and pulmonary function tests between ANCA/UIP patients and IPF/UIP patients except for the percentage of bronchoalveolar lavage neutrophils. HRCT showed subpleural reticulation in both groups. Increased attenuation around honeycombing and cysts was significantly observed in ANCA/UIP. Pathologically, ANCA/UIP had more prominent inflammatory cell infiltration, lymphoid follicles with germinal centres and cellular bronchiolitis. During the disease course, three of 12 patients (25%) developed microscopic polyangiitis. Immunosuppressive treatment tended to be more effective in ANCA/UIP patients, and the survival time in ANCA/UIP patients tended to be longer than those with IPF/UIP. CONCLUSION: ANCA/UIP may be distinguishable from IPF/UIP with a combination of HRCT findings of increased attenuation around honeycombing and cysts and some of the characteristic pathological findings. In contrast to IPF/UIP, immunosuppressive treatment could be a therapeutic option for ANCA/UIP.
Authors: Marco A Alba; Luis Felipe Flores-Suárez; Ashley G Henderson; Hong Xiao; Peiqi Hu; Patrick H Nachman; Ronald J Falk; J Charles Jennette Journal: Autoimmun Rev Date: 2017-05-04 Impact factor: 9.754
Authors: Gabrielle Y Liu; Iazsmin Bauer Ventura; Natalia Achtar-Zadeh; Brett M Elicker; Kirk D Jones; Paul J Wolters; Harold R Collard; Ayodeji Adegunsoye; Mary E Strek; Brett Ley Journal: Chest Date: 2019-06-07 Impact factor: 9.410