KONDO Mitsuko
Department School of Medicine(Tokyo Women's Medical University Hospital), School of Medicine Position Professor (Fixed Term) |
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Article types | Original article |
Language | English |
Peer review | Peer reviewed |
Title | A comparison of the clinical features of ANCA-positive and ANCA-negative idiopathic pulmonary fibrosis patients. |
Journal | Formal name:Respiration; international review of thoracic diseases Abbreviation:Respiration ISSN code:1423-0356(Electronic)0025-7931(Linking) |
Volume, Issue, Page | 77(4),pp.407-15 |
Author and coauthor | Nozu Tomoko, Kondo Mitsuko, Suzuki Kazuo, Tamaoki Jun, Nagai Atsushi |
Authorship | 2nd author |
Publication date | 2009/09 |
Summary | BACKGROUND:The existence of antineutrophil cytoplasmic antibody (ANCA)-positive pulmonary fibrosis (PF) has recently been recognized. Objectives: The aim of this study was to clarify whether there is any difference in the clinical features between ANCA-positive PF and ANCA-negative PF.METHODS:A retrospective study was carried out on 53 patients with idiopathic PF whose myeloperoxidase (MPO)- and proteinase 3 (PR3)-ANCA levels were measured. After dividing the patients into ANCA-positive and ANCA-negative PF, we compared their symptoms, pulmonary function tests, chest CT findings, bronchoalveolar lavage findings, the effects of therapy and survival rates.RESULTS:17 of the 53 patients with idiopathic PF were MPO-ANCA-positive, 2 were PR3-ANCA-positive and 34 were negative for both ANCA types. Lactate dehydrogenase was lower in ANCA-positive PF than in ANCA-negative PF. However, there was no significant difference in the symptoms, lung function tests, CT findings and bronchoalveolar lavage findings. Corticosteroid therapy tended to be more effective in ANCA-positive PF, but the overall survival rate in ANCA-positive PF was not different from that in ANCA-negative PF. However, the low-titer (RESULTS:<RESULTS:50 EU) group showed better survival than the high-titer (RESULTS:>RESULTS:or =50 EU) group in ANCA-positive PF. ANCA titers of the patients who developed microscRESULTS:opic polyangiitis were higher than of those who did not develop it in ANCA-positive PF.CONCLUSIONS:Our studies showed little difference in the clinical features between ANCA-positive and ANCA-negative PF. However, it may be important to measure ANCA when idiopathic PF is diagnosed, because a high titer of ANCA suggests a poor prognosis and the association with microscopic polyangiitis. |
DOI | 10.1159/000183754 |
Document No. | 19077381 |