The clinicopathological spectrum of pulmonary diseases in MPO-ANCA-associated vasculitides.
| Project/Area Number |
10670568
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Respiratory organ internal medicine
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| Research Institution | Okinaka Memorial Institute for Medical Research |
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Principal Investigator |
HOMMA Sakae Okinaka Memorial Institute for Medical Research, Researcher, 研究員 (20190275)
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| Co-Investigator(Kenkyū-buntansha) |
NAKATA Koichiro Okinaka Memorial Institute for Medical Research, Researcher, 研究員
TANAKA Sayuri Okinaka Memorial Institute for Medical Research, Researcher, 研究員
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| Project Period (FY) |
1998 – 2000
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| Project Status |
Completed (Fiscal Year 2000)
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| Budget Amount *help |
¥2,100,000 (Direct Cost: ¥2,100,000)
Fiscal Year 2000: ¥900,000 (Direct Cost: ¥900,000)
Fiscal Year 1999: ¥700,000 (Direct Cost: ¥700,000)
Fiscal Year 1998: ¥500,000 (Direct Cost: ¥500,000)
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| Keywords | Pulmonary fibrosis / MPO-ANCA / Vasculitis / Histopathology / Prognosis / 治療 / 肺病変 / 間質性肺炎 / 肺出血 / 細気管支炎 |
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| Research Abstract |
The aim of this study is to assess pulmonary fibrosis (PF) in patients who tested positive for myeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA) by enzyme-linked immunosorbent assay. We reviewed 43 patients (21 males and 22 females with a mean age of 65 yrs) diagnosed as having pulmonary disease with positive MPO-ANCA levels ranging from 10 to 840 EU with a mean of 141.7 EU, during the period from 1993 through 2000. The pulmonary disorders observed in the 43 patients were PF in 31, bronchiolitis in 6, pulmonary hemorrhage in 5 and bronchial asthma in 1. Evidence of glomerulonephritis was demonstrated in 16 patients. Among 31 patients with PF, some had underlying systemic diseases as follows : 8 had microscopic polyangiitis ; 4 had sjogren's syndrome ; 3 patients had progressive systemic sclerosis (PSS) ; rheumatoid arthritis (RA) was -found in 2 ; polymyositis, PSS/RA, mixed connective tissue disease, relapsing polychondritis, and sarcoidosis were each found in 1 patient
… More
while 9 patients had PF of unknown etiology . The clinical features included a history of dry cough and/or fine crackles in all 31 patients. Chest radiographs showed honeycomb lesions in the lung bases in 26 of the patients. Histopathological features of the diseased lung tissues were analyzed in 15 patients with PF.The interstitial fibrosis was not confined to the alveolar walls, but extended to the proximal interstitial tissues and interlobular septa in 13. Honeycomb lesions were noted in 12 cases. Vasculitis was confirmed in the bronchial arteries and/or pulmonary arterioles in 5. The mortalitiy was as high as 13 out of 31 patients with PF.The causes of death were : exacerbation of PF in 5 (two of whom were associated with pulmonary hemorrhage), lung cancer in 2, pneumonia in 4 and digestive tract bleeding in 2. Although there was no correlation between MPO-ANCA titers and the activity of PF, the results emphasize that the positive NPO-ANCA is an important unfavorable prognostic factor in patients with PF. Less
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Report
(4 results)
Research Products
(18 results)
