Abstract | BACKGROUND: METHODS: We enrolled 61 consecutive patients with IPF and measured the MPO- ANCA titers at initial presentation and during the follow-up period. Clinical, radiologic and histologic features of MPO- ANCA-positive cases were examined. RESULTS: Of 61 patients, 3 (4.9%) had positive MPO- ANCA titers at the initial presentation of IPF. During the disease course, MPO- ANCA-positive conversion occurred in 6 patients and the prevalence of ANCA increased to 14.8%. Among the nine patients positive for MPO- ANCA, two patients developed MPA during follow-up. Histologic features of MPO- ANCA-positive pulmonary fibrosis were compatible with the usual interstitial pneumonia pattern in which alveolar hemorrhage and capillaritis were not observed. The patients with MPO- ANCA-positive conversion showed increased percentages of bronchoalveolar lavage eosinophils and more frequent complication of pulmonary emphysema compared to those with MPO- ANCA-negative IPF. CONCLUSIONS: The findings of the present study demonstrated that patients with an initial diagnosis of IPF occasionally acquire MPO- ANCA, which develops to MPA during the disease course of IPF. The presence of pulmonary eosinophilia and low attenuation areas on computed tomography scans might be predictive of MPO- ANCA positive conversion.
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Authors | Masaru Ando, Eishi Miyazaki, Toshihiro Ishii, Yutaka Mukai, Mari Yamasue, Hideaki Fujisaki, Takeo Ito, Shin-ichi Nureki, Toshihide Kumamoto |
Journal | Respiratory medicine
(Respir Med)
Vol. 107
Issue 4
Pg. 608-15
(Apr 2013)
ISSN: 1532-3064 [Electronic] England |
PMID | 23434037
(Publication Type: Journal Article)
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Copyright | Copyright © 2013 Elsevier Ltd. All rights reserved. |
Chemical References |
- Antibodies, Antineutrophil Cytoplasmic
- Glucocorticoids
- Immunosuppressive Agents
- Peroxidase
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Topics |
- Adult
- Aged
- Aged, 80 and over
- Antibodies, Antineutrophil Cytoplasmic
(blood)
- Bronchoalveolar Lavage Fluid
(cytology)
- Female
- Follow-Up Studies
- Glucocorticoids
(therapeutic use)
- Humans
- Idiopathic Pulmonary Fibrosis
(complications, diagnostic imaging, drug therapy, immunology)
- Immunosuppressive Agents
(therapeutic use)
- Male
- Microscopic Polyangiitis
(diagnostic imaging, drug therapy, etiology, immunology)
- Middle Aged
- Peroxidase
(immunology)
- Tomography, X-Ray Computed
- Treatment Outcome
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