Development of microscopic polyangiitis-related pulmonary fibrosis in a patient with autoimmune pulmonary alveolar proteinosis

被引：7

作者：

Kinehara, Yuhei ^{[1
]}

Kida, Hiroshi ^{[1
]}

Inoue, Yoshikazu ^{[2
]}

Hirose, Masaki ^{[2
]}

Nakabayashi, Akihiko ^{[1
]}

Takeuchi, Yoshiko ^{[1
]}

Hayama, Yoshitomo ^{[1
]}

Fukushima, Kiyoharu ^{[1
]}

Hirata, Haruhiko ^{[1
]}

Inoue, Koji ^{[1
]}

Minami, Toshiyuki ^{[1
]}

Nagatomo, Izumi ^{[1
]}

Takeda, Yoshito ^{[1
]}

Funakoshi, Toshiki ^{[3
]}

Kijima, Takashi ^{[1
]}

Kumanogoh, Atsushi ^{[1
]}

机构：

[1] Osaka Univ, Grad Sch Med, Dept Resp Med Allergy & Rheumat Dis, Suita, Osaka 5650871, Japan

[2] Natl Hosp Org, Kinki Chuo Chest Med Ctr, Sakai, Osaka 5918555, Japan

[3] SenriSaiseikai Hosp, Suita, Osaka 5650862, Japan

来源：

BMC PULMONARY MEDICINE | 2014年 / 14卷

关键词：

Pulmonary alveolar proteinosis; Pulmonary fibrosis; Myeloperoxidase antineutrophil cytoplasmic antibody; ANCA; JAPAN; AUTOANTIBODIES; INVOLVEMENT; VASCULITIS; DIAGNOSIS;

D O I：

10.1186/1471-2466-14-172

中图分类号：

R56 [呼吸系及胸部疾病];

学科分类号：

摘要：

Background: Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare lung disease caused by the autoantibody against granulocyte-macrophage colony stimulating factor (GM-CSF). The clinical course of aPAP is variable; in severe cases, patients develop lethal respiratory failure due to pulmonary fibrosis. However, the pathogenesis of pulmonary fibrosis in aPAP has never been delineated. Case presentation: Here, we describe a rare case of aPAP that was subsequently complicated by microscopic polyangiitis related pulmonary fibrosis. The patient was a 75-year-old Japanese man diagnosed with aPAP based on the crazy-paving appearance on high-resolution computed tomography (HRCT), "milky" appearance of broncho-alveolar lavage fluid (BALF), and elevated serum levels of the anti-GM-CSF antibody. The patient was followed-up without aPAP-specific treatment for 3 years. During this period, both hematuria and proteinuria appeared; in addition, serum myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA) turned positive and increased markedly. The second BAL performed one year after the diagnosis, showed that the "milky" appearance had resolved. The HRCT showed that fibrotic changes had developed and that the crazy-paving appearance had disappeared. These data suggest an association between pulmonary fibrosis that developed during the natural course of aPAP and ANCA-related systemic vasculitis. Conclusion: This is the first case report that suggests the existence of a pathogenetic relationship between ANCA-associated systemic vasculitis and aPAP-related pulmonary fibrosis. The link between ANCA-associated systemic vasculitis and aPAP-related pulmonary fibrosis requires further investigation.

引用

页数：4

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