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

BMC Pulmonary Medicine

Atsushi Kumanogoh¹  Takashi Kijima¹  Toshiki Funakoshi³  Yoshito Takeda¹  Izumi Nagatomo¹  Toshiyuki Minami¹  Koji Inoue¹  Haruhiko Hirata¹  Kiyoharu Fukushima¹  Yoshitomo Hayama¹  Yoshiko Takeuchi¹  Akihiko Nakabayashi¹  Masaki Hirose²  Yoshikazu Inoue²  Hiroshi Kida¹  Yuhei Kinehara¹ 

[1] Department of Respiratory Medicine, Allergy and Rheumatic Diseases, Osaka University Graduate School of Medicine, 2-2 Yamada-oka, Suita, Osaka 565-0871, Japan;National Hospital Organization Kinki-Chuo Chest Medical Center, 1180 Nagasone-Cho, Kita-Ku, Sakai, Osaka 591-8555, Japan;SenriSaiseikai Hospital, 1-1-6 Tsukumodai, Suita, Osaka 565-0862, Japan

关键词: Myeloperoxidase antineutrophil cytoplasmic antibody;    Pulmonary fibrosis;    Pulmonary alveolar proteinosis;   

Others  :  1091534
DOI  :  10.1186/1471-2466-14-172

 received in 2014-07-22, accepted in 2014-10-23,  发布年份 2014

【 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.

【 授权许可】

   
2014 Kinehara et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

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