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01.12.2015 | Research article | Ausgabe 1/2015 Open Access

BMC Pulmonary Medicine 1/2015

Outcome of corticosteroid administration in autoimmune pulmonary alveolar proteinosis: a retrospective cohort study

BMC Pulmonary Medicine > Ausgabe 1/2015
Keiichi Akasaka, Takahiro Tanaka, Nobutaka Kitamura, Shinya Ohkouchi, Ryushi Tazawa, Toshinori Takada, Toshio Ichiwata, Etsuro Yamaguchi, Masaki Hirose, Toru Arai, Kentaro Nakano, Takahito Nei, Haruyuki Ishii, Tomohiro Handa, Yoshikazu Inoue, Koh Nakata
Wichtige Hinweise

Electronic supplementary material

The online version of this article (doi:10.​1186/​s12890-015-0085-0) contains supplementary material, which is available to authorized users.

Competing interests

The authors declare that they have no competing interests.
The authors report no potential conflicts of interest with any companies or organizations whose products or services are mentioned in this article.

Authors’ contributions

KA and KN are responsible for the entire manuscript. KA, TT, NK, RT, and KN designed this study. TT and NK designed data base and performed statistical analysis. KA, SO, TT, TI, EY, MH, TA, KN, TN, HI, TH, and YI collected the patient’s data. KA, TT, NK, RT, and KN wrote this manuscript. All authors read and approved the final manuscript.



Although no report has demonstrated the efficacy of corticosteroid therapy for autoimmune pulmonary alveolar proteinosis (aPAP), we sometimes encounter patients who have received this therapy for various reasons. However, as corticosteroids can suppress alveolar macrophage function, corticosteroid therapy might worsen disease severity and increase the risk of infections.


For this retrospective cohort study, we sent a screening form to 165 institutions asking for information on aPAP patients treated with corticosteroids. Of the resulting 45 patients screened, 31 were enrolled in this study. We collected demographic data and information about corticosteroid treatment period, dose, disease severity score (DSS) over the treatment period, and complications.


DSS deteriorated during corticosteroid therapy in 23 cases (74.1 %) and the estimated overall cumulative worsening rate was 80.8 % for the total observation period. The worsening rate was significantly higher in patients treated with high-dose prednisolone (>18.9 mg/day, n = 16) than treated with low-dose prednisolone (≤18.9 mg/day, n = 15) divided by median daily dose (p < 0.02). Of patients with worsening, one died of disseminated aspergillosis and another of respiratory failure. Infections newly emerged in 6 cases during corticosteroid therapy (p < 0.05). Median serum granulocyte/macrophage colony-stimulating factor (GM-CSF) autoantibody levels were similar to previously reported data in a large cohort study.


The results demonstrate that corticosteroid therapy may worsen DSS of aPAP, increasing the risk for infections.
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