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Case report
Secondary pulmonary alveolar proteinosis during corticosteroid therapy for organising pneumonia associated with myelodysplastic syndrome
  1. Daiki Inoue1,
  2. Satoshi Marumo1,
  3. Haruyuki Ishii2 and
  4. Motonari Fukui1
  1. 1 Respiratory Disease Center, Tazuke Kofukai Medical Research Institute, Kitano Hospital, Osaka, Japan
  2. 2 Department of Respiratory Medicine, Kyorin University School of Medicine, Tokyo, Japan
  1. Correspondence to Dr Satoshi Marumo, marumosatoshi{at}


Myelodysplastic syndrome (MDS) is frequently complicated by pulmonary disease. Here, we describe secondary pulmonary alveolar proteinosis (sPAP) that developed during corticosteroid therapy for organising pneumonia (OP) associated with MDS. A 75-year-old woman with MDS complained of cough for 2 weeks. Chest CT showed bilateral patchy consolidations with reversed halo sign. Bronchoalveolar lavage (BAL) examination showed remarkably increased cell density with an increased lymphocyte proportion. Abnormal radiological findings improved rapidly on administration of systemic corticosteroid under the diagnosis of OP; however, they relapsed a few weeks later. Transbronchial lung biopsy showed periodic acid-Schiff stain-positive amorphous materials. Autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF) in serum and BAL fluid (BALF) were both negative, while GM-CSF level in BALF was elevated. The patient was diagnosed with sPAP. When chest radiological findings show exacerbation during corticosteroid therapy for OP in a patient with MDS, physicians should consider sPAP complication as a differential diagnosis.

  • interstitial lung disease
  • haematology (incl blood transfusion)
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  • Contributors DI treated the patient consulting to HI and MF. DI wrote the manuscript. SM and MF corrected the manuscript. HI advised on the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Patient consent for publication Next of kin consent obtained.

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