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Case Reports
Autoimmune Pulmonary Alveolar Proteinosis Complicated by Myelodysplastic Syndrome: A Case Report.
- Minako Shimaya, Yuji Inagaki, Toru Arai, Mayu Kawakami, Naoko Takeuchi, Hiromitsu Sumikawa, Shigeki Shimizu, Takayuki Takimoto, and Yoshikazu Inoue.
- Department of Internal Medicine, NHO Kinki Chuo Chest Medical Center, Japan.
- Intern. Med. 2024 May 15; 63 (10): 145114571451-1457.
AbstractPulmonary alveolar proteinosis (PAP) is characterized by an abnormal surfactant accumulation in peripheral air spaces. Autoimmune PAP (APAP) results from macrophage dysfunction caused by anti-granulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibodies, and the presence of antibodies more than the cutoff value is specific for APAP. In contrast, secondary PAP (SPAP) does not require anti-GM-CSF autoantibodies and is complicated by other diseases, including myelodysplastic syndrome (MDS). A 73-year-old man with anemia and thrombocytopenia was diagnosed with APAP and MDS simultaneously. The measurement of serum anti-GM-CSF autoantibodies is important for the correct diagnosis and management of PAP, even with an established diagnosis of underlying SPAP-suggestive disease.
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