TY - JOUR AU - Hirose, Masaki AU - Arai, Toru AU - Sugimoto, Chikatoshi AU - Takimoto, Takayuki AU - Sugawara, Reiko AU - Minomo, Shojiro AU - Shintani, Sayoko AU - Takeuchi, Naoko AU - Katayama, Kanako AU - Inoue, Yasushi AU - Kagawa, Tomoko AU - Kasai, Takahiko AU - Akira, Masanori AU - Inoue, Yoshikazu PY - 2021 DA - 2021/03/02 TI - B cell‐activating factors in autoimmune pulmonary alveolar proteinosis JO - Orphanet Journal of Rare Diseases SP - 115 VL - 16 IS - 1 AB - Autoimmune pulmonary alveolar proteinosis (APAP) results from the suppression of granulocyte-macrophage colony-stimulating factor (GM-CSF) signaling by a neutralizing autoantibody against GM-CSF. B cell-activating factor (BAFF) and a proliferation-inducing ligand (APRIL) are involved in immunoglobulin G production and are overproduced in various autoimmune disorders. We hypothesized that BAFF and/or APRIL levels would be elevated in serum and bronchoalveolar lavage fluid (BALF) and serum and BALF levels of BAFF and APRIL respond to the treatments (whole lung lavage (WLL) or inhalation of recombinant human granulocyte-macrophage colony-stimulating factor (GM-CSF)) in patients with APAP. SN - 1750-1172 UR - https://doi.org/10.1186/s13023-021-01755-y DO - 10.1186/s13023-021-01755-y ID - Hirose2021 ER -