Treatment of secondary pulmonary alveolar proteinosis (SPAP) focuses on managing the underlying disease, typically myelodysplastic syndrome. However, immunosuppressive therapy may worsen the condition in autoimmune disease-associated SPAP. We report the case of a 74-year-old woman with hemophagocytic syndrome and adult-onset Still's disease, who developed ground-glass opacities and consolidations during immunosuppressive treatment. SPAP was confirmed by a biopsy and negative for anti-granulocyte-macrophage colony-stimulating factor antibodies. Although whole-lung lavage was considered if SPAP worsened significantly, no further deterioration was observed with tapering of prednisolone. A literature review and details of this case highlight the importance of attenuating immunosuppressive therapy, rather than its intensification, for SPAP in autoimmune diseases.
Keywords: hemophagocytic syndrome; immunosuppressive therapy; pulmonary alveolar proteinosis.