A33-43 Recurrent Hemophagocytic Lymphohistiocytosis and Isolated Pulmonary Hypertension in a Young Woman With Adult-Onset Still’s Disease: A Case Report
Abstract
Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder often complicated by hemophagocytic lymphohistiocytosis (HLH). Pulmonary arterial hypertension (PAH) in AOSD is uncommon and poorly understood.
We report a 26-year-old woman with AOSD complicated by drug-associated immune reactions (DAIRs), recurrent HLH and subsequent PAH. She initially presented with sore throat and cervical lymphadenopathy which was partially responsive to short courses of corticosteroids, but did not completely resolve. Four months later, she developed quotidian fever and lymphadenopathy with neutrophilic leukocytosis, thrombocytosis, elevated liver enzymes, and elevated ferritin (∼5,000 ng/mL), together with a pruritic eruption not typical for AOSD. Infectious studies were negative, and lymph node and bone marrow biopsies were not concerning for malignancy. Yamaguchi criteria for AOSD were met. She was initially treated with anakinra and prednisone with improvement but later developed recurrent fevers prompting a switch to canakinumab. At seventeen months, she developed HLH with persistent fever, hepatosplenomegaly, ferritin >16,000 ng/mL, and elevated sIL2R 5777 U/ml. She was managed with corticosteroids and tocilizumab with incomplete response, and her tocilizumab was changed to tofacitinib. Eighteen months after onset, she developed dyspnea and new oxygen requirement at rest. CT angiography excluded pulmonary embolism and no abnormal parenchymal findings noted. Pulmonary…
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