title: Clinical characteristics and follow-up analysis of adult-onset Still's disease complicated by hemophagocytic lymphohistiocytosis.
abstract: We evaluated clinical characteristics and prognosis for adult-onset Still's disease (AOSD) complicated by hemophagocytic lymphohistiocytosis (HLH). We retrospectively identified cases of AOSD with (n = 10) and without (n = 305) HLH complications. We reviewed their medical records, completed follow-up through outpatient clinic and telephone interviews, and analyzed their clinical symptoms, signs, laboratory test results, treatments, and prognosis. More AOSD patients with HLH developed hepatomegaly, bleeding, serositis, and neurologic symptoms than those without HLH, and they more commonly presented with leukopenia, thrombocytopenia, severe anemia, severe liver function abnormalities, decreased fibrinogen, elevated immunoglobulin, and bone marrow hemophagocytosis. The ten patients with AOSD complicated by HLH were treated with high-dose steroids or pulse steroid therapy, and eight of them also received cytotoxic drugs, while biological agents showed poor response. Follow-up results indicated that AOSD patients overall had good prognosis, while those with HLH showed worse prognosis, including higher relapse and readmission rates and increased mortality. In patients with AOSD, unexplained decreased blood cells, severe liver dysfunction, and/or hemophagocytosis in the bone marrow should be considered as signs of HLH complication. Patients with AOSD complicated by HLH have worse prognosis and higher relapse rates compared to AOSD patients without HLH complications. Thus, these patients should undergo frequent and careful follow-up.
authors.affiliation: Department of General Internal Medicine, Peking Union Medical College Hospital (PUMCH), Chinese Academy of Medical Science (CAMS), Peking Union Medical College (PUMC), Beijing, China.
authors.forename: Yun
authors.initials: Y
authors.lastname: Zhang
authors.affiliation: Department of Internal Medicine, Peking Union Medical College Hospital (PUMCH), Academy of Medical Science (CAMS), Peking Union Medical College (PUMC), Beijing, China.
authors.forename: Yingyun
authors.initials: Y
authors.lastname: Yang
authors.affiliation: Peking Union Medical College (PUMC), Chinese Academy of Medical Science (CAMS), Beijing, China.
authors.forename: Yujia
authors.initials: Y
authors.lastname: Bai
authors.affiliation: Peking Union Medical College (PUMC), Chinese Academy of Medical Science (CAMS), Beijing, China.
authors.forename: Dan
authors.initials: D
authors.lastname: Yang
authors.affiliation: Peking Union Medical College (PUMC), Chinese Academy of Medical Science (CAMS), Beijing, China.
authors.forename: Yangyang
authors.initials: Y
authors.lastname: Xiong
authors.affiliation: Department of General Internal Medicine, Peking Union Medical College Hospital (PUMCH), Chinese Academy of Medical Science (CAMS), Peking Union Medical College (PUMC), Beijing, China. zxjpumch@126.com.
authors.forename: Xuejun
authors.initials: X
authors.lastname: Zeng
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000740:Anemia / Q000150:complications; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007970:Leukopenia / Q000150:complications; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000188:drug therapy; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012008:Recurrence; D012189:Retrospective Studies; D016706:Still's Disease, Adult-Onset / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D063189:Symptom Assessment; D013921:Thrombocytopenia / Q000150:complications; D055815:Young Adult
chemical_list: D005938:Glucocorticoids
keywords: Adult-onset Still’s disease; Complication; Follow-up; Hemophagocytic lymphohistiocytosis
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