Reactive haemophagocytic syndrome in adult-onset Still’s disease: a report of six patients and a review of the literature

• Published in: Annals of the rheumatic diseases 2006-12, Vol.65 (12), p.1596-1601
• Main Authors: Arlet, J-B, Le Thi Huong, D, Marinho, A, Amoura, Z, Wechsler, B, Papo, T, Piette, J-C
• Format: Article
• Language: English
• Subjects: acute respiratory distress syndrome; Adult; adult-onset Still’s disease; Adults; Aged; AOSD; ARDS; Biological and medical sciences; Cytotoxicity; DIC; Disease; Diseases of the osteoarticular system; disseminated intravascular coagulopathy; Drug dosages; Extended Report; Family medical history; Female; Fever; Glucocorticoids - therapeutic use; Humans; Immunoglobulins, Intravenous - therapeutic use; Inflammatory joint diseases; intravenous immunoglobulin; IVIG; Laboratories; Literature reviews; Lymphocytes; Lymphohistiocytosis, Hemophagocytic - diagnosis; Lymphohistiocytosis, Hemophagocytic - etiology; Lymphohistiocytosis, Hemophagocytic - therapy; Male; Medical sciences; Middle Aged; Patients; reactive haemophagocytic syndrome; Respiratory distress syndrome; Retrospective Studies; RHS; SOJIA; Statistical analysis; Still's Disease, Adult-Onset - complications; systemic onset juvenile idiopathic arthritis; TNFα; Treatment Outcome; Triglycerides; tumour necrosis factor α
• ISSN: 0003-4967; 1468-2060
• DOI: 10.1136/ard.2005.046904

Objective: To examine the prevalence and characteristics of patients with reactive haemophagocytic syndrome (RHS) complicating adult-onset Still’s disease (AOSD). Methods: Of 50 patients with AOSD fulfilling Yamaguchi and Fautrel criteria followed in our department, clinical and laboratory data, course and treatment of six patients with histologically proven RHS and without any obvious cause other than AOSD were retrospectively recorded. Results: RHS led to AOSD in two cases, whereas it appeared after a mean duration of 3.5 years from onset of AOSD in the other cases. The main symptoms were fever (n = 6), polyarthralgias or myalgias (n = 4), lymphadenopathy or splenomegaly (n = 3), pharyngitis (n = 3), rash (n = 3), pleuritis (n = 3), hepatomegaly (n = 1), normal or low leucocyte count (n = 4), anaemia (n = 6), lymphocytopenia (n = 6), thrombocytopenia (n = 4), hyperbasophilic lymphocytes (n = 2), abnormal liver function tests (n = 6) and increased serum triglyceride level (n = 6). Serum ferritin concentration was constantly increased (>10 000 µg/l in five cases, with