title: Characteristics and long-term outcome of 15 episodes of systemic lupus erythematosus-associated hemophagocytic syndrome.
abstract: Reactive hemophagocytic syndrome (HS) occurs mainly in the setting of serious infections and lymphomas. HS can occur in the course of 2 active systemic diseases, without simultaneous infection: adult Still disease and systemic lupus erythematosus (SLE). Observations of specific lupus-associated HS are rare, and the long-term outcome of these patients with active SLE is unknown. We retrospectively studied 15 episodes of SLE-associated HS in 12 patients (10 women, 2 men) and noted the long-term outcome. HS occurred at a mean age of 25 years. All patients were febrile with >or=2 cytopenias, and bone marrow aspiration indicated hemophagocytosis. HS revealed SLE in 9 patients and recurred in 3. The main features of SLE-associated HS were a low frequency of hepatosplenomegaly, a high frequency of heart involvement (5 pericarditis, 4 myocarditis requiring transfer to intensive care unit), and a low C-reactive protein level (mean, 15 mg/L). Cutaneous-mucous symptoms of SLE, arthritis, and nephritis were present respectively in 8 (53%), 6 (40%), and 4 (27%) episodes, but symptoms of SLE were absent in 4 episodes at admission. All patients had anti-nuclear antibodies when the HS occurred. Anti-double-stranded DNA antibodies were present in 12 episodes. Treatment was steroids in 14 cases but cyclophosphamide was the only treatment able to control HS in 2 cases. All the cases of SLE-associated HS were controlled by the immunosuppressive regimen. Intravenous immunoglobulins seemed poorly effective. No infectious agent was found. Clinical presentations of the 23 patients with SLE-associated HS described in the literature were reviewed and were similar to those of the current series. The mean follow-up was 88 months (range, 7-240 mo). One patient died at 15 months (sepsis). Among the 5 patients with a follow-up >8 years, 4 always had active disease. During the follow-up of SLE, immunosuppressive drugs were added in 8 patients (cyclophosphamide in 7, azathioprine in 3, mycophenolate mofetil in 2) with significant adverse drug reactions. In the long-term, SLE-associated HS seems to define a severe SLE form with frequent flares, possible HS recurrences, and the need for prolonged immunosuppression.
authors.affiliation: From Department of Internal Medicine (OL, VM, CG, JFD) and Department of Pediatrics (BBM), Bicêtre University Hospital, AP-HP, Le Kremlin Bicêtre; Department of Internal Medicine (MK, BG), Henri Mondor University Hospital, AP-HP, Créteil; Department of Internal Medicine (ZA, JCP), Pitié-Salpêtrière University Hospital, AP-HP, Paris, France; and Department of Internal Medicine (HH), Ibn Sina Hospital, Rabat, Morocco.
authors.forename: Olivier
authors.initials: O
authors.lastname: Lambotte
authors.forename: Mehdi
authors.initials: M
authors.lastname: Khellaf
authors.forename: Hicham
authors.initials: H
authors.lastname: Harmouche
authors.forename: Brigitte
authors.initials: B
authors.lastname: Bader-Meunier
authors.forename: Véronique
authors.initials: V
authors.lastname: Manceron
authors.forename: Cécile
authors.initials: C
authors.lastname: Goujard
authors.forename: Zahir
authors.initials: Z
authors.lastname: Amoura
authors.forename: Bertrand
authors.initials: B
authors.lastname: Godeau
authors.forename: Jean-Charles
authors.initials: JC
authors.lastname: Piette
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Delfraissy
mesh_terms: D000293:Adolescent; D000328:Adult; D002097:C-Reactive Protein / Q000032:analysis; D002648:Child; D003520:Cyclophosphamide; D016572:Cyclosporine / Q000627:therapeutic use; D018450:Disease Progression; D017050:Episode of Care; D005260:Female; D006801:Humans; D007362:Intensive Care Units; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use; D011379:Prognosis; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D016572:Cyclosporine; D003520:Cyclophosphamide; D002097:C-Reactive Protein; D011241:Prednisone; D008775:Methylprednisolone