[Hemophagocytic syndrome complicating adult's seropositive rheumatoid arthritis].

Clinique médicale 1, CHU A.-Le-Dantec, Dakar, Sénégal. <>
La Revue de Médecine Interne (Impact Factor: 1.07). 12/2004; 25(11):826-8.
Source: PubMed


Macrophage activation syndrome (MAS) is a severe complication of chronic rheumatic diseases, particularly juvenile rheumatoid arthritis. However, MAS is rarely described in adult rheumatoid polyarthritis.
We report a case of MAS complicating a seropositive rheumatoid polyarthritis after 20 years of evolution. Pancytopenia with fever, renal failure and hepatic dysfunction revealed the disease that was confirmed by multiple macrophages and monocytes invading the bone marrow specimen.
Outcome has been spectacular under corticosteroids.

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    ABSTRACT: We report a patient with rheumatoid arthritis (RA) who showed bicytopenia with hyperferritinemia and hepatic dysfunction ascribable to hemophagocytic syndrome (HPS) 2 weeks after commencement of bucillamine. Pathology of the bone marrow showing infiltration of macrophages confirmed the diagnosis of HPS. On the basis of renal dysfunction with an increase in fibrin degradation products, disseminated intravascular coagulation was considered to be concurrent with HPS. Oral prednisolone and cyclosporine A were started right after cessation of bucillamine, and yielded complete normalization of hepatic and renal function and hematology. As there was neither disease activity of RA nor associated infection throughout the clinical course, bucillamine was suspected of being the cause of HPS in our patient. HPS is a very rare complication in RA, but should be actively considered when abnormalities in laboratory data, especially pancytopenia and hepatic dysfunction, quickly worsen.
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    ABSTRACT: BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH), is a potentially fatal hyperinflammatory syndrome characterized fever, hepatosplenomegaly, and cytopenias. HLH can be either primary, with a genetic aetiology, or secondary, associated with malignancies, autoimmune diseases, or infections. Among rheumatic disorders, HLH occurs most frequently in systemic juvenile idiopathic arthritis. AIM: To draw attention on this severe syndrome that may often go undiagnosed in patient with rheumatic diseases. MATERIALS AND METHODS: PubMed search was performed by combining the terms (haemophagocytic, haemophagocytosis, hemophagocytosis, hemophagocytic, erythrophagocytosis, macrophage activation syndrome) and (rheumatic, rheumatologic, arthritis, lupus, Sjögren's syndrome, scleroderma, polymyositis, dermatomyositis, polymyalgia rheumatic, mixed connective tissue disease, polychondritis, sarcoidosis, polyarteritis nodosa, Henoch-Schönlein, serum sickness, wegener's granulomatosis, giant cell arteritis, temporal arteritis, Takayasu's arteritis, Behçet's syndrome, Kawasaki, Buerger's). RESULTS: 117 papers describing 421 patients were considered. HLH was described in systemic lupus erythematosus in 94 patients, in Still's disease in 37 patients, in rheumatoid arthritis in 13 patients, in systemic juvenile arthritis in 219 patients, in dermatomyositis in 7 patients, in Kawasaki disease in 25 patients, in systemic sclerosis in 5 patients, in Behcet disease in one patient, in polyarteritis nodosa in 6 patients, in ankylosing spondylitis in 2 patients, in mixed connective tissue disease in one patient, in sarcoidosis in 5 patients, in Sjögren's syndrome in 3 patients, in Wegener's granulomatosis in one patient, and in unclassifiable disorders in two patients. CONCLUSIONS: HLH occurring in the course of rheumatic diseases is an important and often underdiagnosed clinical entity, which can affect prognosis.
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    ABSTRACT: Introduction: We reported a case of hemophagocytic syndrome complicating microscopic polyangitis presented by crescentic glomerulonephritis. Case Presentation: A 22-year-old female patient originated from Dakar, Senegal presented with nephrotic syndrome and rapidly progressive glomerulonephritis. On physical examination, we noticed hyperchromic diffuse punctilious purpura skin lesions predominant on the trunk, the neck and the upper thigh. Immunology investigations revealed strongly positive anti SSA/Ro and anti-SSB. Anti-neutrophil cytoplasmic antibodies had positive results with a peri-nuclear type fluorescence, specific to myeloperoxidase. In optic microscopy, renal biopsy showed a crescentic glomerulonephritis with circumferential cellular and fibrous proliferation affecting 85% of glomeruli. The diagnosis of microscopic polyangitis with renal and skin involvement was retained. The patient received methylprednisolone and cyclophosphamide 700 mg/m2 every 15 days for the first 3 pulses and every 21 days thereafter. After the 5th month, she developed obnubilation, fever and central pancytopenia. Bone marrow aspiration was performed, which showed medullary invasion by macrophages with signs of hemophagocytosis. Diagnosis of hemophagocytic syndrome complicating a microscopic polyangitis was retained and methylprednisolone pulses started. The patient was under hemodialysis after follow-up of about 9 months with stable clinical state. Conclusions: The occurrence of SAM in pauci-autoimmune vasculitis is rarely described, particularly in Africa. Our case is an illustration of the reality of this association.
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