Successful treatment of refractory lupus-associated haemophagocytic lymphohistiocytosis with infliximab

Hemophagocytic Lymphohistiocytosis in a Rheumatoid Arthritis Patient Treated with Infliximab

“…In contrast, four cases of HLH in systemic lupus erythematosus (SLE) and JIA patients who received anti-TNFtherapy have been reported (13)(14)(15)(16), all of which were considered as autoimmune-associated hemophagocytic syndrome (AAHS) (17,18). These patients received infliximab and etanercept for HLH, which resulted in cure.…”

Etanercept for the treatment of intractable hemophagocytic syndrome with systemic lupus erythematosus

“…Steroid pulse therapy, massive IVIG therapy, and administration of cyclosporine A and methotrexate, which is effective for AOSD associated with hypercytokinemia [6] had been used, but none of these drugs was sufficiently effective. TNF-a inhibition by infliximab [7,8] and etanercept [9] has been reported to be effective for intractable HPS complicating SLE. Etanercept was selected rather than frequent steroid pulse therapy and the administration of etoposide, cyclophosphamide, and azathioprine, because the patient had a history of osteonecrosis of the femoral head and desired to have children; therefore, treatment using agents that posed a slight risk to the ovaries was not conducted.…”

“…2 Clinical course of the patient. PSL prednisolone, mPSL methylprednisolone, IVIG intravenous immunoglobulin, CSA cyclosporine A, MTX methotrexate, ETA etanercept, wk week, CRP C-reactive protein, TNF-a tumor necrosis factor-a, PCT procalcitonin, WBC white blood cell count, Plt platelet count, IL-6 interleukin-6, CD64 expression of CD64 on peripheral blood neutrophils a TNF-a inhibitor have manifested HPS [10,11], and Ideguchi et al [7] noted this inconsistency. The major cause of HPS in such circumstances may be infection due to immunosuppression, but the definitive cause is unknown.…”

“…Ideguchi et al [7] measured serum cytokine levels before and after therapy for HPS complicating SLE, and observed the involvement of inflammatory cytokines and the efficacy of TNF-a inhibition therapy. In addition to investigating serum TNF-a and IL-6, as inflammatory markers, before and after treatment we investigated the expression of CD64 on peripheral blood neutrophils (which is useful for differentiation between rheumatoid arthritis and infection [12]), as well as investigating the serum procalcitonin (PCT) level, which rises in systemic bacterial infection and sepsis [13].…”

Traitements anti-TNFα dans les maladies auto-immunes et/ou inflammatoires systémiques

“…Bien que les taux circulants de TNFa soient corrélés à l'activité de la maladie, les anti-TNFa ont été très peu utilisés dans le traitement du LES. Une efficacité de l'infliximab a été rapportée dans des observations isolées de patients ayant un LES compliqué d'activation macrophagique [57,58] ou dans le cas de LES réfractaire aux immunosuppresseurs [59,60]. Par ailleurs, une étude ouverte portant sur 6 patients ayant une atteinte rénale ou articulaire modérée, et traités par 4 perfusions d'infliximab a montré une efficacité de l'infliximab sur le nombre d'articulations inflammatoires (3/3 patients) et sur la protéinurie (4/4 patients) [61].…”