Efficacy of Cyclosporine in the Induction and Maintenance of Remission in a
Systemic Lupus Erythematosus Patient Presenting with Macrophage-Activating
Syndrome
#MMPMID29552369
Cruz-Pérez F
; Vilá S
; Ríos G
; Vilá LM
Case Rep Rheumatol
2018[]; 2018
(?): 1961585
PMID29552369
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Macrophage-activating syndrome (MAS) is a rare condition characterized by
dysfunctional macrophage activation leading to overproduction of cytokines and
phagocytosis of erythrocytes, leukocytes, and platelets. MAS is associated with
infectious diseases, malignancies, and autoimmune rheumatic disorders. Herein, we
present a 22-year-old Hispanic woman with SLE who was hospitalized because of a
three-week history of fever, fatigue, polyarthralgia, nausea, and abdominal pain.
Initial laboratories showed severe pancytopenia with marked elevation of liver
enzymes and ferritin levels. Bone marrow biopsy revealed macrophages with
engulfed erythrocytes consistent with MAS. The patient was treated with high-dose
corticosteroids, intravenous immunoglobulins, and cyclosporine 3?mg/kg/day. She
had a remarkable clinical response to this therapy. She was continued on
cyclosporine, and prednisone dose was gradually decreased to 7.5?mg daily without
experiencing recurrent disease. She remained in full clinical remission for 12
months. Our case, together with other reports, suggests that combination therapy
with corticosteroids, immunoglobulins, and cyclosporine appears to be effective
for patients with SLE-associated MAS. Furthermore, cyclosporine seems to be a
good drug for maintenance of remission.
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