Abstract
Dear Editor,Since systemic lupus erythematosus (SLE) is a complexsystemic autoimmune disease associated with frequentXares of manifesting various symptoms, eVective and safelong-term immunosuppressive therapy is needed to achievea favorable outcome. Although intermittent monthly use ofintravenous pulse cyclophosphamide (CPA) is reportedlyeVective even for patients with pediatric-onset SLE [1],CPA is a potent immunosuppressive agent associated withclinical toxicities, including myelotoxicity, gonadal toxicityand increased risk of secondary malignancy [2]. Thus, wenow favor using the calcineurin inhibitor tacrolimus (Tac)for maintenance therapy in young SLE patients with lupusnephritis [3, 4]. However, the optimal treatment strategy forSLE Xare while on Tac has yet to be determined. Wetreated a Japanese girl with lupus nephritis who had beensuccessfully managed with prednisolone (PDN) plus Tac.However, she developed severe thrombocytopenia withhypocomplementemia following PDN tapering. She wasthen successfully treated with a selective inhibitor of ino-sine monophosphate dehydrogenase in the de novo path-way mizoribine (MZR) [5] in addition to the PDN and Tacafter transient escalation of the PDN dose. This novel com-bination therapy proved eVective for SLE Xare withoutclinical toxicity, despite subsequent tapering of the PDNdose. This report may lend further support to the eYcacy ofmultidrug therapy consisting of two immunosuppressiveagents with diVerent modes of action in combination withPDN [6].An 11-year-old Japanese girl with a 2-year history ofSLE with International Society of Nephrology/RenalPathology Society classi Wcation class V lupus nephritis wasreferred to our hospital because of sudden development ofrefractory epistaxis due to severe thrombocythemia (plate-let count, 7,000/ l) associated with serum C4 depression(6 mg/dl, normal 13–35 mg/dl). The patient had been suc-cessfully managed with PDN, 30 mg daily combined withTac 4.5 mg (0.075 mg/kg) daily with blood levels of the lat-ter ranging from 2.9 to 4.6 ng/ml. Renal functions remainedwithin the normal range. Although the initially observedsevere urinary abnormalities (urine protein excretion, maxi-mum 2.0 g per day) and serum anti-dsDNA antibody positivityshowed complete resolution following immunosuppressivetreatment, she later developed refractory epistaxis afterPDN tapering to 7.5 mg daily. Immunologic examinationrevealed increased serum platel ets-associated IgG elevation(56.4 ng/10
Published Version
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