Rao Vijay Kr, N Thanushree, Rs Manasa
Consultant, Department of Rheumatology, Divisha Arthritis and Medical Center, Bengaluru, Karnataka, India, Corresponding Author, Orcid: https://orcid.org/0009-0005-0926-4912.
Department of Clinical Pharmacology, Divisha Arthritis and Medical Center, Bengaluru, Karnataka, India.
J Assoc Physicians India. 2025 Jul;73(7S):33-36. doi: 10.59556/japi.73.0958.
Systemic lupus erythematosus (SLE) is an autoimmune disorder with heterogeneous phenotypes. The symptoms range from mild to life-threatening features. Azathioprine (AZA) is a routinely used immunosuppressive agent in mild to moderate SLE. Although bone marrow suppression is reported in AZA usage, severe alopecia is not very common with AZA.
We report a 45-year-old female with stable clinical and serological lupus maintained on mycophenolate mofetil 500 mg twice per day and hydroxychloroquine 200 mg once per day. She was lost to follow-up with us for >1 year. Since her disease was stable, we switched to AZA 25 mg twice per day to start with and escalated to 50 mg in the morning and 25 mg at night after 2 weeks, with an advice for 4-week follow-up after starting AZA. Hydroxychloroquine was continued at 200 mg once per day. No corticosteroids were used at this time as it was not deemed necessary. Monitoring blood tests for AZA were planned at 4 weeks. She presented at 6 weeks with severe leukopenia as summarized in the table of investigations below and the graph summarizing the trend in leukocyte counts after AZA usage. She was managed in the hospital with intravenous dexamethasone, antibiotic prophylaxis, and hematology consultation, who opined as AZA-induced severe bone marrow suppression and severe alopecia due to AZA. Bone marrow examination was not deemed necessary by the hematologist. AZA was stopped in the hospital and mycophenolate mofetil was prescribed in the immediate follow-up after discharge, as she had previously responded to this drug. Hydroxychloroquine continued throughout her hospital stay. Although her blood counts responded very well after AZA withdrawal, it took nearly 3 months for her to have her normal scalp hair. One of the major differentials that was considered was SLE flare-up, but her clinical features and serology did not support a lupus flare.
Bone marrow suppression is a severe complication of AZA in SLE. Leukopenia and hair loss are the major adverse effects reported during the therapy of AZA. It is sensible to recognize this relationship as prompt diagnosis and treatment is crucial.
系统性红斑狼疮(SLE)是一种具有异质性表型的自身免疫性疾病。症状范围从轻微到危及生命。硫唑嘌呤(AZA)是轻度至中度SLE中常用的免疫抑制剂。尽管有报道称使用AZA会出现骨髓抑制,但严重脱发在使用AZA时并不常见。
我们报告一名45岁女性,临床和血清学狼疮病情稳定,每天服用两次500毫克霉酚酸酯和每天一次200毫克羟氯喹。她失访我们超过1年。由于她的病情稳定,我们开始每天两次服用25毫克AZA,2周后增至早上50毫克、晚上25毫克,并建议在开始使用AZA后进行4周随访。羟氯喹继续每天一次服用200毫克。此时未使用皮质类固醇,因为认为没有必要。计划在4周时监测AZA的血液检查。她在6周时出现严重白细胞减少,如下表所示,以及总结使用AZA后白细胞计数趋势的图表。她在医院接受了静脉注射地塞米松、抗生素预防和血液学咨询治疗,血液科医生认为这是AZA引起的严重骨髓抑制和AZA导致的严重脱发。血液科医生认为没有必要进行骨髓检查。在医院停用了AZA,出院后立即随访时开了霉酚酸酯,因为她之前对这种药物有反应。整个住院期间羟氯喹继续服用。尽管停用AZA后她的血细胞计数恢复得很好,但她的头皮毛发恢复正常花了近3个月时间。考虑的主要鉴别诊断之一是SLE病情复发,但她的临床特征和血清学不支持狼疮复发。
骨髓抑制是SLE患者使用AZA的严重并发症。白细胞减少和脱发是AZA治疗期间报告的主要不良反应。认识到这种关系是明智的,因为及时诊断和治疗至关重要。