Department of Endocrinology and Metabolism, Hospital das Clinicas, Faculdade de Medicina da Universidade de Sao Paulo, São Paulo, Brazil.
Division of Oncology, Instituto do Câncer do Estado de São Paulo, Universidade de São Paulo, São Paulo, Brazil.
J Clin Endocrinol Metab. 2019 Aug 1;104(8):3245-3248. doi: 10.1210/jc.2018-02221.
Lipodystrophy syndromes are rare disorders characterized by the selective loss of adipose tissue. We aimed to report a case of acquired generalized lipodystrophy possibly associated with nivolumab.
A woman was referred to our Endocrinology Department for uncontrolled diabetes mellitus. At 50 years of age, she was diagnosed with type 2 diabetes after a routine laboratory test and her diabetes was well controlled with low doses of metformin. In 2010, she was diagnosed with clear cell renal carcinoma. The cancer progressed in the following years, leading to the initiation of treatment with nivolumab in 2017. Two months later she presented with facial lipoatrophy, with loss of the buccal fat pads and prominent zygomatic arch. Her neck, shoulders, arms, and buttocks were also affected. Her diabetes control worsened. She received maximal doses of metformin and pioglitazone and was administered 1.5 units/kg/d insulin. Subcutaneous biopsy of medial surface of the arm revealed chronic lobular panniculitis. Despite nivolumab's possible involvement in the onset of lipodystrophy, the maintenance of nivolumab therapy was justified by the observed reduction in the progression of the cancer, combined with the lack of an alternative chemotherapy. The therapy was withdrawn after 8 months of treatment because of grade 3 hepatitis.
Anti-PD1 therapy has great potential. Early recognition of the onset of unusual collateral effects is important to improve decision making regarding the treatment of patients with tumors.
脂肪营养不良综合征是一种罕见的疾病，其特征是脂肪组织的选择性丧失。我们旨在报告一例可能与纳武利尤单抗相关的获得性全身性脂肪营养不良。
一名女性因糖尿病控制不佳被转至我院内分泌科就诊。50 岁时，她因常规实验室检查被诊断为 2 型糖尿病，血糖控制良好，仅使用小剂量二甲双胍。2010 年，她被诊断为肾透明细胞癌。此后几年癌症进展，导致 2017 年开始使用纳武利尤单抗治疗。两个月后，她出现面部脂肪萎缩，颊脂垫和颧骨突出。她的颈部、肩部、手臂和臀部也受到影响。她的糖尿病控制恶化。她接受了最大剂量的二甲双胍和吡格列酮治疗，并给予 1.5 单位/公斤/天的胰岛素。手臂内侧皮下活检显示慢性小叶性脂膜炎。尽管纳武利尤单抗可能与脂肪营养不良的发生有关，但考虑到癌症进展的减少以及缺乏替代化疗，维持纳武利尤单抗治疗是合理的。由于 3 级肝炎，治疗在 8 个月后被停用。
抗 PD-1 治疗具有巨大的潜力。早期识别不常见的副作用的发生对于改善肿瘤患者的治疗决策非常重要。
