Department of Nephrology, The First Affiliated Hospital of Zhejiang Chinese Medical University (Zhejiang Provincial Hospital of Chinese Medicine), Hangzhou, Zhejiang, China.
Department of Traditional Chinese Medicine, Jiande First People's Hospital, Jiande, Hangzhou, Zhejiang, China.
Medicine (Baltimore). 2024 Jul 5;103(27):e38790. doi: 10.1097/MD.0000000000038790.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare disease characterized by the inflammation and destruction of small blood vessels and circulating ANCAs. Drugs such as antithyroid drugs (ATDs), especially propylthiouracil (PTU), have been used for the production of ANCAs and cause the development of drug-induced AAV. The pathogenesis of this disease is unclear but could be related to the physiological processes affecting the degradation of neutrophil extracellular traps (NETs). At present, PTU is widely used in patients with Graves' disease (GD) who are preparing for pregnancy and whose condition has not been controlled. Once drug-induced AAV has occurred with important organ damage, considering NETs have a significant role in the immune system, whether the cessation of drugs could stop the progression of organ damage is unclear, and a consensus regarding standard treatment has not been established.
In this case report, a female patient who planned pregnancy was hospitalized with multiple joint pain, impaired renal function, and hematuria. Immunofluorescence of the renal biopsy demonstrated spherical and diffuse mesangial distribution of IgA (3+). Autoimmune serology demonstrated positivity for autoantibodies against p-ANCA and an anti-MPO titer 74.72 RU/mL.
She was diagnosed with PTU-induced p-ANCA-associated and IgA-associated vasculitis (IgAV).
The patient accepted low doses of glucocorticoid, immunosuppressive therapy and RAI treatment.
Both her kidney function and thyroid function remained were on the mend.
The authors believe that this type of patient needs to fully consider their pregnancy preparation needs, suspend pregnancy when a small chance of GD remission is indicated, and avoid the use of drugs with reproductive toxicity and other serious adverse events. The multidisciplinary combination therapy of low-dose glucocorticoids and immunosuppressants combined with iodine radiotherapy is one reasonable scheme. At the same time, it is necessary to eliminate the organ damage caused by other reasons. This report provides a clinical treatment basis for patients with drug-induced vasculitis manifestations who cannot receive an accurate diagnosis.
抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）是一种罕见疾病，其特征为小血管的炎症和破坏以及循环 ANCAs。抗甲状腺药物（ATD），特别是丙基硫氧嘧啶（PTU）等药物已被用于产生 ANCAs，并导致药物诱导的 AAV 的发展。该疾病的发病机制尚不清楚，但可能与影响中性粒细胞细胞外陷阱（NETs）降解的生理过程有关。目前，PTU 广泛用于准备怀孕且病情未得到控制的格雷夫斯病（GD）患者。一旦发生具有重要器官损害的药物诱导性 AAV，鉴于 NETs 在免疫系统中具有重要作用，停止药物治疗是否会阻止器官损害的进展尚不清楚，也尚未就标准治疗达成共识。
在本病例报告中，一名计划怀孕的女性因多发性关节痛、肾功能受损和血尿住院。肾活检的免疫荧光显示 IgA 呈球形和弥漫性系膜分布（3+）。自身免疫血清学检查显示针对 p-ANCA 的自身抗体和抗 MPO 滴度为 74.72 RU/mL 阳性。
她被诊断为 PTU 诱导的 p-ANCA 相关性和 IgA 相关性血管炎（IgAV）。
患者接受了低剂量糖皮质激素、免疫抑制治疗和 RAI 治疗。
她的肾功能和甲状腺功能均在好转。
作者认为，此类患者需要充分考虑其妊娠准备需求，在 GD 缓解可能性较小的情况下暂停妊娠，并避免使用具有生殖毒性和其他严重不良事件的药物。低剂量糖皮质激素和免疫抑制剂联合碘放疗的多学科联合治疗是一种合理的方案。同时，有必要消除其他原因引起的器官损害。本报告为不能明确诊断的药物性血管炎表现患者提供了临床治疗依据。
