Woller Samantha, Sood Akshat, Maclean Alyssa K W
Department of Internal Medicine, Creighton University School of Medicine, Omaha, Nebraska, USA.
Creighton University School of Medicine, Omaha, Nebraska, USA.
Proc (Bayl Univ Med Cent). 2023 Jul 24;36(5):647-649. doi: 10.1080/08998280.2023.2230830. eCollection 2023.
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune condition characterized by necrotizing inflammation of small vessels throughout the body. Pharmaceutical agents have been noted as an emerging etiology. This case presents a 41-year-old woman with a longstanding history of Graves' disease who previously failed other interventions and was started on propylthiouracil (PTU) nearly 2 years prior to symptom onset. The patient presented with severely pruritic purpuric lesions on her lower extremities that transformed into large bullae and became extremely painful. A thorough workup revealed only slightly elevated perinuclear ANCA and a mild protein S deficiency. Tissue biopsy was consistent with thrombotic vasculitis. A presumptive clinical diagnosis of PTU-induced vasculitis was made. Because the condition is relatively uncommon, the best course of treatment has not clearly been defined. Though PTU was immediately discontinued, the patient also required corticosteroids and referral for tissue debridement. While some cases have had symptom resolution after cessation of PTU, this case adds to a growing body of evidence for the timely use of corticosteroids in controlling PTU-induced vasculitis.
抗中性粒细胞胞浆抗体(ANCA)相关性血管炎是一种自身免疫性疾病,其特征为全身小血管的坏死性炎症。药物已被确认为一种新出现的病因。本病例报告了一名41岁女性,她有长期的格雷夫斯病病史,之前其他治疗均失败,在症状出现前近2年开始使用丙硫氧嘧啶(PTU)。患者下肢出现严重瘙痒的紫癜性皮损,后转变为大疱且疼痛剧烈。全面检查仅发现核周型ANCA轻度升高和轻度蛋白S缺乏。组织活检符合血栓性血管炎。临床初步诊断为PTU诱发的血管炎。由于这种情况相对不常见,最佳治疗方案尚未明确界定。虽然立即停用了PTU,但患者还需要使用糖皮质激素并转诊进行组织清创。虽然有些病例在停用PTU后症状缓解,但本病例进一步证明了及时使用糖皮质激素控制PTU诱发血管炎的证据越来越多。