Patel Anandkumar, Patel Umangkumar M, Sojitra Vani
Medicine, Maharshi Hospital Private Limited, Surendranagar, IND.
Neurology, Shalby Multispeciality Hospitals, Ahmedabad, IND.
Cureus. 2023 May 6;15(5):e38651. doi: 10.7759/cureus.38651. eCollection 2023 May.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of disorders that causes severe small-vessel inflammation with systemic manifestations. There are three subtypes of AAV, namely granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic GPA (EGPA). The most commonly affected organs are the upper and lower respiratory tract and the kidneys with occasional and varied neurological manifestations. Here we report a case of a 61-year-old female who presented with a one-month history of numbness, paresthesia, and asymmetric distal weakness of both lower limbs without any bladder or bowel involvement. Similar complaints appeared in her upper limbs three days prior to admission. She also suffered from myalgia, arthralgia, reduced appetite, and lost 8-10 kg weight over the past six months. Her nerve conduction study (NCV) revealed asymmetrical, predominantly motor, mixed, axonal and demyelinating polyneuropathy affecting both lower limbs, which was suggestive of mononeuritis multiplex. After a detailed workup, she tested strongly positive for cytoplasmic ANCA (c-ANCA). Although there was no clinical involvement of the respiratory tract, a contrast-enhanced computed tomography scan of the thorax and abdomen showed multifocal subpleural and lung parenchymal soft tissue lesions and mediastinal and bilateral hilar lymphadenopathy suggestive of a granulomatous lesion. She was diagnosed with the GPA variant of ANCA-associated vasculitis. Remission induction was achieved with high-dose methylprednisolone and cyclophosphamide along with alternate-day cotrimoxazole. Remission was maintained with tapering doses of steroid and mycophenolate mofetil with a slow but sustained recovery. On follow-up after one year, she walked without support with mild residual burning paresthesia in both feet. This case highlights the fact that neurological symptoms can be the presenting manifestation of AAV, and clinicians should have a high level of suspicion for AAV in patients presenting with mononeuritis multiplex, especially after ruling out common causes. By considering such etiologies, it may be possible to diagnose the condition at an earlier stage and initiate treatment to prevent potential pulmonary or renal damage.
抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）是一组导致严重小血管炎症并伴有全身表现的疾病。AAV有三种亚型，即肉芽肿性多血管炎（GPA）、显微镜下多血管炎（MPA）和嗜酸性肉芽肿性多血管炎（EGPA）。最常受累的器官是上、下呼吸道和肾脏，偶尔也有各种不同的神经表现。在此，我们报告一例61岁女性患者，她有1个月的双下肢麻木、感觉异常和不对称性远端无力病史，无膀胱或肠道受累。入院前3天，她的上肢也出现了类似症状。她还伴有肌痛、关节痛、食欲减退，在过去6个月体重减轻了8 - 10千克。她的神经传导研究（NCV）显示双下肢不对称、以运动为主、混合性、轴索性和脱髓鞘性多发性神经病，提示多发性单神经炎。经过详细检查，她的胞浆型ANCA（c-ANCA）检测呈强阳性。尽管呼吸道无临床受累，但胸部和腹部增强计算机断层扫描显示多灶性胸膜下和肺实质软组织病变以及纵隔和双侧肺门淋巴结肿大，提示肉芽肿性病变。她被诊断为ANCA相关性血管炎的GPA变异型。通过大剂量甲基强的松龙和环磷酰胺以及隔日服用复方新诺明实现了诱导缓解。通过逐渐减量的类固醇和霉酚酸酯维持缓解，患者恢复缓慢但持续。1年后随访时，她无需支撑就能行走，双足有轻度残留的烧灼样感觉异常。该病例突出了神经症状可能是AAV的首发表现这一事实，对于出现多发性单神经炎的患者，临床医生应高度怀疑AAV，尤其是在排除常见病因之后。通过考虑此类病因，有可能在更早阶段诊断病情并启动治疗，以预防潜在的肺部或肾脏损害。
