Lankapothu Prem Balaji Reddy, Chinnadurai Saranya, Bhaskaran Shrinidhi, Bathena Arun Kumar, Dasi Sharath Chandra
General Medicine, Saveetha Medical College and Hospitals, Saveetha Institute of Medical and Technical Sciences, Saveetha University, Chennai, IND.
Rheumatology, Saveetha Medical College and Hospitals, Saveetha Institute of Medical and Technical Sciences, Saveetha University, Chennai, IND.
Cureus. 2024 Sep 26;16(9):e70261. doi: 10.7759/cureus.70261. eCollection 2024 Sep.
Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a rare autoimmune vasculitis that primarily affects small to medium-sized blood vessels, typically involving the respiratory tract and kidneys. However, central nervous system involvement, particularly in the form of pachymeningitis, is an exceptionally rare presentation. This case report details a 55-year-old female who presented with pachymeningitis as the initial manifestation of GPA. The patient exhibited non-specific symptoms, including ear pain, voice changes such as hoarseness of voice, and dysphagia, suggestive of neurological deficits affecting lower cranial nerves IX, X, and XII. Diagnostic imaging, particularly MRI, revealed characteristic smooth dural thickening and enhancement, suggesting an inflammatory etiology. Laboratory investigations, including positive cytoplasmic-antineutrophil cytoplasmic autoantibody and anti-proteinase 3 antibodies, confirmed the diagnosis of GPA. The patient was treated with high-dose corticosteroids and mycophenolate mofetil, followed by cyclophosphamide due to a relapse, resulting in significant improvement in her condition. This case underscores the importance of considering GPA in the differential diagnosis of pachymeningitis and highlights the diagnostic challenges posed by its nonspecific symptoms. Early recognition and a multidisciplinary approach are crucial for effective management and prevention of severe neurological complications. The report also emphasizes the need for adherence to treatment regimens to avoid relapses and manage the disease effectively.
肉芽肿性多血管炎（GPA），以前称为韦格纳肉芽肿，是一种罕见的自身免疫性血管炎，主要影响中小血管，通常累及呼吸道和肾脏。然而，中枢神经系统受累，尤其是硬脑膜炎形式，是一种极其罕见的表现。本病例报告详细介绍了一名55岁女性，她以硬脑膜炎为GPA的初始表现。患者表现出非特异性症状，包括耳痛、声音改变如声音嘶哑和吞咽困难，提示影响下颅神经IX、X和XII的神经功能缺损。诊断性影像学检查，尤其是MRI，显示出特征性的硬脑膜光滑增厚和强化，提示炎症病因。实验室检查，包括胞浆抗中性粒细胞胞浆自身抗体和抗蛋白酶3抗体阳性，确诊为GPA。患者接受了高剂量皮质类固醇和霉酚酸酯治疗，由于复发随后使用环磷酰胺，病情得到显著改善。本病例强调了在硬脑膜炎鉴别诊断中考虑GPA的重要性，并突出了其非特异性症状带来的诊断挑战。早期识别和多学科方法对于有效管理和预防严重神经并发症至关重要。该报告还强调了坚持治疗方案以避免复发和有效管理疾病的必要性。
