Al-Qaysi Ghassan, Abu-Abaa Mohammad, Abdulsahib Ali, Ruppel Andreas, Prabhakaran Sajina
Internal Medicine, Capital Health Regional Medical Center, Trenton, USA.
Cureus. 2023 May 17;15(5):e39121. doi: 10.7759/cureus.39121. eCollection 2023 May.
Hypertrophic pachymeningitis (HPM) is a rare but extremely debilitating disease. It is even rarer for HPM to be seen in association with antineutrophil cytoplasmic antibody (ANCA)-negative vasculitis. In this case, we are presenting HPM that was diagnosed in a 28-year-old female patient who presented with worsening back pain. Imaging revealed dural-based enhancing masses affecting the thoracic spinal cord with compression. Infectious etiologies were ruled out and a total of three biopsies failed to show any evidence of granulomatous inflammation, malignancy, or evidence of immunoglobulin G4-related disease. ANCA was negative on repeated testing. The patient was managed with repeated short courses of steroids that resulted in symptomatic control as well as radiological stability of the disease. This is an extremely rare case of atypical presentation of spinal HPM that is likely associated with granulomatous and polyangiitis without other manifestations of the disease except for nasal septal perforation. This case is a supplement to a limited body of knowledge and established cases of HPM in ANCA-negative, ANCA-associated vasculitis.
肥厚性硬脑膜炎（HPM）是一种罕见但极为使人衰弱的疾病。HPM与抗中性粒细胞胞浆抗体（ANCA）阴性血管炎相关联的情况更为罕见。在本病例中，我们报告了一名28岁女性患者被诊断为HPM，该患者表现为背痛加重。影像学检查显示硬膜下强化肿块累及胸段脊髓并伴有压迫。排除了感染性病因，总共三次活检均未显示任何肉芽肿性炎症、恶性肿瘤或免疫球蛋白G4相关疾病的证据。多次检测ANCA均为阴性。该患者接受了多次短期类固醇治疗，症状得到控制，疾病在影像学上也保持稳定。这是一例极为罕见的脊柱HPM非典型表现病例，可能与肉芽肿性多血管炎相关，除鼻中隔穿孔外无该疾病的其他表现。本病例补充了关于ANCA阴性、ANCA相关血管炎中HPM的有限知识和已确诊病例。
