Oumlil Soukaina, Zahlane Mouna, Essaadouni Lamiaa
Department of Internal Medicine, University Hospital Mohammed VI, Marrakesh, Morocco.
Eur J Case Rep Intern Med. 2023 Sep 15;10(10):004035. doi: 10.12890/2023_004035. eCollection 2023.
Hypertrophic pachymeningitis (HP) is an uncommon condition characterised by focal or diffuse thickening of the dura mater. An increasing number of cases have been reported of its association with underlying connective tissue diseases. It is a rare complication in systemic lupus erythematosus (SLE) and might be the initial and sole clinical manifestation. We report a case of a 21-year-old man presenting with febrile meningeal syndrome and sphincter dysfunction. Physical examination showed malar rash and joint pain. Biological assessment revealed a regenerative normocytic normochromic anaemia, a leucopenia and a lymphopenia. The 24-hour urine protein was positive at 0.6 g. Immunological evaluation revealed positive antinuclear, anti-Sm and anti-dsDNA antibodies. Brain and spinal magnetic resonance imaging showed hypertrophic pachymeningitis. Cerebrospinal fluid biochemistry was within normal limits. Renal biopsy revealed a mesangial proliferative lupus nephritis. The diagnosis of SLE with neurologic and renal involvement was established, and the patient was treated with intravenous methylprednisolone pulse, followed by oral prednisone in association with azathioprine and hydroxychloroquine. Considering the persistence of symptoms and MRI lesions after 6 months, a treatment with rituximab was initiated with good evolution.
Hypertrophic pachymeningitis is a rare condition of diverse aetiologies.A workup including search for infectious, autoimmune and neoplastic aetiologies should be performed.It is an extremely rare complication in systemic lupus erythematosus and might be the initial and sole clinical manifestation.
肥厚性硬脑膜炎（HP）是一种罕见疾病，其特征为硬脑膜局灶性或弥漫性增厚。越来越多的病例报告显示其与潜在的结缔组织病有关。它是系统性红斑狼疮（SLE）中一种罕见的并发症，可能是最初且唯一的临床表现。我们报告一例21岁男性患者，表现为发热性脑膜综合征和括约肌功能障碍。体格检查发现有蝶形红斑和关节疼痛。生物学评估显示为正细胞正色素性再生障碍性贫血、白细胞减少和淋巴细胞减少。24小时尿蛋白为0.6 g，呈阳性。免疫学评估显示抗核抗体、抗Sm抗体和抗双链DNA抗体均为阳性。脑和脊髓磁共振成像显示为肥厚性硬脑膜炎。脑脊液生化指标在正常范围内。肾活检显示为系膜增生性狼疮性肾炎。确诊为伴有神经和肾脏受累的SLE，患者接受了静脉注射甲泼尼龙冲击治疗，随后口服泼尼松并联合硫唑嘌呤和羟氯喹。考虑到6个月后症状和MRI病变持续存在，开始使用利妥昔单抗治疗，病情好转。
肥厚性硬脑膜炎是一种病因多样的罕见疾病。应进行包括寻找感染性、自身免疫性和肿瘤性病因在内的检查。它是系统性红斑狼疮中极其罕见的并发症，可能是最初且唯一的临床表现。
