Division of Rheumatology, Department of Medicine, National Hospital Organization Tokyo Medical Center, Tokyo, Japan.
Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.
Mod Rheumatol Case Rep. 2023 Jun 19;7(2):388-393. doi: 10.1093/mrcr/rxac095.
Aseptic meningitis is a rare life-threatening complication of primary Sjögren's syndrome (pSS), and its characteristics and prognosis remain unknown. We present our case of aseptic meningitis associated with pSS and reviewed the published literature to elucidate their characteristics and prognosis. An 84-year-old man was admitted to our hospital for fever and disturbance of consciousness. Acute aseptic meningitis was diagnosed based on the results for cerebrospinal fluid and head imaging tests. As an aetiological investigation for his aseptic meningitis, serum anti-Sjögren's-syndrome-related antigen A and anti-Sjögren's-syndrome-related antigen B antibodies were found to be positive, and the biopsy specimen of his labial salivary gland revealed lymphocytic sialadenitis, confirming a diagnosis of pSS. Treatment with moderate-dose glucocorticoid completely improved his aseptic meningitis. Relapse of the disease was not observed during his clinical course over 12 months. Our present case and literature review suggest that aseptic meningitis can be an initial manifestation of pSS and be treatable by immunosuppressive therapy. Thus, early recognition and treatment initiation are critical to prevent the irreversible damage of central nervous system in pSS-associated aseptic meningitis. In aseptic meningitis of unknown origin, pSS should be included in differential diagnoses, and testing for serum anti-Sjögren's-syndrome-related antigen A and anti-Sjögren's-syndrome-related antigen A antibodies may be useful as an initial screening.
无菌性脑膜炎是原发性干燥综合征（pSS）罕见的危及生命的并发症，其特征和预后尚不清楚。我们报告了一例与 pSS 相关的无菌性脑膜炎病例，并复习了已发表的文献，以阐明其特征和预后。一名 84 岁男性因发热和意识障碍而入院。根据脑脊液和头部影像学检查结果，诊断为急性无菌性脑膜炎。为了明确无菌性脑膜炎的病因，患者血清抗干燥综合征相关抗原 A 和抗干燥综合征相关抗原 B 抗体阳性，唇腺活检显示淋巴细胞性唾液腺炎，确诊为 pSS。中等剂量糖皮质激素治疗完全改善了患者的无菌性脑膜炎。在 12 个月的临床病程中，疾病未复发。我们的病例和文献复习表明，无菌性脑膜炎可能是 pSS 的首发表现，且可通过免疫抑制治疗得到缓解。因此，早期识别和治疗启动对于预防 pSS 相关无菌性脑膜炎对中枢神经系统的不可逆损害至关重要。对于病因不明的无菌性脑膜炎，应将 pSS 纳入鉴别诊断，检测血清抗干燥综合征相关抗原 A 和抗干燥综合征相关抗原 B 抗体可能有助于作为初始筛查。
