Kudsi Maysoun, Haidar Ghina, Hadid Batoul, Roumieh Enas, Al-Sayed Safaa
Rheumatology Department, Faculty of Medicine, Damascus University, Damascus, Syria.
Eur J Case Rep Intern Med. 2023 Sep 5;10(10):004051. doi: 10.12890/2023_004051. eCollection 2023.
Primary Sjögren syndrome (pSS) is an immune systemic disease, that may affect the central nervous system. A severe headache unresponsive to treatment is the headache which is persistently nonresponsive to narcotic analgesics.
A 48-year-old woman with a 10-year history of pSS was seen in January 2021, complaining of a headache one week previously. The headache was characterised by a dull persistent pressing intensity and was not responding to paracetamol, NSAIDs or codeine. She had no previous history, nor family history. Physical examination revealed bilateral parotid glands enlargement. Laboratory tests showed anaemia, and elevated levels of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), with positive anti-La and anti-Ro antibodies. She was given topical treatment and different doses of Predlone, in addition to methotrexate10 mg/week. She had received three pulses of methylprednisolone and was started on azathioprine with a mild response to the headaches, so she received two initial IV doses of rituximab 375 mg/m, then every 2 weeks, with a clinical and laboratory response. Two years later, she had no headache.
Headache that may presented in pSS are tension headaches, migraines and cluster headaches. The therapy is disease-modifying antirheumatic drugs, hydroxychloroquine, glucocorticoids and biotherapeutics. Rituximab is used in the treatment of some patients with pSS, especially where it can affect systemic symptoms.
Rituximab treatment may be an option for severe headache in patients with pSS. The mechanism is unknown but may be due to depletion of brain auto-reactive B cells. Further research is needed.
An unresponsive headache in a patient with primary Sjögren's syndrome treated with tryptophan, opioids and NSAIDs responded successfully to B-cell depletion with rituximab.We hypothesise that brain-autoreactive B cells were involved in the pathogenesis of the headache.
原发性干燥综合征（pSS）是一种可能影响中枢神经系统的自身免疫性全身性疾病。治疗无效的严重头痛是指对麻醉性镇痛药持续无反应的头痛。
一名有10年pSS病史的48岁女性于2021年1月就诊，主诉一周前出现头痛。头痛的特点是持续性钝痛，对扑热息痛、非甾体抗炎药或可待因无反应。她既往无相关病史，家族史也无此类情况。体格检查发现双侧腮腺肿大。实验室检查显示贫血，红细胞沉降率（ESR）和C反应蛋白（CRP）水平升高，抗La和抗Ro抗体阳性。除每周10毫克甲氨蝶呤外，她还接受了局部治疗和不同剂量的泼尼松龙。她接受了三次甲泼尼龙冲击治疗，并开始使用硫唑嘌呤，对头痛有轻度反应，因此她接受了两剂初始静脉注射利妥昔单抗，剂量为375毫克/平方米，然后每2周一次，临床和实验室检查均有反应。两年后，她不再头痛。
pSS中可能出现的头痛类型有紧张性头痛、偏头痛和丛集性头痛。治疗方法包括改善病情抗风湿药、羟氯喹、糖皮质激素和生物治疗药物。利妥昔单抗用于治疗一些pSS患者，尤其是在其可影响全身症状的情况下。
利妥昔单抗治疗可能是pSS患者严重头痛的一种选择。其机制尚不清楚，但可能是由于脑自身反应性B细胞的耗竭。需要进一步研究。
一名原发性干燥综合征患者，使用色氨酸、阿片类药物和非甾体抗炎药治疗无效的头痛，通过利妥昔单抗进行B细胞清除后成功缓解。我们推测脑自身反应性B细胞参与了头痛的发病机制。
