BACKGROUND The association of macrophage activation syndrome and primary Sjögren's syndrome has been rarely reported in the literature. We report the first observation of this association in Africa, south of the Sahara, and we discuss the diagnosis and therapeutic challenge. CASE PRESENTATION A 26-year-old Mauritanian and Berber woman was followed for primary Sjögren's syndrome. After a voluntary cessation of her usual background treatment, she was admitted to our department for an outbreak of her illness. A clinical examination revealed anemic syndrome, peripheral polyarthritis, coughing rales at both pulmonary bases, and fever at 39.5 °C. On biologic examination, there was bicytopenia with anemia at 5.70 g/dl, lymphopenia at 796/mm3, a biological inflammatory syndrome with a sedimentation rate at 137 mm in the first hour, C-reactive protein at 97 mg/l, hyperferritinemia at 1778 mg/l (9 normal value), and hypergammaglobulinemia at 20.7 g/l of polyclonal appearance. The triglycerides were 1.95 g/l (1.4 normal value) and the lactate dehydrogenase level was 491 IU/l (1.5 normal value). Cytological examination of a medullary puncture revealed an image of hemophagocytosis. An infectious screening was negative. Thoracic computed tomography showed non-specific interstitial lung disease. A diagnosis of macrophage activation syndrome complicating primary Sjögren's syndrome was selected with a probability of 97.2%, according to H-Score. The evolution was favorable under a treatment including etoposide (VP-16). CONCLUSION Macrophage activation syndrome is a rare entity, rarely reported during primary Sjögren's syndrome. Its spontaneous evolution is invariably fatal. There is no consensus on therapeutic treatment. Etoposide is a therapeutic option especially in forms refractory to corticosteroid therapy.

中文翻译：

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巨噬细胞活化综合征，原发性干燥综合征的罕见并发症：病例报告。

背景技术在文献中很少报道巨噬细胞活化综合征和原发性干燥综合征的关联。我们报告了在撒哈拉以南非洲地区对该协会的首次观察，并讨论了诊断和治疗挑战。病例介绍一名26岁的毛里塔尼亚和柏柏尔妇女因原发性干燥综合征而被随访。在自愿停止常规治疗后，她因病暴发而被送进我们的部门。临床检查显示贫血综合症，周围性多关节炎，两个肺底咳嗽声以及在39.5°C时发烧。在生物学检查中，第一小时出现了2.70克/分升的贫血双细胞减少症，796 / mm3的淋巴细胞减少症，一种生物炎症综合症，沉积速率为137毫米，C反应蛋白为97 mg / l，高铁蛋白血症为1778 mg / l（9正常值），高球蛋白血症为20.7 g / l。甘油三酸酯为1.95 g / l（1.4正常值），乳酸脱氢酶水平为491 IU / l（1.5正常值）。髓样穿刺的细胞学检查显示了吞噬细胞的图像。传染性筛查为阴性。胸部计算机断层扫描显示非特异性间质性肺疾病。根据H-Score，选择诊断为巨噬细胞活化综合征并发原发性干燥综合征的可能性为97.2％。在包括依托泊苷（VP-16）的治疗下，其进化是有利的。结论巨噬细胞活化综合征是一种罕见的实体，在原发性干燥综合征期间很少报道。它的自发进化总是致命的。关于治疗方法尚无共识。依托泊苷是一种治疗选择，尤其是对皮质类固醇疗法难治的形式。