Martinho Aurélia L, Capela Andreia, Duarte Fernanda
Serviço de Medicina 2, Hospital Fernando Fonseca, Amadora, Portugal.
Acta Med Port. 2012 Mar-Apr;25(2):122-4. Epub 2012 Jun 25.
The Sjögren's syndrome is a systemic autoimmune disorder characterized by chronic inflammation of the exocrine glands with extraglandular manifestations in up to 25% patients. Renal involvement occurs in 18.4-67% of cases, with tubulointerstitial nephritis being the most frequent pathology. We present the case of a 37 year-old woman admitted because of generalized grade 2 muscle weakness which developed over a week. We detected: hypokalemia, rhabdomyolysis, urinary pH 6.5, proteinuria and metabolic acidemia. The laboratory tests suggestive of distal renal tubular acidosis with hypokalaemia led to the diagnosis of lymphoplasmocytic tubulointerstitial nephritis, which was confirmed by renal biopsy, and to a clinical suspicion of Sjögren's syndrome. Primary Sjögren's syndrome was diagnosed in this patient based on the following criteria: xerophthalmia, xerostomia, sialadenitis, positive anti-SSA and anti-SSB antibodies, and absence of criteria for lupus and rheumatoid arthritis. During hospitalization, the patient developed deep vein thrombosis. Tests showed positive antiphospholipid antibodies and the diagnosis of secondary antiphospholipid syndrome was made. She was treated with potassium, bicarbonate, steroids, ramipril and warfarin. The authors wish to highlight the extraglandular manifestations and in particular the rarity of hypokalemic paralysis as the presenting manifestation of primary Sjögren's syndrome.
干燥综合征是一种全身性自身免疫性疾病，其特征为外分泌腺的慢性炎症，高达25%的患者会出现腺体外表现。18.4% - 67%的病例会出现肾脏受累，其中肾小管间质性肾炎是最常见的病理类型。我们报告一例37岁女性患者，因在一周内逐渐出现的全身性2级肌无力入院。我们检测到：低钾血症、横纹肌溶解、尿pH值6.5、蛋白尿和代谢性酸血症。提示存在低钾性远端肾小管酸中毒的实验室检查结果，导致诊断为淋巴细胞浆细胞性肾小管间质性肾炎，肾活检证实了这一诊断，并临床怀疑为干燥综合征。根据以下标准，该患者被诊断为原发性干燥综合征：干眼症、口干症、涎腺炎、抗SSA和抗SSB抗体阳性，且不符合狼疮和类风湿关节炎的标准。住院期间，该患者发生了深静脉血栓形成。检查显示抗磷脂抗体阳性，诊断为继发性抗磷脂综合征。她接受了钾、碳酸氢盐、类固醇、雷米普利和华法林治疗。作者希望强调腺体外表现，尤其是低钾性麻痹作为原发性干燥综合征首发表现的罕见性。
