Mangal Vishal, Vohra Gaurav, Adhikari Sudipt, Vasudeva Anil
Department of Internal Medicine, Military Hospital Ambala, Haryana, India.
Department of Nephrology, Command Hospital Western Command, Chandimandir, Haryana, India.
Mediterr J Rheumatol. 2023 Sep 4;34(4):555-559. doi: 10.31138/mjr.040923.acs. eCollection 2023 Dec.
Sjögren's syndrome (SS) is a systemic chronic autoimmune disorder that classically affects the exocrine glands. Only 15% of the patients with primary SS (pSS) develop extraglandular symptoms involving the lungs, kidneys, joints, nervous system, and skin. Hypokalaemic paralysis is a rare presentation. The most common cause of hypokalaemia is distal renal tubular acidosis. The prevalence of clinically significant lung involvement in pSS is 9-20 %. Primary SS is an indolent disease leading to increased morbidity and poor quality of life. We present a case of a 40-year-old female with severe hypokalaemic paralysis, tubulointerstitial nephritis, and lung involvement as the initial presentation of catastrophic pSS without sicca symptoms. The course of hospitalisation was complicated by ventilator-associated pneumonia. She was managed with broad spectrum antibiotics, five sessions of plasma exchange and alternate-day haemodialysis followed by oral glucocorticoids and intravenous cyclophosphamide. To the best of our knowledge, this is the first case of catastrophic presentation of pSS with a favourable outcome.
干燥综合征（SS）是一种全身性慢性自身免疫性疾病，典型表现为侵犯外分泌腺。仅15%的原发性干燥综合征（pSS）患者会出现累及肺部、肾脏、关节、神经系统和皮肤的腺体外症状。低钾性麻痹是一种罕见的表现。低钾血症最常见的原因是远端肾小管酸中毒。临床上pSS患者出现明显肺部受累的发生率为9% - 20%。原发性干燥综合征是一种进展缓慢的疾病，会导致发病率增加和生活质量下降。我们报告一例40岁女性患者，以严重低钾性麻痹、肾小管间质性肾炎和肺部受累为首发表现，诊断为灾难性pSS，且无干燥症状。住院过程中并发呼吸机相关性肺炎。对其采用广谱抗生素治疗、5次血浆置换和隔日血液透析，随后给予口服糖皮质激素和静脉注射环磷酰胺。据我们所知，这是首例pSS灾难性表现且预后良好的病例。
