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.

Cite this article as: Mangal V, Vohra G, Adhikari S, Vasudeva A. A Conundrum of Severe Hypokalaemic Quadriparesis, Acute Kidney Injury, and Lung Involvement as the Initial Presentation of Catastrophic Primary Sjögren’s Syndrome: Is it a New Entity? A Case Report. Mediterr J Rheumatol 2023;34(4):555-9

Article Submitted: 11 Jan 2023; Revised Form: 28 Feb 2023; Article Accepted: 1 Mar 2023; Available Online: 4 Sep 2023

https://doi.org/10.31138/mjr.040923.acs

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©2023 The Author(s).