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Volume 34, Issue 4, December 2023



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Mediterr J Rheumatol 2023;34(4):555-9
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
Authors Information

1Department of Internal Medicine, Military Hospital Ambala, Haryana, India

2Department of Nephrology, Command Hospital Western Command, Chandimandir, Haryana, India

3Department of Pulmonology, Command Hospital Western Command, Chandimandir, Haryana, India

4Military Hospital Ambala, Haryana, India

Abstract

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

This work is licensed under a Creative Commons Attribution 4.0 International License.

©2023 The Author(s).