Sudden Onset Paresis in a Middle-aged Female? Evaluate for Hypokalemia due to Undiagnosed Sjögren Syndrome

Introduction: Sjögren’s syndrome is an autoimmune disorder which leads to chronic B-cell infiltration of the exocrine glands, most commonly salivary and lacrimal glands. This leads to xerostomia and keratoconjuctivitis sicca which are the most common clinical manifestations reported in literature. However, it can present in a wide range of clinical spectrum. Such patients may pose a diagnostic challenge for the treating physicians. Aim: The aim of the article is to establish the significance of autoimmune workup in any middle-aged female presenting with hypokalemic quadriparesis with hyperchloremic normal anion metabolic acidosis. Materials and methods: This case series was reported from a tertiary care center in North-West India from June, 2022 to July, 2024. Here we will discuss five middle-aged female patients who presented with sudden onset quadriparesis, as a result of severe hypokalemia, which was the initial manifestation of Sjögren’s syndrome due to interstitial involvement of the kidneys, manifesting as distal (type I) renal tubular acidosis (RTA), thus leading to hypokalemia. In these patients, arterial blood gases (ABG) reveal hyperchloremic normal anion gap metabolic acidosis (NAGMA). Antinuclear antibodies (ANA) Hep-2 positivity was seen associated with a positive SSA antibody, thus making a diagnosis of Sjögren syndrome. Conclusion: Patients of Sjögren’s syndrome may present with manifestations due to extraglandular involvement of kidneys. The most common renal manifestation is interstitial nephritis, presenting as distal (type I) RTA. Hypokalemia is the most common electrolyte abnormality, that causes sudden onset quadriparesis.