# Sjogren’s Syndrome Presenting With Proximal Myopathy Due to Osteomalacia Complicating Renal Tubular Acidosis: A Case Report

## TL;DR

A woman with Sjogren’s Syndrome developed severe muscle weakness due to a rare kidney-related acidosis complication, which led to bone issues.

## Contribution

This case report highlights a rare and overlooked complication of primary Sjögren’s syndrome involving renal tubular acidosis and osteomalacia.

## Key findings

- The patient had dRTA confirmed by normal anion gap metabolic acidosis and elevated urine pH.
- Osteomalacia was diagnosed as a secondary complication of dRTA in the context of pSS.
- The case emphasizes the importance of timely diagnosis to prevent long-term disability.

## Abstract

Primary Sjögren’s syndrome (pSS) is typically associated with dryness of the eyes and mouth, but it can also involve other organs, including the lungs, kidneys, nervous system, and joints. Among its less common manifestations is distal renal tubular acidosis (dRTA), which can lead to metabolic acidosis, hypokalemia, and bone-related complications due to chronic acid-base imbalance. We report the case of a 42-year-old woman with a four-year history of recurrent hypokalemic quadriparesis, who recently developed progressive difficulty walking over the past two months, severely limiting her mobility. Laboratory investigations revealed a normal anion gap metabolic acidosis and elevated urine pH, consistent with dRTA. Further evaluation confirmed a diagnosis of pSS with objective evidence of glandular involvement. Imaging and biochemical findings supported the presence of osteomalacia secondary to dRTA. This case highlights a rare and often overlooked complication of pSS. Timely diagnosis and appropriate management are crucial to preventing long-term disability and improving patient outcomes.