Hypokalaemic quadriparesis with respiratory failure due to latent Sjogren syndrome

Abstract

Sjogren’s syndrome (SS) is an autoimmune disease with involvement of multiple organs, including both glandular and extraglandular organs. Usually involvement of glandular organs manifests before the extraglandular ones, but when the sequence is reversed, diagnosis may be missed. Hypokalaemic quadriparesis in SS is not uncommon. Respiratory failure in hypokalaemia is not usually seen, but in SS, it has been reported. We report a case of a 55-year-old woman who presented with sudden onset flaccid quadriparesis and respiratory muscle paralysis secondary to severe hypokalaemia. On detailed investigation, she was detected to have distal renal tubular acidosis secondary to clinically inapparent and asymptomatic SS.

Distal Renal Tubular Acidosis in Patients with Autoimmune Diseases—An Update on Pathogenesis, Clinical Presentation and Therapeutic Strategies

“…Two additional case reports with this association were published in 2020, and another study published recently showed a prevalence of 14.81% of DRTA (16 patients) in a cohort of 108 patients with SLE [ 6 , 7 , 8 ]. Generally, the majority of cases with complete DRTA published in literature are diagnosed due to severe symptoms associated with hypokalemia, such as quadriparesis or even respiratory arrest, and therefore, the real prevalence of DRTA associated with autoimmune diseases is difficult to estimate [ 6 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 ]. Therefore, urinary acidification tests are essential in diagnosing those patients who did not develop any symptoms and would otherwise escape undiagnosed.…”