Hypokalemic Paralysis Secondary to Renal Tubular Acidosis Revealing Underlying Sjogren's Syndrome

Abstract

There is a well-established association of Sjogren's syndrome with renal tubular acidosis (RTA). Rarely there is a retrospective diagnosis where the patient presents with RTA and the workup reveals Sjogren's syndrome. Our case report is about a patient who presented with generalized weakness and hypokalemia, which upon further workup turned out to be RTA. Various tests were performed to find out the cause of RTA. A favorable profile for the anti-nuclear antibody, anti-Ro/SSA, and anti-La/SSB was consistent with Sjogren's syndrome. Treatment with corticosteroid improved hypokalemia. The patient did not have typical glandular symptoms of Sjogren's syndrome, and follow-up is needed to see whether the patient develops symptoms in the future and to prevent any possible complication.

Efficacy and safety of an innovative prolonged-release combination drug in patients with distal renal tubular acidosis: an open-label comparative trial versus standard of care treatments

“…Distal renal tubular acidosis (dRTA) is a rare disorder due to an impaired net secretion of acid by the distal tubule, resulting in hyperchloremic metabolic acidosis, often combined with hypokalemia as a result of renal potassium wasting [ 1 – 3 ]. Distal RTA arises when transporters or transcription factors implicated in renal proton secretion are genetically altered [ 4 – 6 ] or affected as a consequence of an autoimmune disease [ 7 – 9 ].…”

“…If not adequately treated, dRTA causes great damage to bone and kidney [ 2 , 11 – 15 ]. In some cases, particularly when associated to an autoimmune disease, dRTA may also present as a metabolic emergency, including manifestations such as hypokalemic paralysis, metabolic coma, and, in extreme cases, death [ 7 , 10 ].…”

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.…”

“…Aiming for a better understanding of the disease, we performed a search of the PubMed database using MeSH descriptors (Acidosis, Renal Tubular; Lupus Erythematosus, Systemic; Sjogren’s Syndrome, Systemic Vasculitis, Rheumatoid Vasculitis, Arthritis, Rheumatoid, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Churg-Strauss Syndrome, IgA Vasculitis, Spondylitis, Ankylosing, Cryoglobulins, Hepatitis, Autoimmune, Liver Cirrhosis, Biliary), and we identified 37 individual case reports published since December 2016 with the association of distal renal tubular acidosis and the following autoimmune disorders: SLE, Sjögren’s syndrome, autoimmune hepatitis, primary biliary cirrhosis and rheumatoid arthritis (see Table 1 ) [ 6 , 10 , 11 , 13 , 15 , 16 , 17 , 18 , 20 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 ]. We also identified a case of hereditary autoimmune polyendocrine syndrome (DRTA, hypoparathyroidism, Addison disease), published in a series of cases, which was not included in our analysis, due to the lack of individual data availability [ 39 ].…”

“…Consequently, most of the patients (26 out of 37) presented with symptoms associated with hypokalemia (progressive muscular weakness and eventually hypokalemic periodic paralysis). In a limited number of cases (3 out of 37), hypokalemic paralysis led to respiratory failure, eventually with orotracheal intubation and ventilation [ 20 , 24 , 25 ]. In one particular case, respiratory failure with the need of orotracheal intubation occurred after producing brisk diuresis and consequent severe hypokalemia following nephrostomy for hydronephrosis caused by ureteral lithiasis [ 24 ].…”