Hyperkalemia in type 4 renal tubular acidosis associated with systemic lupus erythematosus
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Rheumatology International https://doi.org/10.1007/s00296-020-04546-z
INTERNATIONAL
CASE BASED REVIEW
Hyperkalemia in type 4 renal tubular acidosis associated with systemic lupus erythematosus Döndü Üsküdar Cansu1 · Güven Barış Cansu2 · Sultan Güvenir3 · Cengiz Korkmaz4 Received: 3 November 2019 / Accepted: 29 February 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Renal tubular acidosis (RTA) is a normal anion gap metabolic acidosis that manifests with insufficiency of hydrogen ion excretion or bicarbonate ( HCO3) reuptake as a result of renal tubular dysfunction independent of glomerular filtration rate. Hypokalemic RTA subtypes co-existing with autoimmune diseases particularly appear in Sjogren’s syndrome, but rarely in systemic lupus erythematosus (SLE). Type 4 RTA associated with hyperkalemia is very rare during the course of SLE and hence has been scarcely reported in the literature. Here, we report a 42-year-old patient for whom regular follow-up was ongoing due to class IV lupus nephritis when she developed hyperkalemia. The patient had normal anion gap hyperkalemic metabolic acidosis and her urine pH was 5.5. Type 4 RTA was considered and, therefore, tests for renin and aldosterone levels were requested, which revealed that renin was suppressed and aldosterone was decreased. Upon diagnosis of SLE-associated type 4 RTA, short-term oral H CO3 and fludrocortisone were initiated. Potassium (K) and H CO3 levels improved at day 15 of therapy. In this review, we analyzed our case along with five other reports (a total of seven cases) of SLE-associated type 4 RTA we identified through a literature search. We wanted to highlight RTA for differential diagnosis of hyperkalemia emerging during SLE/lupus nephritis and we also discussed possible underlying mechanisms. Keywords Systemic lupus erythematosus · Renal tubular acidosis · Hyperkalemia
Introduction Renal tubular acidosis (RTA) is a normal anion gap metabolic acidosis that appears as a result of decreased bicarbonate reabsorption in or impaired hydrogen excretion from * Döndü Üsküdar Cansu [email protected] Güven Barış Cansu [email protected] Sultan Güvenir [email protected] Cengiz Korkmaz [email protected] 1
Department of Rheumatology, School of Medicine, Eskişehir Osmangazi University Eskisehir, 26480 Eskişehir, Turkey
2
Department of Endocrinology, Kütahya Health Science University, Kütahya, Turkey
3
Division of Nephrology, Department of Internal Medicine, Eskişehir Osmangazi University, Eskişehir, Turkey
4
Division of Rheumatology, Department of Internal Medicine, Eskişehir Osmangazi University, Eskişehir, Turkey
kidneys. RTA is classified into three main groups including proximal RTA (type 2 RTA), hypokalemic distal RTA (type 1 RTA), and hyperkalemic distal RTA (type 4 RTA). Type 1 and type 2 present with hypokalemia in contrast to type 4, which is characterized by hyperkalemia due to deficiency of or resistance to aldosterone [1]. Some of the causes of hypokalemic RTA include tubu
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