Systemic lupus erythematosus complicated by a Gitelman-like syndrome in an 8-year-old girl

Barathidasan, Gowri S; Krishnamurthy, Sriram; Karunakar, Pediredla; Rajendran, Ranjitha; Ramya, Kagnur; Gunasekaran, Dhandapany; Ramamoorthy, Jaikumar Govindaswamy; Ganesh, Rajesh Nachiappa

doi:10.1007/s13730-019-00440-1

Cited by 10 publications

(7 citation statements)

References 13 publications

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“…A rare, phenotypically similar clinical presentation has been described in patients with autoimmune disorders such as Sjögren syndrome, Systemic lupus erythematosus and Systemic sclerosis, which has been named as "acquired Gitelman syndrome" [8][9][10][11]. There are only eight such cases have been reported in patients with primary Sjögren syndrome and three of these patients presented with hypokalemic paralysis [12][13][14][15].…”

Section: Discussionmentioning

confidence: 90%

Hypokalaemic paralysis and metabolic alkalosis in a patient with Sjögren syndrome: a case report and literature review

2021

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Background Acquired Gitelman syndrome is a very rare disorder reported in association with autoimmune disorders, mostly Sjögren syndrome. It is characterized by the presence of hypokalaemic metabolic alkalosis, hypocalciuria, hypomagnesaemia and hyper-reninaemia, in the absence of typical genetic mutations associated with inherited Gitelman syndrome. Case presentation A 20 year old woman who was previously diagnosed with primary Sjögren syndrome and autoimmune thyroiditis presented with two week history of lower limb weakness and salt craving. Examination revealed upper limb and lower limb muscle weakness with muscle power of 3/5 on MRC scale and diminished deep tendon reflexes. On evaluation, she had hypokalaemia with high trans-tubular potassium gradient, metabolic alkalosis and hypocalciuria, features suggestive of Gitelman syndrome. New onset hypokalaemic alkalosis in a previously normokalaemic patient with Sjögren syndrome strongly favored a diagnosis of acquired Gitelman syndrome. Daily potassium supplementation and spironolactone resulted in complete clinical recovery. Conclusions Acquired Gitelman syndrome associated with Sjögren syndrome is rare. It should be considered as a differential diagnosis during evaluation of acute paralysis and hypokalaemic metabolic alkalosis in patients with autoimmune disorders, especially Sjögren syndrome.

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Section: Discussionmentioning

confidence: 90%

Hypokalaemic paralysis and metabolic alkalosis in a patient with Sjögren syndrome: a case report and literature review

2021

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“…Others have described an acquired Gitelman's syndrome-like phenotype in the setting of autoimmunity. Most of these reports have been in patients with Sj€ ogren's syndrome, although associations with systemic sclerosis and SLE have also been reported (27)(28)(29)(30)(31)(32). To rigorously prove the hypothesis that this is an acquired form of Gitelman's syndrome, we would need to detect an autoantibody to sodium-chloride cotransporter, and show there was a deficiency of this transporter in the distal convoluted tubule in the absence of SLC12A3 mutations.…”

Section: Discussionmentioning

confidence: 99%

Idiopathic Hypokalemia in Lupus Nephritis: A Newly Recognized Entity

et al. 2021

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Background:Various causes of hypokalemia from renal potassium wasting, including distal renal tubular acidosis, have been described in lupus nephritis (LN). We report a phenomenon of otherwise unexplained hypokalemia among a population with LN. Methods:From our population of 403 patients with LN, we identified a cohort of 20 patients with idiopathic hypokalemia (HK), defined by serum potassium < 3.5 mmol/L without any apparent explanation. This cohort is compared to 90 LN controls (CON) and 10 LN patients with distal renal tubular acidosis (RTA) from the same population. Results:The HK cases had lower median serum potassium compared to CON and RTA subjects (3.26 vs 4.00 vs 3.75 mmol/L, respectively; p < 0.001). The median serum bicarbonate was normal in HK and CON but low in RTA (26.0 vs 25.0 vs 19.4 mmol/L; p < 0.001). The median urine pH was abnormally high only in the RTA group (6.00 vs 6.25 vs 6.67; p = 0.012). The median serum magnesium was modestly lower in HK compared to the CON and RTA groups (1.73 vs 2.00 vs 1.85 mg/dL; p = 0.002). While both HK and RTA showed a higher rate of seropositivity than CON for anti-Ro/SSA (79% and 80% vs 37%, respectively; p < 0.001), only HK revealed a higher rate of seropositivity than CON for anti-RNP (84% vs 42%; p = 0.003) and only RTA showed a higher rate of seropositivity than CON for anti-La/SSB (40% vs 12%; p = 0.046). Conclusions:A syndrome of idiopathic hypokalemia was revealed in 20/403 (5%) of patients within our LN population and proved to be distinct from the renal tubular acidosis that occurs in LN. Furthermore, it was associated with a distinct pattern of autoantibodies. We speculate that idiopathic hypokalemia is the result of a novel target of autoimmunity in LN affecting renal tubular potassium transport.

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“… 41 , 42 , 43 , 44 Acquired Gitelman syndrome has been reported in some patients with autoimmune diseases. 44 , 45 , 46 …”

Section: Patients With Potassium Disordersmentioning

confidence: 99%

Use of Urine Electrolytes and Urine Osmolality in the Clinical Diagnosis of Fluid, Electrolytes, and Acid-Base Disorders

Kamel

Halperin

2021

Kidney International Reports

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This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

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Systemic lupus erythematosus complicated by a Gitelman-like syndrome in an 8-year-old girl

Cited by 10 publications

References 13 publications

Hypokalaemic paralysis and metabolic alkalosis in a patient with Sjögren syndrome: a case report and literature review

Hypokalaemic paralysis and metabolic alkalosis in a patient with Sjögren syndrome: a case report and literature review

Idiopathic Hypokalemia in Lupus Nephritis: A Newly Recognized Entity

Use of Urine Electrolytes and Urine Osmolality in the Clinical Diagnosis of Fluid, Electrolytes, and Acid-Base Disorders

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