Association of Bartter's syndrome with vasculitis

Deliyska, Boriana; Lazarov, Valentin; Minkova, V.; Nikolov, D; Tishkov, Ivan

doi:10.1093/ndt/15.1.102

Cited by 5 publications

(7 citation statements)

References 13 publications

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“…Moreover, an association between SLT and autoimmunity (e.g. autoimmune thyroid disease, Sjögren’s syndrome, autoimmune hepatitis, and vasculitis) is recognised 22 – 24 , 26 , 28 ; findings in our cohort confirm this association and our demonstration of dysregulated immunity provide an explanation for this link.…”

Section: Discussionsupporting

confidence: 85%

“…Together, these Salt-Losing Tubulopathies share a number of clinical and biochemical features including chronic salt depletion from renal salt wasting, in addition to hyperaldosteronism and hypokalaemic metabolic alkalosis, commonly associated with renal magnesium wasting and hypomagnesaemia. Patients often report a number of somewhat unexplained symptoms, including recurrent febrile episodes in childhood; a link to autoimmunity in both BS and GS has been described, but not fully explained [22][23][24][25][26][27][28] . Immune reactivity in SLT has not been previously explored.…”

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confidence: 99%

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Inherited salt-losing tubulopathies are associated with immunodeficiency due to impaired IL-17 responses

et al. 2020

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Increased extracellular sodium activates Th17 cells, which provide protection from bacterial and fungal infections. Whilst high salt diets have been shown to worsen autoimmune disease, the immunological consequences of clinical salt depletion are unknown. Here, we investigate immunity in patients with inherited salt-losing tubulopathies (SLT). Forty-seven genotyped SLT patients (with Bartter, Gitelman or EAST Syndromes) are recruited. Clinical features of dysregulated immunity are recorded with a standardised questionnaire and immunological investigations of IL-17 responsiveness undertaken. The effects of altering extracellular ionic concentrations on immune responses are then assessed. Patients are hypokalaemic and hypomagnesaemic, with reduced interstitial sodium stores determined by 23 Na-magnetic resonance imaging. SLT patients report increased mucosal infections and allergic disease compared to age-matched controls. Aligned with their clinical phenotype, SLT patients have an increased ratio of Th2:Th17 cells. SLT Th17 and Tc17 polarisation is reduced in vitro, yet STAT1 and STAT3 phosphorylation and calcium flux following T cell activation are unaffected. In control cells, the addition of extracellular sodium (+40 mM), potassium (+2 mM), or magnesium (+1 mM) reduces Th2:Th17 ratio and augments Th17 polarisation. Our results thus show that the ionic environment typical in SLT impairs IL-17 immunity, but the intracellular pathways that mediate salt-driven Th17 polarisation are intact and in vitro IL-17 responses can be reinvigorated by increasing extracellular sodium concentration. Whether better correction of extracellular ions can rescue the immunophenotype in vivo in SLT patients remains unknown.

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Section: Discussionsupporting

confidence: 85%

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confidence: 99%

Inherited salt-losing tubulopathies are associated with immunodeficiency due to impaired IL-17 responses

et al. 2020

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“…In one study, it was speculated that the existing antibodies from the autoimmune disease further enhanced the malfunctioning of the thick ascending limb and were responsible for clinical manifestations of the preexisting syndrome. It was also suggested that immunosuppressive treatment not only improved autoimmune disorders but also lead to the reduction of hypokalemia in Bartter syndrome [ 12 ]. The clinical course of our patient supports this proposition as the marked hypokalemia in our patient has responded after the introduction of immunosuppressive therapy including steroids and mycophenolate mofetil.…”

Section: Discussionmentioning

confidence: 99%

“…There are few case reports regarding the association of Bartter syndrome with autoimmune disorders. In a review of literature, five reports are published on Sjogren syndrome with associated Bartter [ 2 ], one on vasculitis with Bartter [ 12 ], one on Hashimoto thyroiditis with Bartter [ 4 ], and one on sarcoidosis with Bartter syndrome [ 13 ]. This is the first reported case of Bartter syndrome in an adult with undifferentiated connective tissue disorder.…”

Section: Discussionmentioning

confidence: 99%

Association of Adult-Onset Bartter Syndrome With Undifferentiated Connective Tissue Disorder

Saleem¹,

Nasir²,

Hassan³

et al. 2021

Cureus

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Bartter syndrome is a rare autosomal recessive, salt-losing disorder characterized by hypokalemic hypochloremic metabolic alkalosis. We are reporting a case of 21 years old patient, who presented with lower limb weakness, marked hypokalemia, proteinuria, and renal impairment detected on laboratory evaluation. The diagnosis of Bartter syndrome was suspected by marked hypokalemia and was supported by renal biopsy which showed evidence of Juxtaglomerular (JG) hyperplasia. This is the first case report about clinicopathological features of the patient with acquired Bartter syndrome and associated undifferentiated connective tissue disorder manifesting as hypokalemia with paralysis.

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“…Bartter syndrome has also been reported with the onset of systemic vasculitis. Deliyska, B and colleagues [30] managed a 25-year-old woman who developed tetany along with severe hypokalemia. She complained of polyarthralgia with muscular weakness for the past year before the current presentation.…”

Section: Discussionmentioning

confidence: 99%

Acquired autoimmune Bartter syndrome in a patient with primary hypothyroidism

et al. 2021

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We describe an unusual clinical presentation of autoimmune Bartter syndrome in a patient with primary hypothyroidism. A 65-year-old female patient was admitted with neuromuscular weakness associated with hypokalemia and metabolic alkalosis. She had a suboptimal response to potassium supplementation and potassium-sparing diuretic resulting in re-hospitalization with the same symptoms. A detailed serum and urinary biochemistry analysis in the absence of other causes of potassium wasting helped diagnose Bartter syndrome, a rare entity in adults. An autoimmune profile showed anti-Scl-70 antibody to be positive, although she did not develop other systemic features of the disease. Our patient responded to a steroid-based regimen potassium supplement, Indomethacin, and aldosterone antagonist with remarkable resolution of symptoms and correction of electrolyte derangement. We reviewed the literature to search for similar cases and included twenty-seven fulllength publications on acquired and autoimmune causes of Bartter syndrome. Our case highlights the fact that hypokalemia with metabolic alkalosis in an adult patient should prompt clinicians to evaluate for common and uncommon conditions. While assessing for abnormal conditions, acquired Bartter syndrome should be considered if a patient has an underlying autoimmune, endocrine, or connective tissue disease.

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Association of Bartter's syndrome with vasculitis

Cited by 5 publications

References 13 publications

Inherited salt-losing tubulopathies are associated with immunodeficiency due to impaired IL-17 responses

Inherited salt-losing tubulopathies are associated with immunodeficiency due to impaired IL-17 responses

Association of Adult-Onset Bartter Syndrome With Undifferentiated Connective Tissue Disorder

Acquired autoimmune Bartter syndrome in a patient with primary hypothyroidism

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