Renal Disease in Primary Sjögren’s Syndrome

Aiyegbusi, Oshorenua; McGregor, Laura; McGeoch, Lucy; Kipgen, David; Geddes, Colin C.; Stevens, Kate

doi:10.1007/s40744-020-00264-x

Cited by 33 publications

(36 citation statements)

References 59 publications

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“…In the same group of patients with Bartter syndrome, 20% suffered from acute gouty arthritis [16]. Primary Sjogren's syndrome is a connective tissue disease characterized by lymphocytic infiltration of exocrine glands [17]. It is associated with significant renal tubular disorders, including Type 1 (Distal renal tubular acidosis), Bartter syndrome, Gitelman's syndrome [18], Fanconi syndrome as well as Diabetes insipidus [17].…”

Section: Discussionmentioning

confidence: 99%

“…Primary Sjogren's syndrome is a connective tissue disease characterized by lymphocytic infiltration of exocrine glands [17]. It is associated with significant renal tubular disorders, including Type 1 (Distal renal tubular acidosis), Bartter syndrome, Gitelman's syndrome [18], Fanconi syndrome as well as Diabetes insipidus [17]. Chronic sialadenitis is a central manifestation of Sjogren's syndrome [19].…”

Section: Discussionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Acquired autoimmune Bartter syndrome in a patient with primary hypothyroidism

et al. 2021

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“…In the majority of cases, SS-related glomerulonephritis is a membranoproliferative glomerulonephritis caused by a non-infective cryoglobulinemic vasculitis characterized by deposition of immune complexes [110,111]. The SS-related glomerular disease generally emerges from abnormal routine analyses (proteinuria, elevated serum creatinine) rather than from clinical symptoms (edema/nephrotic syndrome) [91,[110][111][112].…”

Section: Renal Involvementmentioning

confidence: 99%

“…Mildly elevated serum creatinine and low-grade proteinuria may be present [ 91 , 110 , 111 ]. Other less common forms of SS-related tubulointerstitial nephritis are represented by dysfunctions involving cortical collecting duct (principal cells), proximal tubular, loop of Henle and distal convoluted tubule [ 110 , 112 ].…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Sjögren’s syndrome: a systemic autoimmune disease

et al. 2021

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Sjögren’s syndrome is a chronic autoimmune disease characterized by ocular and oral dryness resulting from lacrimal and salivary gland dysfunction. Besides, a variety of systemic manifestations may occur, involving virtually any organ system. As a result, the disease is characterized by pleomorphic clinical manifestations whose characteristics and severity may vary greatly from one patient to another. Sjögren’s syndrome can be defined as primary or secondary, depending on whether it occurs alone or in association with other systemic autoimmune diseases, respectively. The pathogenesis of Sjögren’s syndrome is still elusive, nevertheless, different, not mutually exclusive, models involving genetic and environmental factors have been proposed to explain its development. Anyhow, the emergence of aberrant autoreactive B-lymphocytes, conducting to autoantibody production and immune complex formation, seems to be crucial in the development of the disease. The diagnosis of Sjögren’s syndrome is based on characteristic clinical signs and symptoms, as well as on specific tests including salivary gland histopathology and autoantibodies. Recently, new classification criteria and disease activity scores have been developed primarily for research purposes and they can also be useful tools in everyday clinical practice. Treatment of Sjögren’s syndrome ranges from local and symptomatic therapies aimed to control dryness to systemic medications, including disease-modifying agents and biological drugs. The objective of this review paper is to summarize the recent literature on Sjögren’s syndrome, starting from its pathogenesis to current therapeutic options.

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“…Renal involvement is an extra-glandular condition well recognized in SS patients. The most common histopathological condition is an interstitial lymphocytic infiltrate with tubular atrophy and, consequently, renal fibrosis that leads to a slow progressive deterioration in kidney function [ 60 ].…”

Section: Clinical Fibrotic Manifestation In Ssmentioning

confidence: 99%

Sjögren’s Syndrome-Related Organs Fibrosis: Hypotheses and Realities

Sisto

Ribatti

Lisi

2022

JCM

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Sjögren’s syndrome (SS) is a systemic chronic autoimmune disorder characterized by lymphoplasmacytic infiltration of salivary glands (SGs) and lacrimal glands, causing glandular damage. The disease shows a combination of dryness symptoms found in the oral cavity, pharynx, larynx, and vagina, representing a systemic disease. Recent advances link chronic inflammation with SG fibrosis, based on a molecular mechanism pointing to the epithelial to mesenchymal transition (EMT). The continued activation of inflammatory-dependent fibrosis is highly detrimental and a common final pathway of numerous disease states. The important question of whether and how fibrosis contributes to SS pathogenesis is currently intensely debated. Here, we collect the recent findings on EMT-dependent fibrosis in SS SGs and explore clinical evidence of multi-organ fibrosis in SS to highlight potential avenues for therapeutic investigation.

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Renal Disease in Primary Sjögren’s Syndrome

Cited by 33 publications

References 59 publications

Acquired autoimmune Bartter syndrome in a patient with primary hypothyroidism

Acquired autoimmune Bartter syndrome in a patient with primary hypothyroidism

Sjögren’s syndrome: a systemic autoimmune disease

Sjögren’s Syndrome-Related Organs Fibrosis: Hypotheses and Realities

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