Renal involvement in primary Sjögren’s syndrome

Evans, Rhys; Zdebik, Anselm A.; Ciurtin, Coziana; Walsh, Stephen B.

doi:10.1093/rheumatology/kev223

Cited by 90 publications

(88 citation statements)

References 61 publications

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“…Patients tend to develop renal pathoses relatively early in the disease course, with a mean diagnosis age of 41 years [19]. Recent studies suggest tubular interstitial nephritis is the most common renal disease manifestation in SS patients [20, 21], and this is characterized by renal inflammation [19, 22, 23]. Thus, our findings in NOD.B10 mice mimic the renal histopathology seen in SS patients, although further studies are needed to establish the functional consequence of this in our model.…”

Section: Discussionmentioning

confidence: 99%

Systemic manifestations of primary Sjögren's syndrome in the NOD.B10Sn-H2/J mouse model

Kiripolsky

Shen

Liang³

et al. 2017

Clinical Immunology

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Animal models that recapitulate human disease are crucial for the study of Sjögren’s Syndrome (SS). While several SS mouse models exist, there are few primary SS (pSS) models that mimic systemic disease manifestations seen in humans. Similar to pSS patients, NOD.B10Sn-H2b/J (NOD.B10) mice develop exocrine gland disease and anti-nuclear autoantibodies. However, the disease kinetics and spectrum of extra-glandular disease remain poorly characterized in this model. Our objective was to characterize local and systemic SS manifestations in depth in NOD.B10 female mice at early and late disease time points. To this end, sera, exocrine tissue, lung, and kidney were analyzed. NOD.B10 mice have robust lymphocytic infiltration of salivary and lacrimal tissue. In addition, they exhibit significant renal and pulmonary inflammation. We identified numerous autoantibodies, including those directed against salivary proteins. In conclusion, the NOD.B10 model recapitulates both local and systemic pSS disease and represents an excellent model for translational studies.

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Section: Discussionmentioning

confidence: 99%

Systemic manifestations of primary Sjögren's syndrome in the NOD.B10Sn-H2/J mouse model

Kiripolsky

Shen

Liang³

et al. 2017

Clinical Immunology

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“…4 Some studies correlated RTA and high anti-SS A/Ro and anti-SS B/La dRTA in Primary Sjögren's Syndrome level titers as a surrogate of pSS progression. 15,16 Additionally, other studies have suggested that lymphoplasmacytic infiltrates surrounding renal tubules are associated with renal tubular defects.…”

Section: Discussionmentioning

confidence: 99%

“…3 Primary Sjögren's syndrome (pSS) mainly affects exocrine glands; nevertheless it can impair renal function via tubular and glomerular injury. 4 In adults, pSS has been identified as the leading cause of acquired dRTA. 5 The relevance of this case stands in the atypical clinical presentation of symptomatic, severe hypokalemia as the first manifestation of pSS.…”

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confidence: 99%

Primary Sjögren's Syndrome First Presenting as Hypokalemic Quadriparesis

et al. 2017

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“…Type II proximal tubular dysfunction consists of proteinuria, aminoaciduria, glycosuria, phosphaturia, uricosuria and bicarbonaturia (Fanconi syndrome) and may lead to osteomalacia as a consequence of phosphate wasting. Type I and IV distal renal tubular acidosis is due to a complete or incomplete inadequate H+ secretion in the cortical collecting duct by the acid-secreting a-intercalated cells (30). Distal renal tubular acidosis in pSS was found to be associated with anti-Ro and La antibodies, longer disease duration, xerostomia, hypertension, higher creatinine and proteinuria, and also hypergammaglobulinaemia (31).…”

Section: N Renal Involvementmentioning

confidence: 99%

The clinical spectrum of primary Sjögren’s syndrome: beyond exocrine glands

et al. 2017

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SUMMARYAlthough primary Sjögren's syndrome (pSS) is a mild indolent chronic disease mainly characterized by mucosal dryness in the majority of cases, a consistent subgroup of patients display extra-glandular manifestations. Virtually any organs and systems can be affected, leading to a more serious disease prognosis. Therefore, the prompt identification of patients at higher risk of extra-glandular manifestations is necessary to start a thorough follow up and an aggressive treatment. The aim of this review article is to provide an overview of epidemiological, clinical and serological features of extra-glandular manifestations in pSS as well as current knowledge about putative biomarkers useful in clinical practice. Key words:Sjögren's syndrome; Extra-glandular manifestations; Cardiovascular risk. Reumatismo, 2017; 69 (3): 93-100 n INTRODUCION P rimary Sjögren's syndrome (pSS) is a complex and heterogeneous chronic autoimmune disease primarily characterized by a focal lymphocytic chronic inflammation of exocrine glands leading to progressive loss of secretory function (1). Therefore, the specific case-history of pSS is dominated by signs and symptoms of mucosal dryness. However, although salivary and lachrymal glands represent the main target of pSS, several other organs and systems may be affected during the disease course in at least one third of patients, resulting in a plethora of systemic clinical manifestations, serological abnormalities and complications (2). The most severe complication of pSS is non-Hodgkin lymphoma occurring in about 5% of patients (3) and dramatically worsening disease prognosis. In this regard, the prompt identification of pSS patients with higher risk of developing extraglandular manifestations is mandatory for correct management. Low C3 and C4 levels, cryoglobulins, monoclonal component, anti-Ro/ SSA, anti-La/SSB, rheumatoid factor and hypergammaglobulinaemia represent poor prognostic serological factors in pSS associated with lymphoma and severe extraglandular features (2). Furthermore, histological features such as the presence of germinal centre-like structures in the minor salivary glands have been associated with higher risk of developing lymphoma (4). The purpose of this review article is to discuss current knowledge of extra-glandular manifestations in pSS not secondary to mucosal dryness, excluding pathogenesis and clinical manifestations of lymphoproliferative disorders. n MUSCULOSKELETAL INVOLVEMENTMusculoskeletal manifestations such as myalgia, arthralgia and morning stiffness are present in as many as 90% of patients, while clinically evident arthritis is found in up to 17%. In a recent study conducted on 17 female patients with pSS and 18 patients with secondary SS (sSS), ultrasonography (US) was used to confirm the non-erosive nature of joint involvement in pSS and to discriminate between pSS and RA-associated SS. Synovitis was commonly detected in pSS, mainly in metacarpophalangeal joints (MCP), wrists, and knees. Bone erosions also may occur. Power Doppler signal ...

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Renal involvement in primary Sjögren’s syndrome

Cited by 90 publications

References 61 publications

Systemic manifestations of primary Sjögren's syndrome in the NOD.B10Sn-H2/J mouse model

Systemic manifestations of primary Sjögren's syndrome in the NOD.B10Sn-H2/J mouse model

Primary Sjögren's Syndrome First Presenting as Hypokalemic Quadriparesis

The clinical spectrum of primary Sjögren’s syndrome: beyond exocrine glands

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