A multicentre study of 95 biopsy-proven cases of renal disease in primary Sjögren’s syndrome

Jasiek, M.; Karras, Alexandre; Guern, Véronique Le; Krastinova, Evguenia; Mesbah, Rafik; Faguer, Stanislas; Jourde-Chiche, Noémie; Fauchais, Anne‐Laure; Chiche, Laurent; Dernis, Emmanuelle; Moulis, Guillaume; Fraison, J.B.; Lazaro, Estibaliz; Jullien, Perrine; Hachulla, É.; Quellec, A. Le; Rémy, Philippe; Hummel, Aurélie; Costédoat-Chalumeau, Nathalie; Ronco, Pierre; Vanhille, Philippe; Meas-Yedid, Vannary; Cordonnier, C.; Ferlicot, Sophie; Daniel, Laurent; Séror, Raphaèle; Mariette, Xavier; Thervet, Éric; Harel, François; Terrier, B.

doi:10.1093/rheumatology/kew376

Cited by 44 publications

(38 citation statements)

References 22 publications

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“…In histopathological studies, chronic tubulointerstitial nephritis was the most common finding (5,13,14). This is characterized by the presence of small lymphocytes (mixed T cells and B cells), plasma cells, and monocytes infiltrating the renal interstitium, together with atrophy of the tubules and fibrosis (5).…”

Section: Renal Disease In Sjogren's Syndrome: Glomerular and Tubuloinmentioning

confidence: 99%

Biomarkers and Diagnostic Testing for Renal Disease in Sjogren's Syndrome

et al. 2020

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Primary Sjogren's syndrome (pSS) is an autoimmune disorder in which lymphocytic infiltration leads to lacrimal and salivary glands dysfunction, which results in symptoms of dryness (xerophthalmia and xerostomia). Extraglandular features are common and may affect several organs. Renal involvement has long been known as one of the systemic complications of pSS. The most classical lesion observed in pSS is tubulointerstitial nephritis (TIN) and less frequently membranoproliferative glomerulonephritis (MPGN), which is related to cryoglobulinemia. In some cases, renal biopsy is necessary for the definitive diagnosis of kidney involvement. Patients may present with proximal renal tubular acidosis, distal renal tubular acidosis and chronic kidney disease. Response to treatment is usually favorable. However, occasionally severe and rarely lethal outcomes have been described. Recently, several case series and cross-sectional studies have been published which investigated the factors associated with renal involvement in pSS and the most accurate screening tests for early detection. The presence of xerophthalmia, anti-SSA and rheumatoid factor positivity, low C3 levels and other features have all shown either positive or inverse associations with the development of renal complications. Serum creatinine, alpha-1-microglobulin, cystatin-C have been evaluated as early detection biomarkers with variable accuracy. More advanced techniques may be necessary to confirm proximal and distal renal tubular acidosis, along with nephrogenic diabetes insipidus. The aim of the current paper is to summarize and critically examine these findings in order to provide updated guidance on serum biomarkers and further testing for kidney involvement in pSS.

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Section: Renal Disease In Sjogren's Syndrome: Glomerular and Tubuloinmentioning

confidence: 99%

Biomarkers and Diagnostic Testing for Renal Disease in Sjogren's Syndrome

et al. 2020

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“…A French multicenter study involving 64 pSS patients with TIN who received immunosuppressive therapy did not find a significant difference in renal function improvement between the steroid group and the cytotoxic group. However, the most common cytotoxic agent used in their study was rituximab, not CTX, and the definition of renal function improvement in their study was an improvement of eGFR >20% compared with baseline values [18]. …”

Section: Discussionmentioning

confidence: 99%

Combination Cyclophosphamide/Glucocorticoids Provide Better Tolerability and Outcomes versus Glucocorticoids Alone in Patients with Sjogren’s Associated Chronic Interstitial Nephritis

Shi

Xie

et al. 2017

Am J Nephrol

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Background: Steroid therapy has become an effective option for patients with primary Sjogren’s syndrome with tubulointerstitial nephritis (TIN), while the use of cytotoxic agents is still debated. Our study aimed to compare the clinical outcomes of patients treated with cyclophosphamide (CTX) combined with glucocorticoids with those of patients treated with glucocorticoids alone. Methods: All patients with primary Sjogren’s syndrome with chronic TIN admitted to the Division of Nephrology, Ruijin Hospital, from January 1, 2002, to April 30, 2016, and treated with steroids alone or combined with CTX were included. The immunological prognosis, improvements of renal function, and acquired tubular defects of the patients were retrospectively compared between the 2 therapeutic groups. Results: A total of 70 cases were included. Of these, 36 were diagnosed by renal biopsy. A total of 56 patients were treated with glucocorticoids alone, while 14 patients received glucocorticoids combined with CTX. There were no significant differences in clinical characteristics and laboratory parameters between the 2 therapeutic groups at baseline. Compared with patients in the steroid group, patients in the CTX group showed better estimated glomerular filtration rate (eGFR) improvement (21.35 ± 19.63 vs. 2.72 ± 19.11 mL/min/1.73 m², p = 0.006) but a similar decline in immunoglobulin G (IgG; 450 [interquartile range, IQR 910] vs.176 [IQR 1,910] mg/dL, p = 0.93) at 12 months of follow-up. CTX therapy was associated with better eGFR improvement (β = 12.96 [2.95–22.97]) even after adjusting for dry mouth, anti-Sjögren’s-syndrome-related antigen A and anti-Sjögren’s-syndrome-related antigen B positivity, hemoglobin, initial steroid dose, and baseline eGFR by linear regression analyses. Subgroup analyses revealed that the beneficial effects of CTX therapy on renal function were only observed in patients with baseline IgG ≥1,560 mg/dL or eGFR <90 mL/min/1.73 m². The urine α1-microglobulin improvement was better in the CTX group than in the steroid group at 12 months of follow-up (β = 1.29, 95% CI 0.56–2.02, p = 0.001). Conclusions: CTX therapy is suggested for primary Sjogren’s syndrome patients with chronic TIN, especially those with higher IgG levels and abnormal renal function at baseline.

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“…121 In this context, renal biopsies demonstrate marked infiltration by B lymphocytes and plasma cells, 122 similar to that seen in salivary glands. Typically, TIN in pSS is associated with distal renal tubular acidosis (RTA), 118,119,[123][124][125] and may lead to electrolyte imbalance (acidosis, hypokalemia, hypophosphatemia) 122 and rarely periodic paralysis. 124 Proximal RTA (Fanconi's syndrome) 126 is rare in renal pSS (3-4%).…”

Section: Renal Involvementmentioning

confidence: 99%

“…128,129 Glomerulonephritis (GN), either with or without TIN, has been present in 23 to 48% of pSS patients with renal involvement, 115,118,119 often associated with cryoglobulinemia. 122 Progression to renal failure or death is more common with GN compared with TIN. 115,120 In one study of 35 pSS patients with renal involvement, 8 of 9 deaths and 8 of 9 lymphomas developed in patients with GN.…”

Section: Renal Involvementmentioning

confidence: 99%

Pulmonary and Bronchiolar Involvement in Sjogren's Syndrome

Chung

Wilgus

Fishbein

et al. 2019

Semin Respir Crit Care Med

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Sjogren's syndrome (SS) is a chronic autoimmune disease characterized by mononuclear cells (principally lymphocytes) infiltrating exocrine glands (e.g., salivary and lacrimal glands), leading to destruction of exocrine epithelial cells and dryness of mucosal surfaces. Cardinal symptoms are dry eyes (xerophthalmia) and dry mouth (xerostomia). Extraglandular sites are affected in 30 to 40% of cases of SS (particularly neurological, kidneys, skin, and lungs). B cell hyperactivity, autoantibody production, and hypergammaglobulinemia are cardinal features of SS. Primary SS is not associated with other autoimmune diseases. However, SS can complicate diverse autoimmune disorders (particularly systemic lupus erythematosus, rheumatoid arthritis, and scleroderma); this form is termed “secondary SS.” Pulmonary involvement is usually not a dominant feature of SS, but may be severe in some cases. In this review, we discuss specific tracheal, bronchiolar, and pulmonary complications of SS including xerotrachea, bronchiolitis, bronchiectasis, interstitial lung disease, nonspecific interstitial pneumonia, usual interstitial pneumonia, lymphoid interstitial pneumonia, organizing pneumonia, acute fibrinous and organizing pneumonia, pulmonary cysts, pleural effusions, pulmonary amyloidosis, and bronchus- or lung-associated lymphomas.

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A multicentre study of 95 biopsy-proven cases of renal disease in primary Sjögren’s syndrome

Abstract: Renal involvement of pSS is mostly due to TIN with marked T, B and especially plasma cell infiltration. Renal dysfunction is usually isolated but can be severe. Use of CSs can improve the eGFR, but further studies are needed to define the best therapeutic strategy in this disease.

Cited by 44 publications

References 22 publications

Biomarkers and Diagnostic Testing for Renal Disease in Sjogren's Syndrome

Biomarkers and Diagnostic Testing for Renal Disease in Sjogren's Syndrome

Combination Cyclophosphamide/Glucocorticoids Provide Better Tolerability and Outcomes versus Glucocorticoids Alone in Patients with Sjogren’s Associated Chronic Interstitial Nephritis

Pulmonary and Bronchiolar Involvement in Sjogren's Syndrome

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