Primary Sjögren’s syndrome as a multi-organ disease: impact of the serological profile on the clinical presentation of the disease in a large cohort of Italian patients

Baldini, Chiara; Pepe, Pasquale; Quartuccio, Luca; Priori, Roberta; Bartoloni, Elena; Alunno, Alessia; Gattamelata, Angelica; Maset, M.; Modesti, M; Tavoni, Antonio; Vita, Salvatore De; Gerli, Roberto; Valesini, Guido; Bombardieri, Stefano

doi:10.1093/rheumatology/ket427

Cited by 215 publications

(168 citation statements)

References 19 publications

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“…Clinical and laboratory data were systematically collected from patient medical records according to specific criteria defined in our previous study [21]. Briefly, clinical data included age at diagnosis and inclusion, history of xerophthalmia, xerostomia, recurrent parotid enlargement, extra-glandular manifestations (i.e.…”

Section: Clinical Variables and Outcomesmentioning

confidence: 99%

Cardiovascular disease risk burden in primary Sjögren's syndrome: results of a population‐based multicentre cohort study

et al. 2015

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Abstract. Bartoloni E, Baldini C, Schillaci G, Quartuccio L, Priori R, Carubbi F, Bini V, Alunno A, Bombardieri S, De Vita S, Valesini G, Giacomelli R, Gerli R (University of Perugia, Perugia; University of Pisa, Pisa; Terni University Hospital, Terni; University of Udine, Udine; Sapienza University of Rome, Rome; University of L'Aquila, L'Aquila; and University of Perugia, Perugia; Italy). Cardiovascular disease risk burden in primary Sj€ ogren's syndrome: results of a population-based multicentre cohort study. J Intern Med 2015; 278: 185-192.Objective. Systemic autoimmune diseases, in particular systemic lupus erythematosus and rheumatoid arthritis, are characterized by a high risk of premature cardiovascular (CV) events. Diseaserelated characteristics and traditional CV disease risk factors may contribute to atherosclerotic damage. However, there are limited data on the risk of overt CV events in primary Sj€ ogren's syndrome (pSS).Methods. We retrospectively analysed a cohort of patients with 1343 pSS. Disease-related clinical and laboratory data, traditional CV disease risk factors and overt CV events were recorded. Prevalence of traditional CV disease risk factors and of major CV events was compared between a subgroup of 788 female patients with pSS aged from 35 to 74 years and 4774 age-matched healthy women.Results. Hypertension and hypercholesterolaemia were more prevalent, whereas smoking, obesity and diabetes mellitus were less prevalent, in women with pSS than in control subjects. Cerebrovascular events (2.5% vs. 1.4%, P = 0.005) and myocardial infarction (MI) (1.0% vs. 0.4%, P = 0.002) were more common in patients with pSS. In the whole population, central nervous system involvement (odds ratio (OR) 5.6, 95% confidence interval (CI) 1.35-23.7, P = 0.02) and use of immunosuppressive therapy (OR 1.9, 95% CI 1.04-3.70, P = 0.04) were associated with a higher risk of CV events. Patients with leucopenia had a higher risk of angina (P = 0.01).Conclusions. pSS is associated with an increased risk of cerebrovascular events and MI. Disease-related clinical and immunological markers may have a role in promoting CV events.

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Section: Clinical Variables and Outcomesmentioning

confidence: 99%

Cardiovascular disease risk burden in primary Sjögren's syndrome: results of a population‐based multicentre cohort study

et al. 2015

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“…Microscopic hematuria and proteinuria rather than the clinical signs, such as azotemia, hypertension, and electrolytes imbalance, are well developed in TIN. In addition, an isolated proteinuria in TIN is difficult to detect with only urinary dipstick test because proteinuria from the renal tubular injury is mainly composed of tubular proteins with small molecular weight than the relatively large albumin [20,25]. Thus, the renal involvement in pSS may be underdiagnosed.…”

Section: Renal Involvement In Pssmentioning

confidence: 99%

Renal Involvement in Rheumatic Diseases

Ahn

Jung

2017

J Rheum Dis

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Most rheumatic diseases are chronic inflammatory diseases. Kidney-related symptoms of rheumatic diseases are often present, which increase mortality and morbidity of patients with rheumatic diseases. When patients with rheumatic diseases show signs or symptoms of renal involvement, management for primary rheumatic diseases should be more aggressive. In general, the risk and severity of renal involvement in patients with rheumatic diseases depend on the type of primary rheumatic diseases. Rheumatic disease itself, chronic use of immunosuppressive agents and non-steroidal anti-inflammatory drugs, and comorbidities, such as diabetes, hypertension, and cardiovascular complications, are the main causes of renal involvement in patients with rheumatic diseases. Many studies have reported the predominant features of renal involvement in most rheumatic diseases. We have attempted to summarize the relationships between rheumatic diseases and renal diseases, and clinical or pathophysiological features of renal involvement resulting from primary rheumatic diseases except systemic lupus erythematosus. Review for renal involvement, particularly in relation to early diagnosis and management of renal involvement in rheumatic diseases, is clinically significant because renal involvement in rheumatic diseases generally implies a bad prognosis. (J Rheum Dis 2017;24:174-184)

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“…In addition, as a systemic disease, pSS can affect almost all extraglandular organs besides the exocrine glands [4, 5]. The prevalence of renal involvement reported in pSS patients varies from 0.3 to 33.5% [6-11]. Chronic tubule interstitial nephritis (TIN) is the most frequent form of renal injury in pSS, with a high prevalence of renal tubular acidosis (RTA), particularly distal RTA, which consistently presents initially as hypokalemia.…”

Section: Introductionmentioning

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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Primary Sjögren’s syndrome as a multi-organ disease: impact of the serological profile on the clinical presentation of the disease in a large cohort of Italian patients

Abstract: This study confirmed that the prevalence of the pSS high-risk subset for severe systemic manifestations is ∼15%. Serological markers might help in the early identification of patients who are candidates to receive more aggressive treatments.

Cited by 215 publications

References 19 publications

Cardiovascular disease risk burden in primary Sjögren's syndrome: results of a population‐based multicentre cohort study

Cardiovascular disease risk burden in primary Sjögren's syndrome: results of a population‐based multicentre cohort study

Renal Involvement in Rheumatic Diseases

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

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