Systemic involvement in primary Sjogren's syndrome evaluated by the EULAR-SS disease activity index: analysis of 921 Spanish patients (GEAS-SS Registry)

Ramos‐Casals, M.; Brito-Zerón, Pilar; Solans, Roser; Camps, María Teresa; Casanovas, A.; Sopeña, B.; Díaz-Lόpez, B.; Rascόn, F.-J.; Qanneta, Rami; Fraile, G.; Pérez-Álvarez, R.; Callejas, J.L.; Ripoll, M.; Pinilla, Blanca; Akasbi, M.; Fonseca, E.; Canora, Jesús; Nadal, Marion; Red, Gloria de la; Fernandez-Regal, I.; Jiménez-Heredia, I.; Bosch, J.; Ayala, Mariana; Morera-Morales, L.; Maure, B.; Mera, Antonio; Ramentol, M.; Retamozo, Soledad; Kostov, Belchin

doi:10.1093/rheumatology/ket349

Cited by 213 publications

(130 citation statements)

References 34 publications

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“…Older patients at the time of diagnosis (> 70 years) were less active during follow-up, but had a higher pulmonary activity score. 7 Findings on the chest HRCT scan in our study were more common in patients with arthritis, but the group with these complications was relatively small.…”

Section: Discussionmentioning

confidence: 45%

See 1 more Smart Citation

Chest HRCT findings in patients with primary Sjögren’s syndrome

Sebastian¹,

Misterska-Skóra²,

Silicki³

et al. 2017

Adv Clin Exp Med

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Section: Discussionmentioning

confidence: 45%

“…It is suggested that a score of 5 points is associated with moderate pSS activity and > 14 points with shorter survival. 6,7 The aim of this study was to assess the incidence of pulmonary manifestations in pSS and to determine factors predisposing to this complication.…”

mentioning

confidence: 99%

Chest HRCT findings in patients with primary Sjögren’s syndrome

Sebastian¹,

Misterska-Skóra²,

Silicki³

et al. 2017

Adv Clin Exp Med

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“…The results displayed reliable thresholds for defining activity levels and changes in scores that could be used for designing future clinical trials. Nevertheless, since the number of observational cohorts already used ESSDAI and ESSPRI for the evaluation of their real-life patients,6 26 the usefulness of these thresholds in clinical practice will be further evaluated.…”

Section: Discussionmentioning

confidence: 99%

Defining disease activity states and clinically meaningful improvement in primary Sjögren's syndrome with EULAR primary Sjögren's syndrome disease activity (ESSDAI) and patient-reported indexes (ESSPRI)

et al. 2014

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This study determined disease activity levels, PASS and MCII of ESSDAI and ESSPRI. These results will help designing future clinical trials in SS. For evaluating systemic complications, the proposal is to include patients with moderate activity (ESSDAI≥5) and define response to treatment as an improvement of ESSDAI at least three points. For addressing patient-reported outcomes, inclusion of patients with unsatisfactory symptom state (ESSPRI≥5) and defining response as an improvement of ESSPRI at least one point or 15% seems reasonable.

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“…Overall, renal involvement occurs in 4-5% of pSS patients (25)(26)(27). TIN is the predominant condition, found in 75% of patients, with the remaining 25% of patients having glomerular disease (28,29).…”

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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Systemic involvement in primary Sjogren's syndrome evaluated by the EULAR-SS disease activity index: analysis of 921 Spanish patients (GEAS-SS Registry)

Cited by 213 publications

References 34 publications

Chest HRCT findings in patients with primary Sjögren’s syndrome

Chest HRCT findings in patients with primary Sjögren’s syndrome

Defining disease activity states and clinically meaningful improvement in primary Sjögren's syndrome with EULAR primary Sjögren's syndrome disease activity (ESSDAI) and patient-reported indexes (ESSPRI)

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

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