Prevalence of Severe Extra-Glandular Manifestations in a Large Cohort of Patients with Primary Sjögren’s Syndrome

Chiara Baldini¹, Pasquale Pepe², Luca Quartuccio³, Roberta Priori⁴, Elena Bartoloni Bocci⁵, Alessia Alunno⁶, Serena Colafrancesco⁷, Angelica Gattamelata⁸, Marta Maset⁹, Mariagrazia Modesti⁷, Antonio Tavoni¹⁰, Salvatore De Vita⁹, Roberto Gerli⁵, Guido Valesini⁷ and Stefano Bombardieri¹¹, ¹University of Pisa, Rheumatology Unit, Pisa, Italy, ²Department of Internal Medicine, Rheumatology Unit, University of Pisa, Pisa, Italy, ³Rheumatology, DSMB, University Hospital Santa Maria della Misericordia, Udine, Italy, ⁴Department of Internal Medicine and Medical Specialties, Sapienza University, Rome, Italy, ⁵Rheumatology Unit, Department of Clinical & Experimental Medicine, University of Perugia, Perugia, Italy, ⁶Clinical and Experimental Medicine, Rheumatology Unit, University of Perugia, Perugia, Italy, ⁷Rheumatology Unit, Sapienza University of Rome, Rome, Italy, ⁸Sapienza University of Rome, Rome, Italy, ⁹Rheumatology Clinic, DSMB, University of Udine, Italy, Udine, Italy, ¹⁰University of Pisa, Immunoallergology Unit, Pisa, Italy, ¹¹Department of Clinical and Experimental Medicine, Rheumatology Unit, University of Pisa, Pisa, Italy

Meeting: 2012 ACR/ARHP Annual Meeting

Keywords: Sjogren's syndrome

Session Information

Title: Sjögren's Syndrome - Clinical

Session Type: Abstract Submissions (ACR)

Background/Purpose:

i) to describe the clinico- serological features of a cohort of 1115 patients with primary Sjögren’s syndrome (pSS); ii) to assess the prevalence of systemic extra-glandular manifestations in the cohort; iii) to estimate the impact of the serological and immunological patients’ features on disease different phenotypes and on the utilization of immunosuppressive drugs

Methods: The case records of 1115 patients with a diagnosis of pSS attending four Italian reference centers were reviewed. Clinical and laboratory data of the patients enrolled were retrieved according to a standard form. Independent risk factors for glandular and extra-glandular disease manifestations were identified by logistic regression.

Results: The cohort consisted of 1115 pSS patients (1067 F: 48 M; mean age at the diagnosis of 51.6±13.8 yrs; mean follow-up 5.8±6.5 yrs). All the patients included fulfilled the European classification criteria for pSS, while the AECG criteria were fulfilled in 926/1115 (83%) cases. Xerostomia (93%), xerophtalmia (95%) and articular involvement (62%) were the most commonly detected clinical manifestations followed by hematological involvement (32%) and salivary gland enlargement (31%). A systemic extra-glandular involvement was diagnosed in 475/1115 (42%) patients. Severe extraglandular manifestations included: active synovitis (11%), axonal sensory-motor neuropathy (2%), diffuse purpura or ulcers (6%) renal involvement (0.7%), myositis (0.5%), cerebral vasculitis (0.5%) and transverse myelitis (0.2%). Finally, 50 cases of non-Hodgkin lymphoma were documented. Patients with a systemic disease had a lower mean age at diagnosis (p=0.002), a longer follow up (p<0.0001) and a higher frequency of serologic markers (ANA, RF, anti-Ro/SS-A antibodies, anti-LA/SS-B antibodies, cryoglobulins, hypergammaglobulinemia, low C3/C4 levels) in the univariate analysis. The adjusted multivariate analysis identified as independent serological risk factors for severe extraglandular involvement: low C3 (OR 2.6, 95% CI 1.6-4.1), low C4 (OR1.9, 95% CI 1.1-3.3), hypergammaglobulinemia (OR 2.1, 95% CI 1.5-2.9), cryoglobulins (OR 7.6, 95% CI 2.6-22.3), Rheumatoid factor (OR 2.5, 95% CI 1.5-22.9). No correlation was found among pSS extraglandular involvement and fulfillment of the AECG criteria or positive minor salivary gland biopsy at the diagnosis.

Conclusion: Although the hallmark features of pSS are represented by glandular manifestations, this study support the evidence that severe systemic manifestations may occur in about the 20% of the patients. Patients presenting an active serological profile should be more closely monitored and may deserve more aggressive immunosuppressive drugs.

Disclosure:

C. Baldini,
None;

P. Pepe,
None;

L. Quartuccio,
None;

R. Priori,
None;

E. Bartoloni Bocci,
None;

A. Alunno,
None;

S. Colafrancesco,
None;

A. Gattamelata,
None;

M. Maset,
None;

M. Modesti,
None;

A. Tavoni,
None;

S. De Vita,
None;

R. Gerli,
None;

G. Valesini,
None;

S. Bombardieri,
None.

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