Document Detail


Primary Sjogren'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.
MedLine Citation:
PMID:  24369420     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Objective. The aims of this study were to describe the clinical presentation of primary SS (pSS) in a large cohort of patients by assessing the prevalence of the patient subgroups at high risk for severe extraglandular manifestations and to explore the influence of the patients' serological profile on disease severity and on immunosuppressive drug utilization.Methods. Cumulative demographic, clinical, serological, histological and therapeutic data of 1115 pSS patients were retrospectively evaluated. Independent serological markers for glandular and extraglandular disease manifestations were identified by logistic regression.Results. The cohort included 1115 (1067 female, 48 male) pSS patients. Severe extraglandular manifestations were detectable in 15% of the patients and were represented by active synovitis (11%), axonal sensory-motor neuropathy (2%), severe leucocytopenia (14%), cutaneous vasculitis (6%) and non-Hodgkin's lymphoma (4.5%). We found that low C3/C4, hypergammaglobulinaemia, RF and cryoglobulinaemia were markers of severity for pSS. According to the number of serological variables, the patients were subdivided into three distinct groups: favourable (no serological markers), intermediate (one serological marker) and poor (two or more serological markers). In comparison with the other two patient groups, pSS patients presenting with two or more adverse determinants had a higher frequency of severe visceral disease complications and required more aggressive therapeutic interventions.Conclusion. 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.
Authors:
Chiara Baldini; Pasquale Pepe; Luca Quartuccio; Roberta Priori; Elena Bartoloni; Alessia Alunno; Angelica Gattamelata; Marta Maset; Mariagrazia Modesti; Antonio Tavoni; Salvatore De Vita; Roberto Gerli; Guido Valesini; Stefano Bombardieri
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-12-24
Journal Detail:
Title:  Rheumatology (Oxford, England)     Volume:  -     ISSN:  1462-0332     ISO Abbreviation:  Rheumatology (Oxford)     Publication Date:  2013 Dec 
Date Detail:
Created Date:  2013-12-26     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100883501     Medline TA:  Rheumatology (Oxford)     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
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