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Clinical Characteristics and Outcome of Primary Sjogren's Syndrome: A Large Asian Indian Cohort.

Sandhya P, Jeyaseelan L, Scofield RH, Danda D - Open Rheumatol J (2015)

Bottom Line: Clinical improvement including symptomatic benefit in sicca and musculoskeletal features was noted with immunomodulators in the majority.The first cluster was associated with major systemic illness and high antibody titers, where as the second cluster comprised of seronegative patients with mild disease.Association of antibody positivity with systemic features was further confirmed on logistic regression analysis.

View Article: PubMed Central - PubMed

Affiliation: Department of Clinical Immunology and Rheumatology, Christian Medical College and Hospital, Vellore-632004, Tamil Nadu, India.

ABSTRACT

Objective: To characterise the clinical features, immunological profile and outcome in a cohort of Asian Indian patients with primary Sjögren's syndrome (SS).

Methods: Electronic medical records from a tertiary care teaching hospital in south India were screened for SS between 2004 and 2011. Patients fulfilling American European Consensus group (AECG) 2002 or American College of Rheumatology (ACR) 2012 classification criteria were included. Agglomerative hierarchical cluster analysis to identify patterns of associations between clinical and immunological features was done. Multivariate logistic regression to identify predictors of major systemic involvement was performed. Data on treatment and outcome were retrieved from electronic records.

Results: Of 423 patients suspected to have SS, 332 fulfilled inclusion criteria. Only 8.3% of patients complained of sicca symptoms on their own at initial presentation. Younger age of onset, higher female to male ratio, paucity of cryoglobulinemia, Raynaud's phenomenon and hyperglobulinemia were unique to this cohort. Cluster analysis revealed two subsets: The first cluster comprised of patients having a major systemic illness with high antibody titers and the second comprised of seronegative patients with mild disease. Over a third of SS cases had severe systemic manifestations necessitating treatment with immunosuppressants. In multivariate logistic regression analysis, anti-Ro and anti-La antibody positivity was associated with higher odds for systemic disease features (OR=2.67, P=0.03 and OR=3.25, P=0.003, respectively) whereas chronic pain was associated with lower odds (OR=0.4, p=0.032). Clinical improvement including symptomatic benefit in sicca and musculoskeletal features was noted with immunomodulators in the majority.

Conclusion: Our cohort of patients with SS has characteristic clinical features; some of them are in contrast with previous observations reported in European patients. This cohort consisted of two distinct patient clusters. The first cluster was associated with major systemic illness and high antibody titers, where as the second cluster comprised of seronegative patients with mild disease. Association of antibody positivity with systemic features was further confirmed on logistic regression analysis.

No MeSH data available.


Related in: MedlinePlus

Cluster analysis of clinical and immunological characteristics. Agglomerative hierarchical cluster analysis was done to identifypatterns of associations between different clinical and immunological features of SS. Phi correlation was calculated as a measure ofcorrelation between the categorical variables. Distance between phi correlation coefficient was calculated using the Euclidian distance andthe average linkage method was used to agglomerate the clusters.
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Figure 1: Cluster analysis of clinical and immunological characteristics. Agglomerative hierarchical cluster analysis was done to identifypatterns of associations between different clinical and immunological features of SS. Phi correlation was calculated as a measure ofcorrelation between the categorical variables. Distance between phi correlation coefficient was calculated using the Euclidian distance andthe average linkage method was used to agglomerate the clusters.

Mentions: Cluster analysis results of clinical and immunological characteristics as described earlier are depicted in Fig. (1). The cluster analysis indicated that our cohort consisted of two subsets of SS population. I - The first subset included those with serological positivity (ANA, RF, anti-Ro, anti-La) and extra glandular major systemic manifestations namely dermatological, vasculitic, neurological and renal involvement requiring high dose steroids. II - The other subset consisted of those with sicca symptoms, low 25(OH) D3(<45 nmol/L) and generally mild manifestations including chronic pain, arthralgia or non-deforming chronic arthritis etc. This subset did not cluster with serological positivity.


Clinical Characteristics and Outcome of Primary Sjogren's Syndrome: A Large Asian Indian Cohort.

Sandhya P, Jeyaseelan L, Scofield RH, Danda D - Open Rheumatol J (2015)

Cluster analysis of clinical and immunological characteristics. Agglomerative hierarchical cluster analysis was done to identifypatterns of associations between different clinical and immunological features of SS. Phi correlation was calculated as a measure ofcorrelation between the categorical variables. Distance between phi correlation coefficient was calculated using the Euclidian distance andthe average linkage method was used to agglomerate the clusters.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4493630&req=5

Figure 1: Cluster analysis of clinical and immunological characteristics. Agglomerative hierarchical cluster analysis was done to identifypatterns of associations between different clinical and immunological features of SS. Phi correlation was calculated as a measure ofcorrelation between the categorical variables. Distance between phi correlation coefficient was calculated using the Euclidian distance andthe average linkage method was used to agglomerate the clusters.
Mentions: Cluster analysis results of clinical and immunological characteristics as described earlier are depicted in Fig. (1). The cluster analysis indicated that our cohort consisted of two subsets of SS population. I - The first subset included those with serological positivity (ANA, RF, anti-Ro, anti-La) and extra glandular major systemic manifestations namely dermatological, vasculitic, neurological and renal involvement requiring high dose steroids. II - The other subset consisted of those with sicca symptoms, low 25(OH) D3(<45 nmol/L) and generally mild manifestations including chronic pain, arthralgia or non-deforming chronic arthritis etc. This subset did not cluster with serological positivity.

Bottom Line: Clinical improvement including symptomatic benefit in sicca and musculoskeletal features was noted with immunomodulators in the majority.The first cluster was associated with major systemic illness and high antibody titers, where as the second cluster comprised of seronegative patients with mild disease.Association of antibody positivity with systemic features was further confirmed on logistic regression analysis.

View Article: PubMed Central - PubMed

Affiliation: Department of Clinical Immunology and Rheumatology, Christian Medical College and Hospital, Vellore-632004, Tamil Nadu, India.

ABSTRACT

Objective: To characterise the clinical features, immunological profile and outcome in a cohort of Asian Indian patients with primary Sjögren's syndrome (SS).

Methods: Electronic medical records from a tertiary care teaching hospital in south India were screened for SS between 2004 and 2011. Patients fulfilling American European Consensus group (AECG) 2002 or American College of Rheumatology (ACR) 2012 classification criteria were included. Agglomerative hierarchical cluster analysis to identify patterns of associations between clinical and immunological features was done. Multivariate logistic regression to identify predictors of major systemic involvement was performed. Data on treatment and outcome were retrieved from electronic records.

Results: Of 423 patients suspected to have SS, 332 fulfilled inclusion criteria. Only 8.3% of patients complained of sicca symptoms on their own at initial presentation. Younger age of onset, higher female to male ratio, paucity of cryoglobulinemia, Raynaud's phenomenon and hyperglobulinemia were unique to this cohort. Cluster analysis revealed two subsets: The first cluster comprised of patients having a major systemic illness with high antibody titers and the second comprised of seronegative patients with mild disease. Over a third of SS cases had severe systemic manifestations necessitating treatment with immunosuppressants. In multivariate logistic regression analysis, anti-Ro and anti-La antibody positivity was associated with higher odds for systemic disease features (OR=2.67, P=0.03 and OR=3.25, P=0.003, respectively) whereas chronic pain was associated with lower odds (OR=0.4, p=0.032). Clinical improvement including symptomatic benefit in sicca and musculoskeletal features was noted with immunomodulators in the majority.

Conclusion: Our cohort of patients with SS has characteristic clinical features; some of them are in contrast with previous observations reported in European patients. This cohort consisted of two distinct patient clusters. The first cluster was associated with major systemic illness and high antibody titers, where as the second cluster comprised of seronegative patients with mild disease. Association of antibody positivity with systemic features was further confirmed on logistic regression analysis.

No MeSH data available.


Related in: MedlinePlus