Background: Outcome measures for DD have been developed for different connective tissue disorders, but not for SjS so far. Objectives: A collaborative Italian study was performed to reach an agreement on the definition of DD in SjS and construct a valid index for measurement of that. Methods: Clinical and serological data from patients with primary SjS from different centres were collected using a standardized clinical chart. DD was assessed by the observers in each enrolled patient, by means a specific score (from 0 to 10 points) on the basis of his/her expertise. Univariate and multivariate procedures were performed on the whole database of patients to select the clinical and serological variables which showed to be the best predictors of DD. Correlation coefficients of variables were used as indicators of their relative weight in multivariate models. Using the selected weighted variables, a new scoring system (SjSDDI) was built potentially able to measure DD in patients with SjS. The scores obtained with the SjSDDI were compared to those given by observers. Results: Data from 155 patients with primary SjS provided by 11 centres were collected. They were 4 men and 151 women, aged from 18 to 84 yrs. (mean 54.1), with a disease duration ranging from 1 to 43 yrs. (mean 8.74). Nine variables appeared to be strictly related to the DD as scored by the observers. These were reduced lachrymal production and the consequent ocular damages, reduced salivary flow and loss of teeth, pleural/pulmonary fibrosis, permanent impairment of central and peripheral nervous system, permanent loss of renal function, presence of lymphoproliferative disease. The DD scores calculated by using SjSDDI were closely correlated with the scores given by the observers (r 0.786, p<0.0001). The two scores were identical or differ from 1 point in 76.2%, 2 points in 22%, and 3 point in only 1.8% of patients. Conclusion: The SjSDDI appears to be a promising instrument to objectively measure DD in patients with primary SjS. Further studies in larger series of patients should be carried out to obtain its final definition and validation.
ASSESSMENT OF DISEASE DAMAGE (DD) IN SJØGREN'S SYNDROME (SJS) BY MEANS OF A NEW INDEX (I) (SJSDDI) DEVELOPED FROM THE ANALYSIS OF A COHORT OF PATIENTS COLLECTED BY THE STUDY GROUP FOR SJS OF THE ITALIAN SOCIETY OF RHEUMATOLOGY
GOVONI, Marcello;
2006
Abstract
Background: Outcome measures for DD have been developed for different connective tissue disorders, but not for SjS so far. Objectives: A collaborative Italian study was performed to reach an agreement on the definition of DD in SjS and construct a valid index for measurement of that. Methods: Clinical and serological data from patients with primary SjS from different centres were collected using a standardized clinical chart. DD was assessed by the observers in each enrolled patient, by means a specific score (from 0 to 10 points) on the basis of his/her expertise. Univariate and multivariate procedures were performed on the whole database of patients to select the clinical and serological variables which showed to be the best predictors of DD. Correlation coefficients of variables were used as indicators of their relative weight in multivariate models. Using the selected weighted variables, a new scoring system (SjSDDI) was built potentially able to measure DD in patients with SjS. The scores obtained with the SjSDDI were compared to those given by observers. Results: Data from 155 patients with primary SjS provided by 11 centres were collected. They were 4 men and 151 women, aged from 18 to 84 yrs. (mean 54.1), with a disease duration ranging from 1 to 43 yrs. (mean 8.74). Nine variables appeared to be strictly related to the DD as scored by the observers. These were reduced lachrymal production and the consequent ocular damages, reduced salivary flow and loss of teeth, pleural/pulmonary fibrosis, permanent impairment of central and peripheral nervous system, permanent loss of renal function, presence of lymphoproliferative disease. The DD scores calculated by using SjSDDI were closely correlated with the scores given by the observers (r 0.786, p<0.0001). The two scores were identical or differ from 1 point in 76.2%, 2 points in 22%, and 3 point in only 1.8% of patients. Conclusion: The SjSDDI appears to be a promising instrument to objectively measure DD in patients with primary SjS. Further studies in larger series of patients should be carried out to obtain its final definition and validation.I documenti in SFERA sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
