Transcultural adaptation of the EULAR activity index for primary Sjogren's syndrome in Argentine.

OBJECTIVE: To adapt the EULAR Activity Index for primary Sjögren's syndrome (ESSDAI) to the Argentine population. METHODS: observational, cross-sectional study that included patients in a period of ten months. Three Argentine rheumatologists adapted and translated to Spanish the original version in English and the final version was translated back into English by a research associate whose mother language was English. In order to estimate the constructive validity of the index, the visual analogous scale (VAS) of disease activity was used by experts. A subgroup of patients attended a second visit in order to evaluate test-retest reliability. RESULTS: 51 patients were included, 49 (96.1%) were female, the median age was 58 ((interquartile range (IQR): 49-69)). The median global VAS was 10 (IQR: 4-22.25) and the median total ESSDAI score was 5 (IQR: 3-9). The correlation between the global VAS and the total ESSDAI score of the scale was 0.79. The intraclass correlation coefficient was 0.67 (95% CI: 0.32-0.92) for the total score and 0.98 (95% CI: 0.92-0.995) for the global VAS. The results of the correlation coefficient between the VAS and the scale for each domain were: constitutional symptoms: 0.46; lymphadenopathy: 0.76; glandular: 0.78; joint: 0.61; skin: 1; respiratory: 0.83; renal: 1; muscular:- (no patient had myositis); peripheral nervous system: 0.72; central nervous system: 0.67; hematological: 0.96; biomarkers: 0.86. CONCLUSION: The results of this study showed that the ESSDAI is a reliable and valid index for this pSS argentinian population.

[Lung involvement in systemic sclerosis]. / Compromiso pulmonar en esclerosis sistémica.

The objective of this study was to determine clinical predictors of interstitial lung disease in patients with systemic sclerosis (SSc) and pulmonary involvement as defined by presence of a decreased diffusing capacity for carbon monoxide (DLCO). Forty subjects with SSc were retrospectively evaluated. Patients were categorized according to their level of DLCO (< o > or = 80% of predicted). Sensitivity of dyspnea to detect a decreased DLCO was 46.6% and specificity 90%, whereas oxygen desaturation showed a sensitivity of 71.4% and a specificity of 80%. Patients with decreased DLCO (n = 18) were not different in age (51.1 +/- 13.5 vs. 53.5 +/- 9.3 y, p = 0.5182), sex (male 13.6%, p = 0.6088), prevalence of Raynaud (86.6% vs. 85%, p = 0.6272), sicca syndrome (6.2% vs. 10.5% p = 1.0000) diffuse cutaneous involvement (94.1% vs. 83.3%, p = 0.6026) or esophageal dilatation. The duration of symptoms since diagnosis was no different. Prevalence of pulmonary hypertension assessed by Doppler echocardiography or abnormal nailfold capillaroscopic findings were identical in both populations. Patients with low DLCO had a significatly higher prevalence of anti topoisomerase antibodies. (5/9 vs. 0/11, p = 0.0081) and restrictive lung disease. Patients with low DLCO showed a significantly higher prevalence of abnormal HRCT findings suggestive of ILD (82.3% vs. 5.8%, p < or = 0.0001). We conclude that a low DLCO is a frequent finding in SSc patients, strongly associated with HRCT signs of ILD. We have not found clinical factors predictive for a low DLCO.

Compromiso pulmonar en esclerosis sistémica / Lung involvement in systemic sclerosis

El objetivo del estudio fue determinar las características clínicas de los pacientes con esclerodermia y compromiso pulmonar y evaluar si existen factores clínicos predictores de mayor riesgo de enfermedad intersticial. Se estudiaron en forma retrospectiva 40 pacientes con esclerodermia. Fueron divididos en 2 grupos: capacidad de difusión del monóxido de carbono (DLCO) normal (n = 22) y DLCO disminuida (n = 18, 45%). Los pacientes con DLCO disminuida no fueron diferentes en edad (51.1 más o menos 13.5 vs. 53.5 más o menos 9.3 años, p = 0.5182), sexo (varones 13.6%, p = 0.6088 ), presencia de Raynaud (86.6% vs. 85%, p = 0.6272), síndrome de ojo seco (6.2% vs. 10.5%, p = 1.0000) prevalencia de enfermedad difusa (94.1% vs. 83.3%, p = 0.6026) o de dilatación esofágica. El tiempo de evolución de la enfermedad no fue diferente. La sensibilidad de la disnea para detectar una DLCO alterada fue 46.6% con una especificidad del 90% y la de la caída de la saturación de O2 (SaO2) del 71.4% y 80% respectivamente. Los pacientes con DLCO baja tuvieron mayor prevalencia de anticuerpos anti-Scl 70 positivos (5/9 vs. 0/11, p = 0.0081) y de incapacidad ventilatoria restrictiva aunque en 56.7% de los pacientes con DLCO disminuida la capacidad pulmonar total (CPT) era normal. La presencia de hipertensión pulmonar medida por ecocardiograma Doppler fue idéntica (11/13 vs. 10/11, p = 1.0000). Los pacientes con DLCO disminuida tuvieron una prevalencia muy superior de tomografía computada de tórax con evidencias de compromiso intersticial (82.3% vs. 5.8%, p menor o igual a 0.0001). En conclusión, nuestros datos sugieren que la disminución de la DLCO es un hallazgo, muy frecuentemente asociado a TAC de tórax con compromiso intersticial y que no hay variables clínicas que permitan predecir su anormalidad.

The objective of this study was to determine clinical predictors of interstitial lung disease in patients with systemic sclerosis (SSc) and pulmonary involvement as defined by presence of a decreased diffusing capacity for carbon monoxide (DLCO). Forty subjects with SSc were retrospectively evaluated. Patients were categorized according to their level of DLCO (less than or more than or equal to 80% of predicted). Sensitivity of dyspnea to detect a decreased DLCO was 46.6% and specificity 90%, whereas oxygen desaturation showed a sensitivity of 71.4% and a specificity of 80%. Patients with decreased DLCO (n = 18) were not different in age (51.1 more or less than 13.5 vs. 53.5 more or less than 9.3 y, p = 0.5182), sex (male 13.6%, p = 0.6088), prevalence of Raynaud (86.6% vs. 85%, p = 0.6272), sicca syndrome (6.2% vs. 10.5% p = 1.0000) diffuse cutaneous involvement (94.1% vs. 83.3%, p = 0.6026) or esophageal dilatation. The duration of symptoms since diagnosis was no different. Prevalence of pulmonary hypertension assessed by Doppler echocardiography or abnormal nailfold capillaroscopic findings were identical in both populations. Patients with low DLCO had a significatly higher prevalence of anti topoisomerase antibodies. (5/9 vs. 0/11, p = 0.0081) and restrictive lung disease. Patients with low DLCO showed a significantly higher prevalence of abnormal HRCT findings suggestive of ILD (82.3% vs. 5.8%, p less than or equal to 0.0001). We conclude that a low DLCO is a frequent finding in SSc patients, strongly associated with HRCT signs of ILD. We have not found clinical factors predictive for a low DLCO.

Immune complexes inhibit differentiation, maturation, and function of human monocyte-derived dendritic cells.

The interaction between immune complexes (IC) and the receptors for the Fc portion of IgG (FcgammaRs) triggers regulatory and effector functions in the immune system. In this study, we investigated the effects of IC on differentiation, maturation, and functions of human monocyte-derived dendritic cells (DC). When IC were added on day 0, DC generated on day 6 (IC-DC) showed lower levels of CD1a and increased expression of CD14, MHC class II, and the macrophage marker CD68, as compared with normally differentiated DC. The use of specific blocking FcgammaR mAbs indicated that the effect of IC was exerted mainly through their interaction with FcgammaRI and to a lesser extend with FcgammaRII. Immature IC-DC also expressed higher levels of CD83, CD86, and CD40 and the expression of these maturation markers was not further regulated by LPS. The apparent lack of maturation following TLR stimulation was associated with a decreased production of IL-12, normal secretion of IL-10 and CCL22, and increased production of CXCL8 and CCL2. IC-DC displayed low endocytic activity and a reduced ability to induce allogeneic T cell proliferation both at basal and LPS-stimulated conditions. Altogether, these data reveal that IC strongly affect DC differentiation and maturation. Skewing of DC function from Ag presentation to a proinflammatory phenotype by IC resembles the state of activation observed in DC obtained from patients with chronic inflammatory autoimmune disorders, such as systemic lupus erythematosus disease and arthritis. Therefore, the altered maturation of DC induced by IC may be involved in the pathogenesis of autoimmune diseases.