Primary Sjögren's syndrome: Extraglandular manifestations and hydroxychloroquine therapy.

The use of hydroxychloroquine (HCQ) in Primary Sjögren's Syndrome (pSS) has been assessed in different studies over the last years, with conflicting results regarding its efficacy in sicca syndrome and extraglandular manifestations (EGM). The goal of this study was to compare the incidence rate of EGM in pSS patients with and without HCQ therapy.We performed a multicenter retrospective study, including patients with pSS (European classification criteria) with at least 1 year of follow-up. Subjects with concomitant fibromyalgia, autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis were excluded. Demographics and pSS characteristics were recorded. The EGM were defined by EULAR-SS disease activity index (ESSDAI). Patients were divided into two groups according to their use or not of HCQ therapy. We evaluated the use of HCQ and its relationship to EGM. HCQ therapy was defined as the continuous use of the drug for at least 3 months. A descriptive analysis of demographics and pSS characteristics was performed. We compared the incidence of EGM between groups defined by HCQ therapy using chi2 test or Fisher's exact test. A total of 221 patients were included (97.3% women), mean age, 55.7 years (SD 14). Mean age at diagnosis, 48.8 years (SD 15); median disease duration, 60 months (IQR 35-84). One hundred and seventy patients (77%) received HCQ. About half of the patients had at least one EGM during the course of the disease, 20% of them developed an EGM before the onset of the sicca syndrome and 26% simultaneously with dryness symptom. Overall, EGM were less frequent in those on HCQ therapy (36.5% vs 63.5%, p < 0.001). Considering each EGM individually, the following manifestations were more frequent in the non-treated group: arthritis (p < 0.001), fatigue (p < 0.001), purpura (p = 0.01), Raynaud phenomenon (p = 0.003), and hypergammaglobulinemia (p = 0.006). Immunosuppressive treatment was indicated on 28 patients (12.7%), 13 of which were receiving also HCQ. The first reason for those treatments was the presence of arthritis in 12/28 patients (42.8%), and the drug used in all the cases was methotrexate. Only three patients required immunosuppressive therapy with cyclophosphamide, due to the presence of glomerulonephritis, vasculitis, and interstitial lung disease. None of the patients received biologic therapy. The lower incidence of EGM was observed in patients on HCQ therapy supports its efficacy in pSS. However, further large scale prospective studies are needed to confirm these findings.

Frecuencia de úlceras digitales en esclerodermia / Digital ulcers frequency in scleroderma

Nuestro objetivo fue describir la frecuencia de úlceras digitales en una población de pacientes con Esclerosis Sistémica y comparar las características clínicas de los que desarrollaron úlceras de los que no lo hicieron. Se incluyeron en forma retrospectiva pacientes que cumplían criterios ACR para Esclerosis Sistémica. Se recolectaron datos demográficos, clínicos y serológicos de las historias clínicas. Se clasificó a los pacientes en dos grupos: un Grupo A con úlceras digitales y Grupo B aquellos pacientes sin antecedentes de úlceras digitales. Se compararon ambos grupos. Se estudiaron 60 pacientes con diagnóstico de Esclerosis Sistémica, 33% subtipo difuso, edad promedio al diagnóstico de 50,75 ± 14,75 años, el 15% (9 pacientes) eran de sexo masculino. La frecuencia de úlceras digitales fue de 33,33% (n=20). Los pacientes del grupo A eran más jóvenes al momento del diagnóstico (p=0,03) y tenían más tiempo de evolución de la enfermedad (pNS). En este grupo, fue más frecuente la forma difusa (p=0,002) y el fenómeno de Raynaud se inició a edades más precoces (p=0,006). Los pacientes del grupo A presentaron menor capacidad funcional, medida por HAQ score, y mayor frecuencia de patente tardía en la capilaroscopia, aunque no fue estadísticamente significativo. La totalidad de los pacientes de género masculino (n=9) tuvieron úlceras digitales (p <0,0001).Conclusión: El desarrollo de úlceras digitales en nuestro estudio se asoció a inicio más precoz de la enfermedad, a sexo masculino, a la forma difusa y a inicio más temprano de fenómeno de Raynaud.

The aim of this study was to describe digital ulcer frequency ina patient population with Systemic Sclerosis and to compare theclinical features of those who developed ulcers with those who didnot. Retrospectively, patients meeting ACR criteria for SystemicSclerosis were included. Demographic, clinical, and serologicalinformation was obtained from medical records. Patients wereclassified into two groups: Group A comprised patients with digitalulcers, while Group B included patients with no digital ulcers ontheir records. Findings for both groups were compared. We studied60 patients diagnosed with Systemic Sclerosis, 33% with diffusecutaneous subset, the mean age at the time of diagnosis was 50.75± 14.75 years, 15% (9 patients) were males. The frequency of digitalulcers was 33.33% (n=20). Group A patients were younger at thetime of diagnosis (p=0.03) and had longer time of evolution (pNS).The diffuse variant was more frequent in this group (p=0.002) andRaynaud’s phenomenon onset occurred at earlier ages (p=0.006).All male patients (n=9) entered into the study had digital ulcers (p<0.0001).Conclusions: In our study, development of digital ulcers wasassociated with an earlier onset of disease, males, diffuse subsetand an earlier onset of Raynaud’s phenomenon.

Liver transplantation in systemic lupus erythematosus: case report and review of the literature.

Severe liver involvement requiring liver transplantation is a rare complication in systemic lupus erythematosus (SLE), but very few cases have been reported. We describe a 39-year-old woman with SLE who underwent successful liver transplantation due to acute liver failure. The patient persisted without reactivation of SLE and with good long-term survival. Management and diagnosis considerations are reviewed.

Utilidad de la videocapilaroscopia de lecho ungueal en el síndromede Sjõgren con fenómeno de Raynaud / Utility of nailfold videocapillaroscopy in Sjõgren's syndrome with Raynaud's

La videocapilaroscopia de lecho ungueal (VCLU) es un método sencillo, no invasivo, de bajo costo y útil para la evaluación de las anormalidades microvasculares halladas en las enfermedades del tejido conectivo (ETC)1. Describimos una paciente con síndrome de Sjõgren (SS) primario que presentó alteraciones características en la VCLU y desarrolló una esclerosis sistémica (ES) limitada.