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.

Validación del Índice Funcional para Osteoartritis de Manos (FIHOA) en pacientes con artritis reumatoidea / Validation of the Functional Index for Hand Osteoarthritis (FIHOA) in patients with rheumatoid arthritis

El FIHOA fue desarrollado para evaluar la capacidad funcional de pacientes con OA de manos. Objetivo: Validar FIHOA en pacientes con AR. Métodos: Estudio analítico, observacional prospectivo de corte transversal. Se incluyeron pacientes consecutivos con diagnóstico de AR (ACR/EULAR 2010). Se consignaron datos demográficos y características de la enfermedad. Todos los pacientes completaron los siguientes cuestionarios autoadministrados: FIHOA, HAQ-A, HAQ-UP-A y Quick DASH. En un subgrupo de pacientes, una terapista ocupacional valoró la capacidad funcional de la mano por medio del test de SODA-A. Se evaluó la reproducibilidad de FIHOA. Análisis estadístico: Estadística descriptiva. Confiabilidad con test de Cronbach. Validez de constructo con correlación de Spearman. Reproducibilidad test re-test. Modelo de regresión lineal. Resultados: Se incluyeron 100 pacientes. La prueba alfa de Cronbach fue de 0,94. No se evidenciaron preguntas redundantes. El FIHOA mostró excelente correlación con HAQ-A (r=0,89); HAQ-UP-A (r=0,89); Quick DASH (r=0,90) y SODA-A (r=-0,80); y buena correlación con DAS28-ERS (r=0,65), y con otros parámetros de la enfermedad. La reproducibilidad fue 0,73. La regresión lineal múltiple mostró como principal determinante del FIHOA a la presencia de rigidez matinal seguida por el uso de corticoides y el EVA general de pacientes. Conclusión: El FIHOA resultó ser confiable, válido y reproducible en pacientes con AR

FIHOA was developed to evaluate the functional capacity of patients with OA hands. Objetive: To validate FIHOA in patients with RA. Methods: Analytical, observational, prospective cross-sectional study. Consecutive patients with diagnosis of RA (ACR/EULAR 2010) were included. Demographic and RA characteristics were recorded. Patients completed the following self-administered questionnaires: FIHOA, HAQ-A, HAQ-UP-A and Quick DASH. For a patient subgroup, an occupational therapist performed an objective evaluation of the functional capacity of the hands using the SODA-A. Reproducibility was assessed. Statistical analysis: Descriptive statistics. Reliability with the Cronbach test. Construct validity with Spearman correlation. Reproducibility with test-retest reliability. Linear regression model. Results: One hundred patients were included. Cronbach's alpha test was 0.94. There were no redundant questions. FIHOA showed an excellent correlation with HAQ-A (r=0.89); HAQUP-A (r=0.89); Quick DASH (r=0.90) and SODA-A (r=-0.80); and a good correlation with DAS28- ERS (r=0.65) and with other disease parameters. Questionnaire reproducibility was 0.73. A multiple linear regression showed morning stiffness as the main determinant of FIHOA, followed by glucocorticoid use and patient global assessment. Conclusion: FIHOA was found to be reliable, valid and reproducible in patients with RA

Epidemiological characteristics of pregnancy, delivery, and birth outcome in women with multiple sclerosis in Argentina (EMEMAR study).

BACKGROUND: The influence of pregnancy on Multiple Sclerosis (MS) has been extensively studied but such influence on Latin American women with MS has not been characterized. Our objective was to describe the course of pregnancy and birth outcome in Argentinean MS patients and the evolution of MS during pregnancy and after delivery. METHOD: We used a retrospective design in eight MS centers in Argentina and administered a survey to women with definite MS (Mc Donald) with pregnancies during or after MS onset. We contacted 355 women of which 81 met inclusion criteria. We recorded 141 pregnancies. RESULTS: Involuntary abortion was observed in 16% of pregnancies (95% CI = 10-23). Thirty five women received immunomodulatory therapy (IMT) before 42 pregnancies. Twenty three (55%) out of 42 pregnancies were exposed to IMT. The mean time of IMT discontinuation before conception in 19 (45.2%) pregnancies without exposure, was 104 days (95% CI = 61.0-147.0). There were 103 deliveries: 79% full term. Birth defects were detected in 19% of pregnancies exposed to IMT (95% CI = 4-46) and in 2% of non-exposed (95% CI = 0.3-8.0). The mean relapse rate was: pre-pregnancy year: 0.22 (95% CI = 0.12-0.32); pregnancy: 0.31 in 1st (95% CI = 0.10-0.52), 0.19 (95% CI = 0.03-0.36) in 2nd, and 0.04 in 3rd trimester (95% CI = -0.04-0.12); 1st trimester post delivery: 0.82 (95% CI = 0.42-1.22). CONCLUSION: We observed a higher rate of birth defects among infants exposed to immunomodulators in utero than those not exposed. The reduction in MS relapses during 2nd and 3rd trimester of pregnancy and its increase during postpartum is consistent with previous reports.

Polineuropatía paraneoplásicas: estudio clínico e inmunopatológico / Paraneoplasic polineuropathies: clinical and immunopathological study

Las afecciones paraneoplásicas del sistema nervioso periférico son conocidas desde el año 1948. Sin embargo son aún controvertidos los posibles mecanismos que las condicionan. El presente trabajo se propone evaluar el probable rol de los mecanismos inmunológicos en la génesis de las polineuropatías paraneoplásicas. Se estudiaron 7 pacientes portadores, 6 de carcinomas de pulmón y uno de carcinoma de ovario, en los cuales se desscartaron otros causas de polineuropatías (diabetes, colagenopatías, paraproteinemias, distiroidismos, etilismo, tóxicos, y tratamiento quimioterápico). Los estudios EMG demostraron en 57% de los casos formas asintomáticas. La mayoría de estos estudios fueron compatibles con polineuropatías axónicas o axónicas secundariamente desmielinizantes. Las biopsias musculares evidenciaron cuadrios de atrofía por denervación o atrofía selectiva tipo II y fenómenos de reinervación con reagrupamiento bistoquímico. Los estudios de nervio sural demostraron en el 70% de los casos fenómenos de disminución de las fibras mielínicas y cuadros de remielinización. Se objetivaron depósito inmune (inmunoglobulinas G y M) en las paredes vasculares, espacios perivasculares, subperineurales y endoneurales en 6 de los casos. El estudio de Ac séricos contra estructuras del nervio periférico estudiados en 5 pacientes, fue negativo en todos ellos. Estos hallazgos hacen suponer que si bien existirían desórdenes inmunes en este tipo de polineuropatías, no se puede atribuir a ellos la patogenicidad de estas entidades, constituyendo quizás tan sólo epifenómenos del cuadro nosológico