Unidad de enfermedades autoinmunes: experiencia de un centro / Autoimmune Diseases Unit: experience of a center / Unidade de doenças autoimunes: experiência de um centro

Resumen: Introducción: Las enfermedades autoinmunes sistémicas son un grupo de enfermedades de baja prevalencia, cuya patogenia está basada en la pérdida de la auto-tolerancia. A pesar de su baja incidencia y prevalencia, la profundización en el estudio y conocimiento de estas enfermedades ha permitido importantes avances diagnósticos y terapéuticos en los últimos años, constituyendo un desafío en la práctica clínica. Objetivo: determinar la prevalencia y características clínico-humorales de estas patologías en la Policlínica de Enfermedades Autoinmunes del Hospital Pasteur, en el periodo 2016 -2019. Resultados: El total de población fue de 62 pacientes., 55/62 (88%) son mujeres. La más prevalente fue Lupus Eritematoso Sistémico, representando un 32% de esta población (20/62), la artritis reumatoide en un 21% (13/62), la esclerosis sistémica en un 8% (5/62), vasculitis sistémica 8% (5/62), Overlap 6% (4/62), Bechet 5% (3/62). Conclusiones: Se destaca un claro predominio del lupus y de la artritis reumatoide como enfermedades más prevalentes, así como un incremento mantenido de otras patologías menos habituales. Es de suma importancia fomentar el desarrollo de unidades especializadas en estas enfermedades para poder mejorar y protocolizar el manejo de estos pacientes.

Abstract: Introduction: Systemic Autoimmune Diseases are a group of low-prevalence diseases whose pathogeny is based on the loss of self-tolerance. Despite their low incidence and prevalence, deepening the study and knowledge of these diseases has enabled significant diagnostic and therapeutic advances in recent years, constituting a challenge in clinical practice. Objective: to determine the prevalence and clinical-humoral characteristics of these pathologies in the Polyclinic of Autoimmune Diseases of Pasteur Hospital, in the period 2016 -2019. Results: The total population was 62 patients., 55/62 (88%) were women. Most prevalent was Systemic Lupus Erythematosus (SLE), representing 32% of this population (20/62), Rheumatoid Arthritis by 21% (13/62), Systemic Sclerosis by 8% (5/62), Systemic vasculitis 8% (5/62), Overlap syndrome 6% (4/62), Bechet 5% (3/62). Conclusions: Lupus and rheumatoid arthritis as more prevalent diseases, as well as a maintained increase in other less common diseases. It is very important to promote the development of specialized units in these área, in order to improve and protocol the management of these patients.

Resumo: Introdução: As doenças autoimunes sistêmicas são um grupo de doenças de baixa prevalência, cuja patogênese se baseia na perda de autotolerância. Apesar de sua baixa incidência e prevalência, o aprofundamento no estudo e conhecimento dessas doenças permitiu importantes avanços diagnósticos e terapêuticos nos últimos anos, constituindo um desafio na prática clínica. Objetivo: determinar a prevalência e as características clínico-humorais dessas patologias no Hospital Pasteur Polyclinic of Autoimmune Diseases, no período 2016-2019. Resultados: a população total foi de 62 pacientes, 55/62 (88%) são mulheres. O mais prevalente foi o lúpus eritematoso sistêmico (LES), representando 32% dessa população (20/62), artrite reumatoide em 21% (13/62), esclerose sistêmica em 8% (5 / 62), vasculite sistêmica 8% (5/62), sobreposição de 6% (4/62), Bechet 5% (3/62). Conclusões: Uma clara predominância de lúpus e artrite reumatoide destaca-se como as doenças mais prevalentes, bem como um aumento sustentado de outras patologias menos comuns. É de extrema importância promover o desenvolvimento de unidades especializadas nessas doenças, a fim de melhorar e protocolar o manejo desses pacientes.

Chagas disease and systemic autoimmune diseases among Bolivian patients in Switzerland

BACKGROUND Chronic cardiomyopathy occurs in 20-40% of the patients with Chagas disease. Autoimmune mechanisms may contribute to its pathogenesis. We diagnosed several cases of systemic autoimmune diseases among Bolivian migrants in Geneva with a high prevalence of Chagas disease. OBJECTIVES We tested the hypothesis of a clinical association between systemic autoimmune diseases and Chagas disease, particularly with the development of cardiomyopathy. METHODS We retrospectively searched the medical records of all Bolivian patients visiting Geneva University Hospitals between 2012 and 2015 for diagnosis of Chagas disease or systemic autoimmune diseases. FINDINGS Of the 2,189 eligible patients, 28 [1.3%; 95% confidence interval (CI) = 0.9-1.9%] presented with systemic autoimmune disease. The Chagas status was known in 903 (41.3%) patient, of whom 244 (27.0%; 95% CI = 24.2-30.0%) were positive. Eight (28.6%; 95% CI = 15.3-47.1%) of the 28 cases of systemic autoimmune disease had Chagas disease. We found no association between both entities (p = 1.000) or with Chagasic cardiomyopathy (p = 0.729). Moreover, there was no evidence of a temporal relationship between antiparasitic chemotherapy and the development of systemic autoimmune diseases. CONCLUSIONS Our results do not support a clinical association between chronic Chagas disease and systemic autoimmune diseases. However, prospective studies in areas endemic for Chagas disease should better assess the prevalence of systemic autoimmune diseases and thus a possible relationship with this infection.

The side-effects of cyclop hosphamide in the treatment of systemic autoimmune diseases / 中华风湿病学杂志

Objective To understand the side-effects of cyclophosphamide (CTX) in the treatment of systemic autoimmune diseases and the possible risk factors. Methods Two hundred and forty-one patients with systemic autoimmune diseases were recruited from the Rheumatology Division of Peking University First Hospital during January 1st, 2009 and March 31, 2012. All the patients received oral or intravenous cyclopho-sphamide. The data were collected by medical record review as well as telephone follow-up. Logistic regression analyses were used for statistical analysis. Results Statistical analysis for age, sex, disease, cumulative dose, treatment duration and mode of administration were included in the factor analysis that would impact the CTX related side-effects. Age ( x2=14.8, P=0.002), gender ( x2=11.2, P=0.001), the underlying disease ( x2=26.1, P<0.01), cumulative dose ( x2=9.8, P=0.007) and mode of administration of CTX ( x2=19.5, P<0.01) were all correlated with the incidence of CTX side-effects. Multivariate analysis showed that women [OR=2.32, 95%CI (1.15, 4.70), P=0.02], intravenous-oral sequential use of CTX [OR=5.25, 95%CI (2.30, 11.97), P<0.01] and systemic lupus erythematosus [OR=4.02, 95%CI (2.24, 7.21), P<0.01] as the underlying disease were independent risk factors for CTX side-effects. Conclusion Alopecia, gastrointestinal discomfort and gonads toxicity ware com-monly seen in Chinese patients with systemic autoimmune diseases receiving CTX. Hemor-rhagic cystitis is very rare. Women, intravenous-oral sequential use of CTX and the systemic lupus erythematosus are indepen-dent risk factors for CTX side-effects.