RESUMO
BACKGROUND: Acute transverse myelitis (ATM) is an infrequent but severe complication of systemic lupus erythematosus (SLE). The purpose of study was to describe clinical features and prognostic factors of patients with SLE-related ATM. METHODS: In this medical records review study, data were collected from 60 patients from 16 centers seen between 1996 and 2017 who met diagnostic criteria for SLE and myelitis as defined by the American College of Rheumatology/Systemic International Collaborating Clinics and the Working Group of the Transverse Myelitis Consortium, respectively. Objective neurological impairment was measured with American Spinal Injury Association Impairment Scale (AIS) and European Database for Multiple Sclerosis Grade Scale (EGS). RESULTS: Among patients included, 95% (n = 57) were female, and the average age was 31.6 ± 9.6 years. Myelitis developed after diagnosis of SLE in 60% (n = 36). Symmetrical paraparesis with hypoesthesia, flaccidity, sphincter dysfunction, AIS = A/B, and EGS ≥ 8 was the most common presentation. Intravenous methylprednisolone was used in 95% (n = 57), and 78.3% (n = 47) received intravenous cyclophosphamide. Sensory/motor recovery at 6 months was observed in 75% (42 of 56), but only in 16.1% (9 of 56) was complete. Hypoglycorrhachia and EGS ≥ 7 in the nadir were associated with an unfavorable neurological outcome at 6 months (p < 0.05). A relapse rate during follow-up was observed in 30.4% (17 of 56). Hypoglycorrhachia and hypocomplementemia seem to be protective factors for relapse. Intravenous cyclophosphamide was associated with time delay to relapse. CONCLUSIONS: Systemic lupus erythematosus-related ATM may occur at any time of SLE course, leading to significant disability despite treatment. Relapses are infrequent and intravenous cyclophosphamide seems to delay it. Hypoglycorrhachia, hypocomplementemia, and EGS at nadir are the most important prognostic factors.
Assuntos
Lúpus Eritematoso Sistêmico , Mielite Transversa , Adulto , Feminino , Humanos , América Latina , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Mielite Transversa/diagnóstico , Mielite Transversa/tratamento farmacológico , Mielite Transversa/epidemiologia , Recidiva Local de Neoplasia , Prognóstico , Adulto JovemRESUMO
Resumen La vasculitis reumatoide constituye una complicación inusual, pero severa, de la artritis reumatoide, caracterizada por un proceso inflamatorio que compromete vasos sanguíneos de pequeño y mediano calibre, asociada o no a manifestaciones extraarticulares. Algunos factores predisponentes descritos incluyen ciertos haplotipos de antígenos leucocitarios humanos, fumado y enfermedad de larga data. Una disminución en la incidencia ha sido notada desde los años 90 y confirmada en estudios recientes, asociada en particular a un diagnóstico precoz de artritis reumatoide y estrategias terapéuticas que incluyen un aumento en la prescripción de metotrexate. La vasculitis reumatoide se asocia a tasas de mortalidad hasta del 40% a los 5 años y morbilidad importante. Se presentan dos pacientes con diagnóstico previo de artritis reumatoide, con un cuadro crónico de úlceras y disestesias en miembros inferiores, asociado a síndrome anémico. Los estudios histológicos revelaron vasculitis de mediano vaso compatible con vasculitis reumatoide. Se inició manejo con inmunosupresores más esteroides, con resolución del cuadro clínico inicial.
Abstract Rheumatoid vasculitis remains a rare but serious complication of rheumatoid arthritis characterized by an inflammatory process that primarily affects small to medium-sized blood vessels, it can be associated with other extra-articular manifestations. A number of predictor or predisposing factors including certain human leukocyte antigen haplotypes, smoking and long-standing disease. A declining trend in the incidence of rheumatoid vasculitis has been noted since the 1990s and reconfirmed in recent studies, related to early diagnosis of rheumatoid arthritis, and widespread use of methotrexate. Rheumatoid vasculitis is associated with high rates of premature mortality with up to 40% of patients dying by 5 years, as well as a significant morbidity. We present two patients with diagnosis of rheumatoid arthritis with a chronic condition of ulcers and dysesthesias on legs associated with anemic syndrome; the histological study revealed medium size vasculitis. Treatment with inmunosuppresants and steroids was given with resolution of symptoms.
Assuntos
Humanos , Feminino , Adulto , Pessoa de Meia-Idade , Febre Reumática/complicações , Costa Rica , Vasculite Reumatoide/diagnósticoRESUMO
ResumenJustificación y objetivos:el uso de fármacos antagonistas del factor de necrosis tumoral alfa se asocia a infecciones severas en artritis reumatoide, con una alta morbi-mortalidad en la práctica clínica. No existe casuística publicada respecto al tema en nuestro país. Se pretende aportar información relacionada con la epidemiología local de las infecciones severas e identificar factores de riesgo asociados.Métodos:estudio observacional retrospectivo, que incluyó 50 pacientes tratados al menos un año en el periodo 2006-2012. Se evaluaron las características demográficas, así como las clínicas y epidemiológicas de la(s) infección(es) severa(s) desarrollada(s) y los factores de riesgo asociados.Resultados:la mayor parte de los pacientes fueron mujeres en edad media. Solo se documentó una infección severa, que correspondió a una infección de piel y tejido blando que resolvió con terapia antibiótica intravenosa. Se registraron factores de riesgo, especialmente en uso de terapia inmunosupresora esteroidal y el antecedente quirúrgico reciente, con una baja prevalencia de comorbilidades.Conclusiones:el perfil demográfico, así como el perfil clínico de las infecciones severas es similar a otras poblaciones, la baja incidencia de éstas en la cohorte podría relacionarse a una menor prevalencia de comorbilidades, no obstante se requiere investigaciones futuras para corroborar o descartar dicha observación.
AbstractAim and objetives:the use of tumour necrosis factor alpha therapy antagonist leads to an increased risk of serious infections in rheumatoid arthritis, with a high morbi-mortality in clinical practice. There are no published data in our country. This study pretends to provide knowledge about the local epidemiology of serious infections and to identify associated risk factors.Methods:An observational and retrospective study, included 50 patients treated at least for one year between 2006-2012. Demographic characteristics, clinical and epidemiological characteristics of serious infections and risk factors associated with serious infections were described.Results:A predominant female population in middle age was detected. Just one serious infection was described, corresponding to serious skin and soft tissue infection that resolved with intravenous antibiotics. Risk factors were described including inmunosuppresive therapy with steroids and prior surgery.Conclusions:Demographic profile of analized population and their clinic profile of serious infectios are similar to others populations. Low incidence of serious infections in this cohort could be related with less comorbilities than others populations, although, this topic needs further investigations to corroborate or discard this observation.