RESUMEN
OBJECTIVE: Among specific autoantibodies in DM, the anti-small ubiquitin-like modifier activating enzyme (SAE) antibody is rare. We aim to describe the clinical characteristics, cancer prevalence, and muscle pathology of anti-SAE-positive DM. METHODS: Patients with a diagnosis of DM and sera positive for the anti-SAE antibody were recruited from 19 centres in this retrospective observational study. The available muscular biopsies were reviewed. We conducted a comparison with anti-SAE-negative DM and a review of the literature. RESULTS: Of the patients in the study (n = 49), 84% were women. Skin involvement was typical in 96% of patients, with 10% having calcinosis, 18% ulceration and 12% necrosis; 35% presented with a widespread skin rash. Muscular disease affected 84% of patients, with mild weakness [Medical Research Council (MRC) scale 4 (3, 5)], although 39% of patients had dysphagia. Muscular biopsies showed typical DM lesions. Interstitial lung disease was found in 21% of patients, mainly with organizing pneumonia pattern, and 26% of patients showed dyspnoea. Cancer-associated myositis was diagnosed in 16% of patients and was responsible for the majority of deaths, its prevalence being five times that of the general population. IVIG therapy was administered to 51% of the patients during the course of the disease. Comparison with anti-SAE-negative DM (n = 85) showed less and milder muscle weakness (P = 0.02 and P = 0.006, respectively), lower creatinine kinase levels (P < 0.0001) and less dyspnoea (P = 0.003). CONCLUSION: Anti-SAE positive DM is a rare subgroup associated with typical skin features but a potentially diffuse rash, a mild myopathy. Interstitial lung disease defines an organizing pneumonia pattern. Cancer associated DM prevalence is five times that of the general population. TRIAL REGISTRATION: ClinicalTrials.gov, http://clinicaltrials.gov, NCT04637672.
Asunto(s)
Dermatomiositis , Exantema , Enfermedades Pulmonares Intersticiales , Miositis , Neoplasias , Humanos , Femenino , Masculino , Autoanticuerpos , Dermatomiositis/complicaciones , Miositis/diagnóstico , Exantema/epidemiología , Neoplasias/epidemiología , Neoplasias/complicaciones , Enzimas Activadoras de Ubiquitina , Enfermedades Pulmonares Intersticiales/epidemiología , Enfermedades Pulmonares Intersticiales/complicaciones , Disnea , Estudios Observacionales como AsuntoRESUMEN
BACKGROUND: Thalidomide has shown excellent results for severe cutaneous lupus erythematosus (CLE), but its prescription is limited by potentially severe adverse events. OBJECTIVE: To assess the overall rate of response to thalidomide in CLE with respect to CLE subtypes and the occurrence rate of relevant adverse events on the basis of previously published studies. METHODS: We performed a systematic review and meta-analysis of studies published in MEDLINE, Embase, and the Cochrane Library between 1965 and January 2017. The proportions of responders and rates of adverse events were extracted from individual studies and pooled using random effects or fixed models. RESULTS: Among 548 patients from 21 included studies, the overall rate of response to thalidomide was 90% (95% confidence interval [CI], 85-94), with similar response rates between CLE subtypes. Conversely, the pooled rate of thalidomide withdrawal related to adverse events was 24% (95% CI, 14-35) including confirmed peripheral neuropathy in 16% (95% CI, 9-25) and thromboembolic events in 2% (95% CI, 1-3). The pooled rate of relapse after thalidomide withdrawal was 71% (95% CI, 65-77) compared with 34% (95% CI, 25-44) with a maintenance dose. LIMITATIONS: We found important statistical heterogeneity across included studies. CONCLUSION: Considering the frequent occurrence of adverse events, prescription of thalidomide should be restricted to patients with severely refractory CLE or who are at high risk for severe scarring.
Asunto(s)
Inmunosupresores/uso terapéutico , Lupus Eritematoso Cutáneo/tratamiento farmacológico , Talidomida/uso terapéutico , Humanos , Inmunosupresores/efectos adversos , Enfermedades del Sistema Nervioso Periférico/inducido químicamente , Recurrencia , Talidomida/efectos adversos , Tromboembolia/inducido químicamenteAsunto(s)
Neoplasias del Ano/virología , Neoplasias Primarias Múltiples/virología , Infecciones por Papillomavirus/complicaciones , Lesiones Intraepiteliales Escamosas/virología , Neoplasias del Cuello Uterino/virología , Neoplasias Vaginales/virología , Neoplasias de la Vulva/virología , Adolescente , Adulto , Anciano , Neoplasias del Ano/patología , Femenino , Humanos , Huésped Inmunocomprometido , Persona de Mediana Edad , Neoplasias Primarias Múltiples/patología , Estudios Retrospectivos , Fumar , Lesiones Intraepiteliales Escamosas/patología , Neoplasias del Cuello Uterino/patología , Neoplasias Vaginales/patología , Neoplasias de la Vulva/patología , Adulto JovenAsunto(s)
Inmunosupresores/efectos adversos , Lupus Eritematoso Cutáneo/tratamiento farmacológico , Talidomida/efectos adversos , Tromboembolia/inducido químicamente , Tromboembolia/epidemiología , Adulto , Distribución por Edad , Anciano , Estudios de Cohortes , Relación Dosis-Respuesta a Droga , Esquema de Medicación , Femenino , Estudios de Seguimiento , Francia , Hospitales Universitarios , Humanos , Inmunosupresores/uso terapéutico , Incidencia , Lupus Eritematoso Cutáneo/diagnóstico , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Medición de Riesgo , Índice de Severidad de la Enfermedad , Distribución por Sexo , Tasa de Supervivencia , Talidomida/uso terapéutico , Tromboembolia/fisiopatologíaRESUMEN
We present a case of atopic nipple eczema leading to reactive hyperprolactinemia, by mechanical nipple stimulation. This reactive hyperprolactinemia caused an aggravation of the eczema because of the resulting galactorrhea, by local irritation and inflammation, and was complicated by a galactocele. This benign tumour was a source of concern for the patient and required several diagnostic radiographic examinations.