RESUMO
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.
Assuntos
Dermatomiosite , Exantema , Doenças Pulmonares Intersticiais , Miosite , Neoplasias , Humanos , Feminino , Masculino , Autoanticorpos , Dermatomiosite/complicações , Miosite/diagnóstico , Exantema/epidemiologia , Neoplasias/epidemiologia , Neoplasias/complicações , Enzimas Ativadoras de Ubiquitina , Doenças Pulmonares Intersticiais/epidemiologia , Doenças Pulmonares Intersticiais/complicações , Dispneia , Estudos Observacionais como AssuntoRESUMO
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.
Assuntos
Neoplasias do Ânus/virologia , Neoplasias Primárias Múltiplas/virologia , Infecções por Papillomavirus/complicações , Lesões Intraepiteliais Escamosas/virologia , Neoplasias do Colo do Útero/virologia , Neoplasias Vaginais/virologia , Neoplasias Vulvares/virologia , Adolescente , Adulto , Idoso , Neoplasias do Ânus/patologia , Feminino , Humanos , Hospedeiro Imunocomprometido , Pessoa de Meia-Idade , Neoplasias Primárias Múltiplas/patologia , Estudos Retrospectivos , Fumar , Lesões Intraepiteliais Escamosas/patologia , Neoplasias do Colo do Útero/patologia , Neoplasias Vaginais/patologia , Neoplasias Vulvares/patologia , Adulto JovemAssuntos
Imunossupressores/efeitos adversos , Lúpus Eritematoso Cutâneo/tratamento farmacológico , Talidomida/efeitos adversos , Tromboembolia/induzido quimicamente , Tromboembolia/epidemiologia , Adulto , Distribuição por Idade , Idoso , Estudos de Coortes , Relação Dose-Resposta a Droga , Esquema de Medicação , Feminino , Seguimentos , França , Hospitais Universitários , Humanos , Imunossupressores/uso terapêutico , Incidência , Lúpus Eritematoso Cutâneo/diagnóstico , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Distribuição por Sexo , Taxa de Sobrevida , Talidomida/uso terapêutico , Tromboembolia/fisiopatologiaRESUMO
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.
