RESUMO
BACKGROUND: Dermatomyositis (DM) is an autoimmune connective tissue disease that primarily targets the muscle, skin, and lungs. Many patients have autoantibodies that correspond to distinct clinical phenotypes. Melanoma differentiation-associated gene 5 (anti-MDA5) antibody, a specific antibody that targets the melanoma differentiation-associated gene 5 (MDA5), has been reported in DM cases and is significant for a distinct cutaneous presentation and rapidly progressive interstitial lung disease. OBJECTIVE: Herein, we describe a patient with DM with a positive anti-MDA5 antibody and characteristic clinical phenotype, who subsequently developed coexisting systemic lupus erythematosus (SLE). A diagnosis of SLE was supported by his clinical phenotype, positive serologies, hypocomplementemia, and progression to glomerulonephritis and lupus cerebritis, features of which fulfilled the American College of Rheumatology criteria for SLE. CONCLUSION: DM is known to overlap with other autoimmune diseases, including SLE, and coexistence can lead to a wide variety of clinical presentations. SLE overlapping with anti-MDA5 positive DM may present with distinct clinical features.
RESUMO
Se presentan 20 pacientes con cuadro clínico menor de 72 horas de poliradiculoneuritis aguda a quienes se realizaron estudios de electrofisiología, perfil inmune y biopsia de nervio sural con microscopía electrónica e inmunofluorescencia directa y diagnóstico definitivo de poliradiculoneuritis inflamatoria desmielinizante aguda (PIDA) o síndrome de Guillain-Barré-Strohl (SGBS). Diez de éstos fueron tratados con megadosis de metilprednisolona (1.5 gramos en 24 horas por 5 días) por vía intravenosa y los restantes únicamente recibieron medidas de apoyo. No se observó diferencia significativa de rehabilitación fue mejor en el grupo que recibió esteroides con el mismo período de seguimiento
