Examinando por Autor "Brito Sánchez, María Gabriela"
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- ÍtemSolo MetadatosDermatomiositis clínicamente amiopática, con expresión de auto anticuerpos anti-MDA5, enfermedad excepcional. Reporte de caso clínico en Ecuador(Universidad Católica de Cuenca., 2023) Brito Sánchez, María Gabriela; Salgado Ordóñez, Octavio José; 0105683361Dermatomyositis (DM) is a rare, idiopathic, autoimmune inflammatory myopathy presenting with proximal muscle weakness, myalgias and typical skin manifestations such as erythema heliotrope and Gottron's papules, as well as cardiomyopathies and interstitial lung disease. The subtype of autoantibodies associated with DM conditions clinical and prognostic differences, DM with anti-MDA 5 antibody expression, is characterized by the absence of muscle involvement, but is associated with rapidly progressive interstitial lung disease (PPID); therefore, early mortality and poor prognosis. Prevalence depends on the region: in Asia 15-36%, and in Europe 1.3-10. No existence of data reported for Ecuador or Latin America. Case report: Female patient, 31 years old, started with joint pain in her hands, wrists and knees. After preliminary studies, a diagnosis of rheumatoid arthritis was made, receiving Methylprednisolone 10 mg and Methotrexate 10 mg weekly. Then she presented fever (40 °C), chills, dark spots on the right lower limb, persistent joint pain and malar rush; samples were taken for rheumatologic profile and antinuclear antibodies, the results of which were inconclusive. Subsequently, heliotrope erythema, shawl sign, erythema of the feet, generalized pruritus, persistent arthralgia, periungual inflammation of the hands, and synovitis in the proximal interphalangeal joints of both hands were added to the picture. Clinical laboratory tests: elevated SGOT/AST [2N], elevated SGPT/ALT [2N]; creatine phosphokinase (CPK), antinuclear antibodies (ANA) and other normal paraclinical tests. Myositis panel with positive Mi-2 beta and anti-MDA5 antibodies; normal electromyography, chest CT: infiltrate with ground glass pattern in lower lobes, a biopsy of skin lesions with findings of dermatomyositis, spirometry FVC of 58%, FEV1 of 55% and DLCO of 15.9. It was decided to administer Methylprednisolone at high doses plus biological therapy with Rituximab (4 doses) and Prednisone for maintenance, with successful picture resolution and periodic follow-up by specialty.