Examinando por Autor "Quezada Riofrío, Leidy Azucena"
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- ÍtemSolo MetadatosEsclerosis sistémica sin esclerodermia. Reporte de caso.(Universidad Católica de Cuenca., 2023) Quezada Riofrío, Leidy Azucena; González Ortega, Álvaro Fernando; 1105995464Background: Systemic sclerosis is classified as a rare disease, since its pathogenesis and etiology are not fully clarified. Its cause has been attributed to genetic, environmental, and infectious factors that generate defects in humoral autoimmunity. Among its variants is systemic sclerosis without scleroderma, of which few cases of the disease have been reported, with visceral involvement. Its complications are severe in relation to the diagnosis time delay. Case description: A single forty-year-old male businessman patient presents clinical symptoms of flatulence, belching, reflux, heartburn, mild dysphagia to liquids, and severe to solids for three years. He is treated with proton pump inhibitors and does not improve; esophageal manometry is performed, which reports severe slowing of the distal third of the esophagus. Conclusions: Systemic sclerosis without scleroderma has similarities to the localized and diffuse systemic sclerosis clinical presentation, except for cutaneous involvement at the nail level. When systemic sclerosis without scleroderma is suspected, the determination of positive anticentromere antibodies is typical of the disease. It is important to have high diagnostic suspicion since it has been observed that the disease responds to treatment. Complications associated with systemic sclerosis without scleroderma will depend on the delay in diagnosis. It is important to develop diagnostic protocols for the therapeutic approach to rare diseases.