Síndrome de C.R.E.S.T. Presentación de un Caso. Descripción breve de la Patología

Síndrome de C.R.E.S.T. Presentación de un Caso. Descripción breve de la Patología

2024 | Marcia A. Tama Sánchez, Iván U. Quiroz Mendoza, Ernesto Medina Paredes, José Enrique Paredes Romero, Cesar Vladimir Haro Baque
Progressive systemic sclerosis (PSS), or scleroderma, is a rare disease characterized by skin hardening and thickening, and sometimes multi-organ involvement, especially pulmonary and renal. CREST syndrome is a limited form of PSS, characterized by calcinosis, Raynaud's phenomenon, esophageal dyskinesia, sclerodactyly, and telangiectasias. It is more common in women aged 35-50 years and is rare before the age of 25. The syndrome is associated with interstitial pulmonary fibrosis, which can lead to pulmonary hypertension and right heart failure. CREST syndrome is often preceded by Raynaud's phenomenon. The case presented is of a 55-year-old woman with CREST syndrome and significant dysphagia. Despite her condition, she did not show signs of pulmonary hypertension, cor pulmonale, or renal failure, and her condition was satisfactory. The diagnosis was confirmed through clinical findings, serological tests, and biopsy. CREST syndrome is a limited form of PSS, though not less benign. Treatment includes maintaining hydration, avoiding infections, and using anti-inflammatory drugs. Biological therapies such as tocilizumab and nintedanib are used to manage the disease. The study highlights the importance of early diagnosis and multidisciplinary management in CREST syndrome.Progressive systemic sclerosis (PSS), or scleroderma, is a rare disease characterized by skin hardening and thickening, and sometimes multi-organ involvement, especially pulmonary and renal. CREST syndrome is a limited form of PSS, characterized by calcinosis, Raynaud's phenomenon, esophageal dyskinesia, sclerodactyly, and telangiectasias. It is more common in women aged 35-50 years and is rare before the age of 25. The syndrome is associated with interstitial pulmonary fibrosis, which can lead to pulmonary hypertension and right heart failure. CREST syndrome is often preceded by Raynaud's phenomenon. The case presented is of a 55-year-old woman with CREST syndrome and significant dysphagia. Despite her condition, she did not show signs of pulmonary hypertension, cor pulmonale, or renal failure, and her condition was satisfactory. The diagnosis was confirmed through clinical findings, serological tests, and biopsy. CREST syndrome is a limited form of PSS, though not less benign. Treatment includes maintaining hydration, avoiding infections, and using anti-inflammatory drugs. Biological therapies such as tocilizumab and nintedanib are used to manage the disease. The study highlights the importance of early diagnosis and multidisciplinary management in CREST syndrome.
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