15-01-2024 | Marcia A. Tama Sánchez, Iván U. Quiroz Mendoza, Ernesto Medina Paredes, José Enrique Paredes Romero, Cesar Vladimir Haro Baque
The C.R.E.ST. Syndrome, a rare and morbid form of Progressive Systemic Sclerosis (PSS) or scleroderma, is characterized by calcinosis, Raynaud's phenomenon, esophageal dyskinesia, sclerodactyly, and telangiectasias. This case report presents a 55-year-old woman with significant dysphagia and C.R.E.ST. syndrome, highlighting the rarity of this condition in individuals over 25 years old. Despite the typical complications of pulmonary hypertension and renal failure, the patient's evolution was satisfactory. The report includes clinical details, imaging findings, and laboratory results, emphasizing the importance of early diagnosis and management of symptoms. Treatment recommendations focus on managing symptoms, preventing infections, and controlling Raynaud's phenomenon. The use of biological therapies, such as tocilizumab and nintedanib, is discussed as promising options for treating pulmonary interstitial disease and skin involvement. The article concludes with a discussion of the diagnostic criteria and management strategies for C.R.E.ST. syndrome.The C.R.E.ST. Syndrome, a rare and morbid form of Progressive Systemic Sclerosis (PSS) or scleroderma, is characterized by calcinosis, Raynaud's phenomenon, esophageal dyskinesia, sclerodactyly, and telangiectasias. This case report presents a 55-year-old woman with significant dysphagia and C.R.E.ST. syndrome, highlighting the rarity of this condition in individuals over 25 years old. Despite the typical complications of pulmonary hypertension and renal failure, the patient's evolution was satisfactory. The report includes clinical details, imaging findings, and laboratory results, emphasizing the importance of early diagnosis and management of symptoms. Treatment recommendations focus on managing symptoms, preventing infections, and controlling Raynaud's phenomenon. The use of biological therapies, such as tocilizumab and nintedanib, is discussed as promising options for treating pulmonary interstitial disease and skin involvement. The article concludes with a discussion of the diagnostic criteria and management strategies for C.R.E.ST. syndrome.