Advanced Diffuse Systemic Sclerosis With Progressive Esophageal Dysfunction: Clinical Challenges in a Public Hospital

晚期弥漫性系统性硬化症伴进行性食管功能障碍:公立医院的临床挑战

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Abstract

Diffuse systemic sclerosis (SS) is a rare autoimmune connective tissue disorder characterized by widespread fibrosis, vasculopathy, and autoantibody production. Gastrointestinal involvement, particularly esophageal dysfunction, is among the most frequent and morbid manifestations. Diagnosing and managing these complications can be especially challenging in advanced disease stages and in resource-limited settings, where specialized investigations and treatments may be inaccessible. We report a 34-year-old woman with diffuse SS who developed rapidly progressive oral intolerance, severe malnutrition, and superinfected cutaneous ulcerations. Barium esophagography demonstrated markedly delayed esophageal clearance, consistent with advanced esophageal dysmotility. Esophageal manometry and endoscopy could not be performed due to patient intolerance and restricted oral opening. Despite aggressive management including cyclophosphamide, targeted antibiotic therapy, and supportive care, the patient's condition deteriorated, culminating in multi-organ failure secondary to septic shock. This case illustrates the aggressive and life-threatening nature of gastrointestinal involvement in diffuse SS. It underscores the diagnostic and therapeutic challenges faced in resource-constrained environments and highlights the critical importance of early recognition, vigilant supportive management, and adaptation of evidence-based recommendations to local healthcare realities.

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