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A 62-Year-Old Woman with Scleroderma and Severe Weight Loss

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Abstract

Significant weight loss in systemic sclerosis (SSc) is an ominous sign. Low body mass index (BMI) is one of the few variables able to predict mortality in SSc. Weight loss usually implies severe underlying intestinal disease. Gastrointestinal (GI) tract involvement is frequent in SSc and can be the presenting symptom. It is possibly the second most common site of organ damage after the skin and is a major cause of morbidity. Severe GI disease is found in 8% of patients and carries a high mortality rate. The pathophysiology of intestinal involvement includes vascular and neurologic abnormalities with subsequent smooth muscle atrophy and fibrosis resulting in dilatation of the intestine and altered peristaltic activity. Subsequent problems include pseudo-obstruction, pneumatosis cystoides intestinalis, small intestine bacterial overgrowth (SIBO), malabsorption, and ultimately malnutrition. The panel of diagnostic tools for small bowel disease includes standard and barium radiographic studies, breath tests for small intestinal bacterial overgrowth, and various tests for malabsorption. Unfortunately, there is no cure and treatment is symptom driven. Therapy may, however, improve the patient’s symptoms and quality of life. Team work between rheumatologist, nutritionist, and gastroenterologist is essential for this very complicated problem.

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Gyger, G., Baron, M. (2011). A 62-Year-Old Woman with Scleroderma and Severe Weight Loss. In: Silver, R., Denton, C. (eds) Case Studies in Systemic Sclerosis. Springer, London. https://doi.org/10.1007/978-0-85729-641-2_18

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