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home - Miscellaneous - Rheumatological Disease - Systemic Sclerosis Written by Dr Sebastian Zeki

Systemic Sclerosis

Pancreatic DiseaseIf have steatorrhoea and no response to bacterial overgrowth treatment, treat for pancreatic insufficiency. Usually related to management of PBC (atonic/ volvulus/ fibrosed internal anal sphncter/ pneumatosis) —Faecal incontinence usually related to diarrhoea ConstipationProkinetic agents usually unsuccessfulSurgery useful if localized to recto-sigmoid junction Colonic and anorectal disease Malabsorption (due to bacterial overgrowth)Rotating antibiotics Dysmotility:Limited success with promotility agentsPseudo-obstruction and small bowel failure:1. May need TPN2. Avoid surgery due to adhesions and diffuse nature of SSc3. When in remission, restrict to a lactose-free, low fiber diet.4. Fat should also be replaced as much as possible with medium chain triglycerides.Pneumatosis cystoides intestinalis and pneumoperito-neum1. Treat conservatively2. Reduction of substrates such as lactose, sorbitol, fructose and fiber will promote cyst resolution.3. If symptomatic, O2 therapy plus antibiotics to reduces the number of gas forming bacteria. Small Intestinal Disease Delayed gastric emptying (gastric atony/ hypomotility/ megagastria)- Treat with metaclopramide (disappointing results).Patients should omit solid food.Telangiectasia is best treated with APC. (90% involved on histology)GORDTreat with PPIs (can reverse oesophageal fibrosis)Surgery C/I as hypomotility can cause severe dysphagiaHypomotility (lower 2/3rds low amplitude contractions)Can try erythromycin/ metaclopramide.StrictureCan be dilated at endoscopyFungal infection 90 % of systemic sclerosis have GI involvement. Significant in 50 %. Severe in 10%Usually starts with oesophagus70 % death within 3 years of the onset of malabsorption or recurrent pseudoobstruction,/ TPN needed Treatment of gastrointestinal disease in sys-temic sclerosis (scleroderma) Oropharyngeal disease—Conservative management is indicated eg: facial exercises/ dental hygiene/artificial saliva Biliary Tree Disease Oesophageal Disease Written by Dr Sebastian Zeki

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