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Scleroderma long case summary



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  • 1. Scleroderma Long Case Eyes Anemia (chronic dz, folate and B12 def, Fe def from chr esophagitis, microangiopathic hemolytic anemia)Chronic disorder characterized by diffuse skin and internal organ fibrosis Sjogren’sWomen:men 3:1, symptoms usually appear between 20-40 Mouth3 forms: Microstomia: open <3cm max 1. limited cutaneous systemic sclerosis—aka CREST synd Chest Roman breastplate tight skin 2. diffuse cutaneous systemic sclerosis—a/w systemic involvement Heart RHF/CCF 3. morphea (localized scleroderma)—rarely progresses Pericardial rub Pulmonary HTNCREST syndrome (limited form)—better prognosis Calcinosis Lungs Fibrosis Raynaud’s phemenomen Effusion Esophageal immobility Chest infections Sclerodactyly Alveolar cell carcinoma Telangectasia Other joints Arthropathy Flexion deformities Other BP, urine dipstick for proteinuriaHistorySkin Other organs Raynaud’s (90%) Polyarthralgia Investigations Edema Proximal myopathy To confirm diagnosis Thickened stretched skin Fever To look for complications Cutaneous ulcers Dysphagia Pigmentation, depigmentation Lungs Bloods FBC Anemia (vitiligo) SOB (due to anaemia) U/E/Cr Renal failure Cardiac ANA Nearly always +veDx criteria—1 major or 2 or more minor Chest pain - pericarditis Scleroderma• Major: scleroderma affecting MCP RHF – edema antibody and MTP GI Urine dipstick Proteinuria• Minor: sclerodactyly, digital tip malabsorption urinalysis pitting or loss of subst of digital Renal Radio CXR Effusion, fibrosis, CA finger pads, bibasal pulm fibrosis Decrease urine - CRF Pericardial effusion, cardiomegaly, CCF Frothy urine - proteinuria 2D echo RHFInitial presentationInvestigations done – biopsy etc ManagementTreatment and cx treatment Supportive, symptomaticCx disease Pt education Scleroderma Cytotoxics itself Early stage: cyclophosphamide, MTXPhysical Exam late stage: penicillamineGeneral Cachexia Raynaud’s Vasodilators – Ca++ blockers Bird like facies Esophageal AntacidsHands Raynaud’s symptoms PPIs Calcinosis, ulcers HTN Antihypertensives Telangiectasia Arthropathy Steroids have NO role in scleroderma Digitally signed by DR WANA HLA Contractures SHWEArms/Skin Thick tethered skin DN: cn=DR WANA HLA SHWE, Pigmentation c=MY, o=UCSI University, School of Medicine, KT-Campus, Terengganu, Vitiligo ou=Internal Medicine Group, Proximal myopathy Reason: This document is for UCSIHead Alopecia year 4 students. Date: 2009.02.22 15:19:28 +0800