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7 Cards in this Set
- Front
- Back
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Scleroderma definition |
- Excessive deposition of collagen and other connective tissue macromolecules in CT - Affects blood vessels, skin, GI, respiratory, renal, cardiovascular, musculoskeletal, endocrine, and genitourinary - CREST syndrome is one manifest |
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CREST definition |
- Calcinosis, raynaud's, oesophageal dysmotility, scleroderma and telangiectasia |
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Scleroderma pathophys |
Genetic predisp=>vascular inflammation, leukocyte adhesion, occlusion and hypoxia=>chronic inflammation=>B cells create autoantibodies=>myofibroblasts proliferate and fibrose CT |
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Scleroderma tx |
- Avoid accelerants of disease (radiotherapy, smoking, silica and solvent exposure, cold weather (raynaud's)) - Manage comps/sympts - IL-6 receptor antagonist tocilizumab (immune modulators) |
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Scleroderma presentation |
- f>m, 30-50 y/o - Skin tightening, loss of pigment, pruritis - Raynaud's in 95%, pitting ulcers in fingertips, Cutaneous+mucosal telangiectasias, macrovascular involvement eg. non-atherosclerotic myocardial infarction - GORD, constipation, faecal incontinence, occult blood loss=>Fe def - Restrictive lung disease - arthralgias/myalgias, carpal tunnel, muscle weakness - heart failure - vaginal fibrosis, erectile dysfunction, bladder fibrosis - blindness (retinal artery occlusion), sicca syndrome - fatigue, weight loss, loss of appetite - trigeminal neuralgia, depression/anxiety |
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Mixed connective tissue disease def |
Overlapping clinical features of SLE, scleroderma, and myositis, w distinctive antibody anti U1-ribonucleoprotein (RNP) |
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Mixed connective tissue disease s/s |
- Raynaud phenomenon - Swollen hands - Arthritis/arthralgia - Acrosclerosis - Oesophageal dysmotility - Myositis - Pulmonary HTN - High level of anti–U1-RNP antibodies - Abs against U1-70 kd small nuclear ribonucleoprotein (snRNP) |
