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34 Cards in this Set
- Front
- Back
scleroderma
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a disease manifested as inappropriate scar tissue formation (fibrosis) thorughout the entire body, including skin, arteries, kidneys, GI, lungs, and heart--significant organ dysfunction and characteristic skin changes
sclero = hard; derma = skin |
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epidemiology
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prevalence from 19/100,000 to 75/100,000
male to female from 1:7 to 1:12 |
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classification
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1. localized scleroderma
2. limited scleroderma (CREST) 3. diffuse systemic sclerosis |
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localized scleroderma
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morphea and linear scleroderma
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limted scleroderma
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C= calcinosis
R= raynaud's phenomenon E=esophageal dysmotility S= sclerodactyly T= telangectasias -skin tightening of hands, distal forearm, face, and feet |
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diffuse systemic sclerosis
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skin tightening of arm, forearm, hands, feet, and trunk
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mimic scleroderma
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Eosinophilic fascitis
Eosinophilic myalgia syndrome Scleredema skin tightening (seen in Diabetes)--differentiated by deep skin and fascia biopsy |
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pathology
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immune system dysfunction (T cells, cytokines) may either injure or stimulate the endothelium or the fibroblasts; inappropriate fibroblast activation with collagen production and deposition which disrupts normal organ architecture and function
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Clinical manifestations:
Raynaud's phenomenon |
change in color of peripheral tissues due to stress induced vasospasm from cold temps (or emotional stress)
white to blue to red white: blanching caused by vasospasm blue: cyanosis- RBC deoxygenation red: vasodilation and new oxygenated RBC's |
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Persistent or recurrent ischemia
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result in digital pitting ulcers, loss of digital pads (known as sclerodactyly), and gangrene and autoamputation; >90% of scleroderma patients have Raynaud's phenomenon
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stages of skin involvement
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earliest: edematous skin
fibrotic stage: thick hard skin atrophic: skin is tight -hypopigmentation, hyperpigmentation, subcutaneous calcinosis |
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extra-cutaneous involvement
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GI, Renal, pulmonary, cardiac, musculoskeletal
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GI
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entire tract potentially affected
-mouth: decreased oral aperature and purse string mouth -esophagus: decreased LES tone leads to reflux esophagitis and stricture formation or Barrett's esophagus (premalignant); esophageal dysmotility (decreased peristalsis) of lower 1/3 of esophagus (smooth muscle portion) |
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GI ctd
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SI: reduced peristalsis causes stasis, malabsorption, diarrhea, bacterial overgrowth, intestine dilatation
-peudo-obstruction is rare complication resulting from decreased peristalsis mimicking a recurrent intestinal obstruction |
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GI ctd
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LI: thinning of muscular walls causes wide mouth diverticulum
liver: primary biliary cirhosis is an autoimmune liver disease associtated with limited PSS |
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Renal
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60-80% evidence of kidney involvement
50% have renal dysfnction such as mild proteuria and mild elevation of Cr and/or hypertension -scleroderma renal crisis is a severe and life threatenting disease that occurs in 10-15% of patients and is manifested as accelerated hypertension and rapidly progressive renal failure |
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renal ctd
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worse prognosis if:
male, old, Cr> 3 mg/dl, new anemia, RNA |
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pulmonary
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in 70% of pts;
1. interstitial lung disease (fibrosing alveolitis or pulmonary fibrosis) - most common symptom is dyspnea 2. pulmonary vascular disease later leading to pulmonary hypertension- as many as 1/3 of patients are asymptomatic, but dyspnea is seen commonly |
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cardiac
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pericarditis and pericardial effusion in 40% of patients
-fibrosis can develop in myocardium (band necrosis), which contracts and disrupts conduction system -conduction system abnormalities cause dysrrhymias and may even cause ventricular arrhythmias and sudden death |
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musculo
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arthralgia, mild arthritis (rarely destructive), tendon friction ribs, myositis, DIP tuft absorption
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lab findings
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antinuclear antibodies; anticentromere antibodies in 30-50% of pts with limited variant
antitopoisomerase 1 Ab (SCL 70) in 30-40% of pts with diffuse disease |
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radiographic findings
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GI:abnormal esophageal motility on barium swallow; stricture on barium swallow, wide mouth diverticulum
chest: basal reticulo nodular pattern and pulmonary reticular fibrosis on chest xray; HRCT with ground glass appearance and reticular pattern--active inflammation (alveolitis) or fibrosis |
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PET results
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restrictive lung disease with reduced DLCO
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BAL
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inflammatory cells- lymphocytes early on, neutrophils when 50% of lung affected
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nail fold capillary microscopy
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loss of capillary loops with dilation of both venular and arteriolar links
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treatment and management
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don't know what cuases it, can't treat it
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treatment of raynaud's
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avoid cold, gloves; topical vasodilators (nitroglycerine), oral vasodilators (angiotensin receptor blockers and calcium channel blockers), and parenteral vasodilators: iloprost if severe; finally, surgical digital sympathectomy
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D-penicillamine
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affects both collagen biosynthesis and immune system; dose ranges from 125 mg every other day to 1500 mg/day
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minocycline
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11 pts used low dose and 4 had complete resolution
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GI treatment
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proton pump inhibitors and H2 blockers for GERD
esophageal dilattation of strictures antibiotics dysmotility treated with metoclopramide and or erythromycin |
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renal treatment
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ACE inhibitors- 60% 5 yr survival
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pulmonary treatment
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corticosteroids for interstitial lung disease; steroids have also caused renal crisis, so best when used in combo with cyclophosphamide, both IV and oral (ocassionally substitute azathioprine)
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pulmonary hypertension
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Epoprostenol (Iloprost)--prostacyclin analog delivered as continuous infusion via indwelling right atrial catheter and pump
-iloprost inhalation therapy -bosentan (tracleer)--nonselevtive endothelin receptor antagonist appears to be safe and effective; viagra?! |
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investigational
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stem cell transplant (41 pts showed improvement, but 27% mortality
solid organ transplant: lung and heart transplant if severe enough |