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27 Cards in this Set
- Front
- Back
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Most important diagnostic Ab for SLE
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- Double stranded DNA
- Smith |
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SLE is example of type _ hypersensitivity
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Type III
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SLE is more common in _
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Females (80%)
Blacks, hispanics |
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MOA of SLE
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Immune complexes deposit in essentially all tissues
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Skin involvement in SLE
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- Malar "butterfly" rash
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Joint disease in SLE
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- Over 90% have polyarthralgia - inflammatory synovitis WITHOUT joint destruction
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Renal disease in SLE
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75 % have glomerulonephritis - mainly involved IgG
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4 types of SLE kidney disease
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I - mesangial lupus nephritis - mildest form, immune complexes and complement found in mesangium, excellet prognosis
Type II - Focal proliferative lupus nephritis - increased cellularity but NOT ALL glomeruli, necrosis and fibrin often found, neutrophil infiltration. Prognosis mixed - maintain or renal failure Type III - Diffuse proliferative lupus nephritis - most serious of renal disease, epithelial crescents present, many patients progress to renal failure Type IV - Membranous lupus nephritis - associated with massive proteinuria, minimal increase in cellularity |
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Serous membranes involvement in SLE
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COmmon
Pleuritis, pleural effusions |
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Respiratory disease in SLE
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- Diverse, involve upper airway, pneumonitis, progressive interstitial fibrosis, pulmonary hypertension
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CNS disease in SLE
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- Life threatening complication
- Vasculitis leads to hemorrhage infarction in brain |
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Cardiac manifestations of SLE
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- Pericarditis - most common finding
- Libman-Sacks endocarditis - sterile vegitative growths on valve leaflets (not clinically significant) |
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Drug induced Lupus
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RESEMBLES SLE
Drugs that cause - procainamide, hydralazine, isoniazid - Ranges from asymptomatic positive ANA test to SLE like clinical syndrome - No sex predominance - Most patients over 50 - NO CNS or RENAL DISEASE, Ab's to double stranded DNA is rare - Ab to histones (if ANA is positive) |
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Chronic Discoid Lupus
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- Similar skin lesions - erythema, telangiectasia, depigmented plaques - most common on face and scalp
- Difference from SLE - uninvolved skin doesnt contain immune deposits - ANA positive test NOT to double stranded DNA or Smith |
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Subacute cutaneous Lupus
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Papular and annular rings on trunk - aggravated by UV (sunlight)
- Ab to RNP |
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Sjogren syndrome is characterized by _
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- Keratoconjunctivitis
- Xerostomia |
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Clinical manifestation of Sjogren syndrome
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- Intense lymphocytic infiltrates in lacrimal and salivary glands (CD4 T cells )
- Late stage of disease - gland atrophy and fibrosis - Extraglandular involvement - bronchial gland atrophy, focal atelectasis and bronchiectasis, GI tract (dysphagia), primary billiary atresia |
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Scleroderma is characterized by
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- Vasculopathy and excessive collagen deposition in SKIN + INTERNAL ORGANS (lungs, GI, heart, kidney)
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Patients with scleroderma have 40x increased risk of _
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LYMPHOMA
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Ab specific for scleroderma
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- Nucleolar Ab to RNA polymerase
- Ab to Scl-70 - most common - Anticentromere Ab - CREST |
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Primary lesion in lungs in people with scleroderma
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- Diffuse interstitial fibrosis that can progress to end stage pulmonary fibrosis (honeycomb lung)
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2 types of scleroderma
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- Generalized progressive systemic
- Diffuse cutaneous - CREST |
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Raynaud phenomenon
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- Intermittant episodes of ischemia in the fingers
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CREST stands for_
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Calcinosis
Raynaud phenomenon Esophageal dysmotility Sclerodactyly Telangiectasia |
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In polymyositis mechanism of injury is _
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Muscle injury associated with cytotoxic CD8 cells surrounding muscle fibers which express MHCI antigens, macrophages activated by cytokine production
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In dermatomyositis mechanism of injury is _
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CD4 cells can be present BUT MAIN MECHANISM is humoral Ab production
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Mixed connective tissue disease shows _
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Combined features of SLE and Scleroderma
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