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27 Cards in this Set

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Most important diagnostic Ab for SLE
- Double stranded DNA
- Smith
SLE is example of type _ hypersensitivity
Type III
SLE is more common in _
Females (80%)
Blacks, hispanics
MOA of SLE
Immune complexes deposit in essentially all tissues
Skin involvement in SLE
- Malar "butterfly" rash
Joint disease in SLE
- Over 90% have polyarthralgia - inflammatory synovitis WITHOUT joint destruction
Renal disease in SLE
75 % have glomerulonephritis - mainly involved IgG
4 types of SLE kidney disease
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
Serous membranes involvement in SLE
COmmon
Pleuritis, pleural effusions
Respiratory disease in SLE
- Diverse, involve upper airway, pneumonitis, progressive interstitial fibrosis, pulmonary hypertension
CNS disease in SLE
- Life threatening complication
- Vasculitis leads to hemorrhage infarction in brain
Cardiac manifestations of SLE
- Pericarditis - most common finding
- Libman-Sacks endocarditis - sterile vegitative growths on valve leaflets (not clinically significant)
Drug induced Lupus
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)
Chronic Discoid Lupus
- 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
Subacute cutaneous Lupus
Papular and annular rings on trunk - aggravated by UV (sunlight)
- Ab to RNP
Sjogren syndrome is characterized by _
- Keratoconjunctivitis
- Xerostomia
Clinical manifestation of Sjogren syndrome
- 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
Scleroderma is characterized by
- Vasculopathy and excessive collagen deposition in SKIN + INTERNAL ORGANS (lungs, GI, heart, kidney)
Patients with scleroderma have 40x increased risk of _
LYMPHOMA
Ab specific for scleroderma
- Nucleolar Ab to RNA polymerase
- Ab to Scl-70 - most common
- Anticentromere Ab - CREST
Primary lesion in lungs in people with scleroderma
- Diffuse interstitial fibrosis that can progress to end stage pulmonary fibrosis (honeycomb lung)
2 types of scleroderma
- Generalized progressive systemic
- Diffuse cutaneous - CREST
Raynaud phenomenon
- Intermittant episodes of ischemia in the fingers
CREST stands for_
Calcinosis
Raynaud phenomenon
Esophageal dysmotility
Sclerodactyly
Telangiectasia
In polymyositis mechanism of injury is _
Muscle injury associated with cytotoxic CD8 cells surrounding muscle fibers which express MHCI antigens, macrophages activated by cytokine production
In dermatomyositis mechanism of injury is _
CD4 cells can be present BUT MAIN MECHANISM is humoral Ab production
Mixed connective tissue disease shows _
Combined features of SLE and Scleroderma