Autoimmunity Flashcards
1
Q
APECED
A
- Autoimmune Poly Endocrinopathy Candidiasis Ectodermal Dystrophy
- single gene defect, lacking AIRE gene
- hypothyroidism, hypoparathyroidism
- enamel hypoplasia, punctate nail dystrophy
- vitilago, alopecia, pernicious anemia
2
Q
Which drugs can trigger SLE?
A
- Procainamide
- Hydralazine
- D-penicillamine
- Isoniazid
- Minocycline
- Phenytoin
- Ethosuximide
3
Q
Lupus Diagnosis criteria
A
- Butterfly rash
- Discoid rash
- Photosensitivity
- Oral or nasal ulcers
- Arthritis
- Serositis
- Kidney disease
- Brain abnormalities (seizures or psychosis)
- Low Blood cell counts
- Antinuclear antibodies
- Specific autoantibodies (anti-DNA, anti-sm, anti phospholipid)
4
Q
Why are complement levels important in SLE?
A
- decrease in SLE
- useful in assessing disease activity
- genetic deficiencies of complement C1, C2, and C4 are associated with SLE
5
Q
Immunology features of Lupus
A
- circulating “anticoagulants”
- cardiolipin antibodies
- false positive VDRL and thrombocytopenia
- pancytopenia
6
Q
Diagnosis of Lupus
A
- diagnosis with 4 or more of criteria and positive ANA
- patients with depressed complement, dsDNA, and 2 criteria likely have SLE
7
Q
Lupus treatment
A
- NSAIDs for fever, arthralgia, or arthritis
- anti malarial for skin and arthritis
- corticosteroids in high doses for life threatening SLE
- cytotoxic drugs can be helpful in severe lupus
8
Q
What are the two types of scleroderma
A
- Diffuse cutaneous scleroderma
- limited cutaneous scleroderma
9
Q
What are the symptoms of limited cutaneous scleroderma?
A
- CREST
- calcinosis
- Raynaud’s
- esophageal dysmotility
- sclerodactyly
- telangiectasias
10
Q
Scleroderma Evaluation
A
- ESR
- CXR
- Barium swallow
- ANA
- ECG
- UA
- skin biopsy
11
Q
Treatment for Scleroderma
A
- endothelin antagonist for pulmonary hypertension
- corticosteroids for inflammation
- CCB for raynauld’s
- H2 antagonist for GERD
- ACE inhibitor for renal involvement
- cyclophosphamide for interstitial lung disease
