Term
| What are the three HLA types that are strongly correlated with autoimmune disease and what diseases? |
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Definition
-HLA-DR3; IDDM, SLE, Sjogren, Graves, Myasthenia Gravis
-HLA-DR4; IDDM, RA
-HLA-B27; Ankylosing spondylitis (almost 100% correlation) |
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Term
| What are different nuclear patterns we can see from tagging autoantibodies and what diseases show them (were relevant)? |
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Definition
-Homogenous
-Rim (peripheral); lupus
-Speckled
-Nucleolar; scleroderma (limited CREST form) |
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Term
| Who most commonly has SLE and when does it show up? |
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Definition
-9:1 ratio for women to men and is most common and severe in AA women
-Arises in 20's and 30's
-Note; SLE stands for systemic lupus erythematosus |
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Term
| What are the tests we can run for SLE? Which is/are more diagnostic? |
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Definition
-ANA (antinuclear antibody) test is the most sensitive, but not most diagnostic because of its lower specificity
-Antibodies to dsDNA and Smith (Sm) antigen tests are very specific (less sensitive), and thus are very diagnostic |
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Term
| What diagnostic test can lupus mess with and how? |
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Definition
-Can give false positive for **syphilis (using Venereal Disease Research Laboratory test or *VDRL) because anti-cardiolipin antibodies may be present
-Also, may alter PTT test (rem; intrinsic) because of *Lupus anticoagulant |
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Term
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Definition
| -It is type III, however it has some type II properties if antibodies to RBC, WBC, and platelets are made |
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Term
| Do we expect high or low compliment levels in lupus? |
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Definition
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Term
| What is the typical cause of death in lupus? Give important types and clinical signs? |
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Definition
-Renal lesions;
1.**Diffuse proliferative glomerulonephritis is the most common and most deadly type
-The cells proliferate too much and close off the capillaries, lowering flow, increasing renin, and eventually causing **malignant hypertension (very high BP)
-We often see (nephritic syndrome) hematuria and/or proteinuria (nephrotic syndrome)
2. Membranous glomerulonephritis is less severe
-Here we get a thickening of the basement membranes presenting as "wire loop" form on biopsy
-Get the same problems and signs as above, but not quite as sever (rem, high BP will give edema) |
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Term
| What are the other systemic effects of lupus? |
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Definition
-Butterfly rash that is exacerbated by sunlight (attack is at the dermoepidermal junction)
-Heart problems; pericarditis (friction rub), Libman-Sacks (vegetations on valves), coronary art. disease (from increased thrombosis - rem, type III HSD)
-Joints; **non-erosive (RA is erosive)
-Lungs; pleuritis
-Basically, all kinds of systemic involvement that can present as flare-ups spanning years, or even decades |
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Term
| What is a major way we treat lupus? |
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Definition
| -With corticosteroids during flare-ups |
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Term
| Give two variations on lupus? How do they test? |
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Definition
-Discoid Lupus Erythematosus; only get the skin rash, but no anti-dsDNA antibody or any of the other symptoms
-Drug induced lupus; get +ANA, but -dsDNA tests |
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Term
| What drugs can cause the drug induced lupus? |
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Definition
-Procainamide, hydralazine (classic ones)
-Also D-penicillamine |
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Term
| What is Sjogren syndrome? Who is it more common in? How does it present? How do we diagnose it? |
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Definition
-Autoimmune destruction of lacrimal and salivary glands
-More common in older white females
-Will have chronically dry eyes (blurred vision), trouble swallowing, frequent nose bleeds (all from dryness)
-Also get bilateral parotid inflammation
-We do a lip biopsy to diagnose |
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Term
| What are the two forms of Sjogren syndrome? Which is more common? |
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Definition
-Primary form is less common and is the isolated disorder
-Secondary form is associated with other autoimmune diseases, most often RA
-Rheumatoid factor is seen in 75% of patients
-ANA is seen in 50-80%
-Usually no glomerular lesions* |
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Term
| What is the generic name for lacrimal and salivary gland enlargement that can be applied to Sjogren syndrome? |
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Definition
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Term
| What is scleroderma? What are the two categories of it? Who do we see it more in? |
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Definition
-Characterized by the abnormal accumulation of fibrosis in skin and multiple other organs (systemic fibrosis)
-Can be either the diffuse or limited type
-3x more common in women (usually older) |
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Term
| Explain the two types of scleroderma? What are the two types of antibodies we can look for in the two types? |
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Definition
-Diffuse is fibrosis all over
-Limited is only the CREST syndrome;
Calcinosis; deposition of calcium after damage
Raynaud phenomenon; cold fingers and toes
Esophageal dismotility; disphagia
Sclerodactyly; claw hand deformity
Telangiectasia; dilated capillaries
-anti-Scl 70 in diffuse
-anti-centromeric in crest (get nucleolar pattern with fluorescence) |
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Term
| What are the systemic effects that we see in scleroderma? |
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Definition
-We get fibrosis all over the place;
-Kidney; obliterative vasculopathy (->renin->>BP)
-Lung; interstitial fibrosis
-Heart; pericarditis, myocardial fibrosis (->arrhythmia)
-Joints; arthritis
-Overall is similar to lupus, except we also get;
-Skin; tight and shiny
-Appendages; can lose fingers and get claw hand |
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Term
| What will we see in a biopsy in scleroderma and what must we differentiate it from? |
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Definition
-We see thick layers of collagen
-Similar appearance to keloid biopsy* |
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