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53 Cards in this Set

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Two basic principle of autoimmune disorders.

(1) Characterized by immune-mediated damage of tissues.
(2) Involves loss of self-tolerance
What is the prevalence of autoimmune disorders?
1% in the USA. More common in women; classically affects women of childbearing age.
What is the etiology of autoimmune disorders?
Etiology is likely an environmental trigger in genetically susceptible individuals (increased incidence in twins and associated with certain HLA subtypes).
What mediates the damage in SLE?
Autoantibodies against the host damage multiple tissues via type II HST (cytotoxic) and type III (Ag-Ab complexes) HST.
SLE is more common in ________ (men/women), especially ___________.
women; african-american women.
What are clinical features of SLE?
• Fever, weight loss (chronic inflammation)
• Malar 'butterfly' rash, especially upon sun exposure
• Arthritis
• Serositis (pleura, pericardium)
• Inflammation of brain; CNS psychosis
The most common cause of death in lupus patients is _________. Most commonly due to what pattern of injury? Aside from this, a common cause of death is ________.
renal damage; diffuse proliferative glomerulonephritis; infection.
True or false: SLE can involve the heart.
True. All three layers. Can produce a characteristic Libman-Sacks endocarditi. One of the key findings is the presence of vegetations on BOTH sides of the heart valve involved.
True or false: In SLE there may be autoantibodies to formed blood elements.
True. There may be formed antibodies against RBCs, PLTs, WBCs.
This autoantibody is seen in CREST syndrome.
C is for Calcinosis and anti-Centromere antibody
This autoantibody is seen in celiac disease.
(1) Antiendomysial IgA
(2) Antigliadin IgA
(3) Anti-tissue transglutaminase IgA
This autoantibody is seen in drug-induced lupus.
Anti-histone
This antibody is seen in DM type I.
Anti-insulin
This autoantibody is seen in Hashimoto's.
Antimicrosomal antibody
Antithyroglobulin
This autoantibody is seen in SLE.
(1) Anti-Smith (Sm)
(2) ANA
(3) Anti-dsDNA
This autoantibody is seen in primary biliary cirrhosis.
Antimitochondrial antibody
This autoantibody is seen in Sjögrens.
Anti-SS-A (Ro)
Anti-SS-B (La)
This antibody is seen in microscopic polyangiitis.
Antimyeloperoxidase antibody.
This autoantibody is seen in mixed connective tissue disease.
Anti-ribonucleoprotein.
This autoantibody is seen in Wegener's granulomatosis.
(1) C-ANCA
(2) Antiproteinase 3 antibody
This autoantibody is seen in systemic sclerosis.
(1) Anti-topoisomerase
(2) ANA
This autoantibody is seen in dermatomyositis.
ANA
Anti-dsDNA is highly ______ (sensitive/specific) for SLE.
specific
ANA is ________ (sensitive/specific) for SLE.
sensitive
These drugs are common causes of drug-induced SLE.
(1) Hydralazine
(2) Procainamide
(3) Isoniazid
What is the treatment of drug-induced SLE?
Remove drug. It usually results in remission.
This syndrome is associated with SLE in _____% of cases.
antiphospholipid syndrome; 30%
Antiphospholipid syndrome is associated with what?
Autoantibody against proteins bound to phospholipids.
In antiphospholipid syndrome the MC antibodies are [...]
Anticardiolipin and lupus anticoagulant

The presence of autoantibodies in antiphospholipid syndrome could interfere with what?

Lead to false-positive syphilis test and falsely-elevated PTT lab studies, respectively
Why is lupus anticoagulant called an anticoagulant.
It produces a falsely elevated PTT.
Lupus anticoagulant results in _______________. Treatment is?
Results in arterial and venous thrombosis including deep venous thrombosis, hepatic vein thrombosis, placental thrombosis (recurrent pregnancy loss), and
stroke. Lifelong anticoagulation.
What is Sjögren syndrome? What type of damage is it?
Autoimmune destruction of lacrimal and salivary glands. Lymphocyte-mediated damage (type IV hypersensitivity) with fibrosis.
Sjögren clinically presents as?
Classically presents as dry eyes (keratoconjunctivitis), dry mouth (xerostomia), and recurrent dental carries in an older woman (50-60 years)—"Can't chew a cracker, dirt in my eyes".
A woman has bilaterally enlarged parotid glands with dry eyes. Treatment is begun and parotid glands shrink back to normal. A few years later, the left parotid gland has again swelled up. What is going on?
Sjögrens syndrome has an increased risk for B-cell (marginal zone) lymphoma.
Autoimmune disorders are often associated with _________________.
other autoimmune disorders
A woman with dry eyes and mouth develops nodules on her forearm close to her elbow. What is going on?
Sjögren is associated with other autoimmune diseases, especially Rheumatoid arthritis.
Another word for scleroderma is ___________.
systemic sclerosis
What is scleroderma?
Autoimmune tissue damage with activation of fibroblasts and deposition of collagen (fibrosis) . Primarily targets the skin (scleroderma), gastrointestinal tract, lungs, and kidneys.
A woman has the following sign. This is the MC initial sign of what?
A woman has the following sign. This is the MC initial sign of what?
Raynaud's phenomenon, MC initial sign of systemic sclerosis.

Sequential color changes (white to blue to red) caused by digital vessel vasculitis and fibrosis
Describe the two types of systemic sclerosis.
(1) Diffuse type (skin and early visceral involvement). Almost any organ can be involved; esophagus is commonly affected, resulting in disordered motility (dysphagia for solids and liquids).
(2) Localized type (local skin and late visceral involvement). Prototype is CREST syndrome: Calcinosis/anti-Centromere antibodies, Raynaud
phenomenon. Esophageal dysmotility, Sclerodactyly, and Telangiectasias of the skin.
What is MCTD?
Mixed connective tissue disease.

Autoimmune-mediated tissue damage with mixed features of SLE, systemic sclerosis, and polymyositis. Characterized by serum antibodies against U1 ribonucleoprotein
In systemic sclerosis, the MCC of death is ____________.
respiratory failure
What is your first clinical suspicion?
What is your first clinical suspicion?
The MC cardiac finding in SLE is ___________________.
fibrinous pericarditis with effusion
A woman with SLE delivers a newborn. What complications in the newborn could you expect to see?
Complete heart block in newborns
• Caused by IgG anti-SS-A (Ro) antiboilies crossing the placenta
A woman with SLE has recurrent spontaneous abortions. The doctor states that this is unrelated to her condition. Is he right?
He is wrong. Recurrent spontaneous abortions can be caused by antiphospholipid antibodies.
Would serum complement be high or low in SLE?
Low due to complement activation due to immune complexes.
In the pathogenesis of SLE, genetic links appear to be located on chromosome __.
6
Provide five examples of environmental factors that may be important in exacerbating or triggering SLE.
(1) Infections (virus/bacteria)
(2) UV
(3) Estrogen
(4) Medications
(5) Extreme stress

They destroy cells, leading to Abs directed against various nuclear antigens.
A serum __________ is the most useful screening test for autoimmune diseases.
ANA

This type of transplant has the best allograft survival rate. It is associated with what?

cornea; danger of transmission of Creutzfeldt-Jakob
The fetus is an ___________ that is not rejected by the mother. What tissue may prevent maternal T cells from entering the fetus?
allograft

trophoblastic tissue