CT Disorders and Vasculitides Flashcards
What is used to test for autoimmune disease?
What feature of this test makes it important to gather more information about the patient?
1) ANA
2) Not specific
Systemic lupus Erythematosus has autoantibodies to what antigens?
What type of hypersensitivity is it?
1) Nuclear
2) Type III
What are notable cutaneous features of SLE?
1) Malar butterfly rash
2) Photosensitivity
What is a notable hematologic feature of SLE?
Thrombosis
What are notable cardiopulmonary features of SLE?
1) Pericarditis
2) Libman-Sacks endocarditis
3) MI
What body position does pericarditis get better/worse?
What does it look like on ECG?
What can be heard on auscultation?
1) Worse in supine, better with sitting upright
2) ST elevation
3) Pericardial friction rub
Why are SLE patients at an increased risk of MI?
Accelerated atherosclerosis
What is a notable renal feature of SLE?
Nephritis
Where does the malar butterfly rash spare on the face?
Nasolabial folds
What specific antigens do SLE patients have autoantibodies for?
Which one correlates with disease activity?
1) dsDNA (Correlates with disease activity)
2) Sm (Smith)
What complement proteins will be decreased on SLE serology because of their increased consumption with this condition?
C3 or C4
What causes Libman-Sacks endocarditis in SLE?
Immune deposits on the valves
What are the treatment/prevention options for SLE?
1) Avoid sun exposure/wear sunscreen
2) NSAIDs
3) Glucocorticosteroids
4) Hydroxychloroquine
In the early years after diagnosis what are causes of SLE mortality?
In later years?
1) Opportunistic infections, kidney, or CNS disease
2) MI or thromboembolic events
What does type one antiphospholipid antibodies cause biologic false-positive tests for?
Syphilis
What is type two antiphospholipid antibodies?
What does it cause risk factors for?
1) Lupus anticoagulant
2) Venous/arterial thrombosis and miscarriage
What is type three antiphospholipid antibodies?
The antibodies are directed towards what serum cofactor?
1) Anti-cardiolipin antibodies
2) Beta-2 glycoprotein I
What is the treatment for APS?
Anticoagulation
Both SLE/APS cause retinopathy, what diagnostic feature is seen in the eye?
Cotton wool spots
What symptoms are not seen in Lupus-like syndrome/Drug-induced?
No renal or neuro symptoms
Lupus-like syndrome/Drug-induced are positive for what antibodies?
Anti-histone Abs
What may happen to SLE patients when given Sulfa antibiotics?
Exacerbate lupus symptoms
Neonatal lupus affects children born of mothers with what Abs?
What can this condition cause?
1) Anti-Ro (SSA) or La (SSB) Abs
2) Permanent complete heart block
Discoid lupus erythematosus (DLE) is a variant of which lupus?
Where does it most commonly occur?
What is it characterized by?
1) Cutaneous lupus
2) Head
3) Inflammatory plaques that evolve into atrophy, disfiguring scars
What is the hallmark of scleroderma?
Thickening and hardening of the skin and visceral organs (fibrosis)
What causes the dry itchy skin seen in scleroderma?
Obliteration of eccrine sweat and sebaceous glands
What is usually the first symptom seen in caucasian scleroderma patients?
In African American patients?
1) Secondary Raynaud phenomenon
2) Changes in skin pigmentation
Which type of scleroderma is seen in children and causes discrete areas of discolored skin induration?
Localized scleroderma
How does raynaud’s present in localized scleroderma?
What are the patches seen on the skin called?
1) There is no Raynaud’s
2) Morphea
Which type of scleroderma is associated with the CREST syndrome?
Limited Cutaneous Systemic Sclerosis
What is the CREST syndrome?
1) Cutaneous calcinosis
2) Raynaud’s (2⁰)
3) Esophageal dysmotility
4) Sclerodactyly
5) Telangiectasia
How are the vascular manifestations more pronounced in limited cutaneous systemic sclerosis?
1) Digital ischemia
2) Pulmonary artery HTN
How does pulmonary artery HTN present in limited cutaneous systemic sclerosis?
How does esophageal dysmotility present?
1) As shortness of breath
2) GERD
Which type of scleroderma causes fibrosis, loss of body hair, and impaired sweating?
Diffuse cutaneous systemic sclerosis