vasculitis & scleroderma Flashcards
what are vasculitides (aka vasculitis)?
inflammation of blood vessels
what two things can cause vasculititis?
- primary autoimmune
2. secondary to drugs or toxins
vasculitis: immune complexes lead to what?
deposition in the blood vessels, and secondary blockage, which leads to ischemia
how are vasculitis types classified?
by size of the blood vessels affected
3 findings that suggest vasculitis
- Palpable purpura-purple skin- blood clots under skin (small vessel)
- Hemoptysis- coughing up blood (pulmonary hemorrhage)
- Glomerulonephritis- immune complexes circulating end up in kidney- inflamm of glomneph.(immune complexes)
txt for vasculitis?
immune suppression
what is “giant cell arteritis”?
AKA: Temporal arteritis and polymyalgia rheumatica
- TA in cerebral arteries
- polymyalgia rheumatica in axial musculature
what symptoms do temporal arteritis and polymyalgia rhematica cause?
TA: Headache (unilateral) and blindness, tender temporal artery
PR: proximal muscle pain/stiffness/perceived weakness
Dx giant cell arteritis?
Dx: clinically + Elevated inflammatory markers (ESR, CRP) maybe Bx (show "multinucealted giant cell)
what is Takayasu’s arteritis?
Chronic systemic granulomatous vasculitis
Large vessels, primarily aorta and branches of aorta
“Pulseless” disease, or “aortitis”- causes occlusion of major arteries
what is Wegener’s granulomatosis? (what organs does it effect)?
granulomatosis with polyangiitis
- Small to medium vessels, broad distribution
- Granulomatous destruction of tissues, especially in lungs and kidneys
tissue Bx of BOTH wegener’s and churg strauss show what?
ANCA positive (anti-neutrophil cytoplasmic autoantibodies)
what is churg- strauss syndrome?
Eosinophilia/allergic characteristics, asthma association
-multiple organs effected
txt for BOTH Wegener’s granulomatosis & Churg- Strauss syndrome?
steroids
what is Polyarteritis nodosa?
Medium vessel vasculitis, causing necrotic lesions and painful nodules, organ infarcts
-Affects many organs
what two diseases are associated with polyarteritis nodosa? (maybe weeds cause we dont know why)
Hep B and C
TXT for MOST vasculitis types? short term vs long term
steroids (short term)
MTX or other cytotoxic txt (long term)
hallmark of “small cell vasculitis” ? what are the two types?
palpable purpura
- henoch-schonlein
- leukoclastic
Henoch-Schönlein purpura: who gets it? what does it cause?
Usually pediatric
IgA deposition in kidneys, leading to glomerulonephritis
txt for Henoch-Schönlein purpura
Supportive Tx with antiinflammatory drugs
Leukoclastic vasculitis: what causes it?
Often drug-induced
txt for leukoclastic vasculitis?
Withdraw inciting drug, steroids
in general, vasculitis Dx often depends on what two things?
bx or angiography
maybe…Serology for autoantibodies (ANCA in several vasculitides)
what is anti-phospholipid antibody syndrome? what two things does it cause?
Autoimmune antibodies against phospholipids
- -> occluded blood vessels.
- thromobotic events + miscarriages
anti-phospholipid antibody syndrome often appears with what other disease?
SLE
what is scleroderma?
Sclerosis and calcification of subcutaneous tissues due to abnormal fibroblast activity
Dermal thickening and fibrotic replacement of hair follicles, sweat glands, fat, etc.
what causes these to things…
- Skin of extremities often appears as if dipped in wax
- Contractures of extremities
scleroderma
scleroderma can extend internally to where?
internally to esophagus
REALLY bad reflux
scleroderma vascular effects
Damage to vascular endothelium leading to intimal layer thickening and occlusion, but without inflammation
what three things can result from the scleroderma vascular effects (thickening + occlusion)
- high prevalence of Raynaud’s phenomenon in Scleroderma patients
- Pulmonary hypertension can result
- Renal artery involvement –> HTN
what is the Dx… can’t move face b/c of thick facial skin - can’t show expression
scleroderma
xray evidence of scleroderma (2)
- soft tissue calcifications
2. acro-osteolysis: bones being chewed up at the tips
what is CREST syndrome?
Cutaneous calcinosis Raynaud’s phenomenon Esophageal dysmotility Sclerodactyly Telangiectasia (hands + face)
3 Dx keys to scleroderma/CREST syndrome? (3)
- Characteristic skin changes
- Antinuclear antibodies in the presence of other features of scleroderma (e.g. sclerodactyly, GERD)
- New onset HTN in scleroderma– monitor BP, suspect renal artery involvement
txt for scleroderma/ CREST (maybe weeds)
- specific for HTN, GERD, and raynaud’s
- Possible early methotrexate or cyclophosphamide (for interstitial lung disease)
- Prostacyclin analogs for pulmonary HTN
- PPI for GERD (Lots of PPI)
- Vasodilators for Raynaud’s (e.g. Ca++ blockers)
