vasculitis & scleroderma

Question 1

what are vasculitides (aka vasculitis)?

Answer 1

inflammation of blood vessels

Question 2

what two things can cause vasculititis?

Answer 2

1. primary autoimmune

2. secondary to drugs or toxins

Question 3

vasculitis: immune complexes lead to what?

Answer 3

deposition in the blood vessels, and secondary blockage, which leads to ischemia

Question 4

how are vasculitis types classified?

Answer 4

by size of the blood vessels affected

Question 5

3 findings that suggest vasculitis

Answer 5

1. Palpable purpura-purple skin- blood clots under skin (small vessel)
2. Hemoptysis- coughing up blood (pulmonary hemorrhage)
3. Glomerulonephritis- immune complexes circulating end up in kidney- inflamm of glomneph.(immune complexes)

Question 6

txt for vasculitis?

Answer 6

immune suppression

Question 7

what is “giant cell arteritis”?

Answer 7

AKA: Temporal arteritis and polymyalgia rheumatica

• TA in cerebral arteries
• polymyalgia rheumatica in axial musculature

Question 8

what symptoms do temporal arteritis and polymyalgia rhematica cause?

Answer 8

TA: Headache (unilateral) and blindness, tender temporal artery
PR: proximal muscle pain/stiffness/perceived weakness

Question 9

Dx giant cell arteritis?

Answer 9

Dx: clinically + Elevated inflammatory markers (ESR, CRP)
maybe Bx (show "multinucealted giant cell)

Question 10

what is Takayasu’s arteritis?

Answer 10

Chronic systemic granulomatous vasculitis
Large vessels, primarily aorta and branches of aorta
“Pulseless” disease, or “aortitis”- causes occlusion of major arteries

Question 11

what is Wegener’s granulomatosis? (what organs does it effect)?

Answer 11

granulomatosis with polyangiitis

• Small to medium vessels, broad distribution
• Granulomatous destruction of tissues, especially in lungs and kidneys

Question 12

tissue Bx of BOTH wegener’s and churg strauss show what?

Answer 12

ANCA positive
 (anti-neutrophil cytoplasmic autoantibodies)

Question 13

what is churg- strauss syndrome?

Answer 13

Eosinophilia/allergic characteristics, asthma association

-multiple organs effected

Question 14

txt for BOTH Wegener’s granulomatosis & Churg- Strauss syndrome?

Answer 14

steroids

Question 15

what is Polyarteritis nodosa?

Answer 15

Medium vessel vasculitis, causing necrotic lesions and painful nodules, organ infarcts
-Affects many organs

Question 16

what two diseases are associated with polyarteritis nodosa? (maybe weeds cause we dont know why)

Answer 16

Hep B and C

Question 17

TXT for MOST vasculitis types? short term vs long term

Answer 17

steroids (short term)

MTX or other cytotoxic txt (long term)

Question 18

hallmark of “small cell vasculitis” ? what are the two types?

Answer 18

palpable purpura

• henoch-schonlein
• leukoclastic

Question 19

Henoch-Schönlein purpura: who gets it? what does it cause?

Answer 19

Usually pediatric

IgA deposition in kidneys, leading to glomerulonephritis

Question 20

txt for Henoch-Schönlein purpura

Answer 20

Supportive Tx with antiinflammatory drugs

Question 21

Leukoclastic vasculitis: what causes it?

Answer 21

Often drug-induced

Question 22

txt for leukoclastic vasculitis?

Answer 22

Withdraw inciting drug, steroids

Question 23

in general, vasculitis Dx often depends on what two things?

Answer 23

bx or angiography

maybe…Serology for autoantibodies (ANCA in several vasculitides)

Question 24

what is anti-phospholipid antibody syndrome? what two things does it cause?

Answer 24

Autoimmune antibodies against phospholipids

• -> occluded blood vessels.
• thromobotic events + miscarriages

Question 25

anti-phospholipid antibody syndrome often appears with what other disease?

Answer 25

SLE

Question 26

what is scleroderma?

Answer 26

Sclerosis and calcification of subcutaneous tissues due to abnormal fibroblast activity Dermal thickening and fibrotic replacement of hair follicles, sweat glands, fat, etc.

Question 27

what causes these to things... - Skin of extremities often appears as if dipped in wax - Contractures of extremities

Answer 27

scleroderma

Question 28

scleroderma can extend internally to where?

Answer 28

internally to esophagus | REALLY bad reflux

Question 29

scleroderma vascular effects

Answer 29

Damage to vascular endothelium leading to intimal layer thickening and occlusion, but without inflammation

Question 30

what three things can result from the scleroderma vascular effects (thickening + occlusion)

Answer 30

- high prevalence of Raynaud’s phenomenon in Scleroderma patients - Pulmonary hypertension can result - Renal artery involvement --> HTN

Question 31

what is the Dx... can’t move face b/c of thick facial skin - can’t show expression

Answer 31

scleroderma

Question 32

xray evidence of scleroderma (2)

Answer 32

1. soft tissue calcifications | 2. acro-osteolysis: bones being chewed up at the tips

Question 33

what is CREST syndrome?

Answer 33

``` Cutaneous calcinosis Raynaud’s phenomenon Esophageal dysmotility Sclerodactyly Telangiectasia (hands + face) ```

Question 34

3 Dx keys to scleroderma/CREST syndrome? (3)

Answer 34

1. Characteristic skin changes 2. Antinuclear antibodies in the presence of other features of scleroderma (e.g. sclerodactyly, GERD) 3. New onset HTN in scleroderma– monitor BP, suspect renal artery involvement

Question 35

txt for scleroderma/ CREST (maybe weeds) | - specific for HTN, GERD, and raynaud's

Answer 35

1. Possible early methotrexate or cyclophosphamide (for interstitial lung disease) 2. Prostacyclin analogs for pulmonary HTN 3. PPI for GERD (Lots of PPI) 4. Vasodilators for Raynaud’s (e.g. Ca++ blockers)