Clinical Presention And Evaluation Of Vasculitis

Question 1

Vasculitis broad details

Answer 1

Is considered a syndrome since it encompasses a wide variety of different issues and multiples organs can be affected.

• all issues deal with damage to vessels (usually arteries/capillaries)
• can occur in every organ system, but can also be limited to only one organ
• can be caused by an ischemic injury
• is rare and often presents with delayed diagnosis
• can be primary or secondary to other diseases
• widespread presentation (minimal -> life threatening)

Question 2

Polyangiitis

Answer 2

Vasculitis in small vessels

Question 3

Possible causes of large vessel vasculitis

Answer 3

Takayasu arteritis

Giant cell arteritis

Question 4

Possible causes of Medium vessel vasculitis

Answer 4

Polyarteritis Nodosa

Burgers syndrome

Kawasaki disease

Question 5

Possible secondary causes of vasculitis syndromes

Answer 5

Drug-induced

Hepatitis B/C associated

Cancer associated

SLE associated

Sarcoidosis vasculitis

RA associated

Question 6

What are the three anti neutrophilic cytoplasmic antibody (ANCA) associated vasculitis causes?

Answer 6

Microscopic polyangiitis

Granulomatosis w/ polyangiitis

Eosinophillic granulomatosis w/ polyangiitis

Question 7

Specific Clinical feature of vasculitis in post-capillary venules

Answer 7

Palpable purpura

Question 8

Specific clinical features of vasculitis in glomerular capillaries

Answer 8

Haematuria

RBCs in urine

Proteinuria

Declined renal function

Question 9

Specific clinical features of vasculitis in pulmonary capillaries

Answer 9

Lung hemorrhage w/ severe dyspnea

Widespread alveolar shadowing on chest radiography

Question 10

Specific clinical features of vasculitis in extra cranial branches of the carotid artery

Answer 10

Temporal artery = temporal severe headache

Ophthalmic artery = partial blindness

Facial artery and its branches = jaw claudication

Question 11

What type of questions to ask when faced w/ potential cases of vasculitis

Answer 11

1) is there a differential differential that could mimic vasculitis?
2) is there a secondary underlying cause
3) what is the extent of vasculitis
4) how do I confirm the diagnosis
5) what is the specific type of vasculitis

Question 12

Cryoglobulinemic vasculitis

Answer 12

Small vessel vasculitis (polyangiitis) associated w/ Hep C
- serum proteins precipitate out in the cold (cold agglutinins) usually around 36C

While most associated with Hep C, the following can also be causes:

• SLE
• RA
• HIV
• Cardiac/HTN drugs

Question 13

Wegeners syndrome (granulomatosis w/ polyangiitis)

Answer 13

Presents with

• granulomas in the lungs
• coughing up streaks of blood
• dark urine w/ blood
• creatinine increases in CMP
• splinter hemorrhages

Need cANCA and PR3 tests

Question 14

ANCA associated vasculitis w/ microscopic polyangiitis (MPA)

Answer 14

Presents with

• high blood in urine
• proteinuria
• high platelets
• high creatinine
• stupid high ESR and CRP levels
• blue extremities (Reynolds looking)
• small palpable rash (possible but not always)
• myalgia and fever

Need pANCA/anti-MPO blood tests to confirm

Question 15

Chung-Strauss syndrome

Eosinophillic granulomatosis w/ polyangitis (EGPA)

Answer 15

Presents w/
- high eosinophil levels (>10%)*
- x rays appear really spotty and patchy on the lungs
- asthma w/ no past history
- wheezing in both lungs
- muscle wasting and deformity I of specifically the left hand
- fever, malaise
- anorexia
- pain/tingling in the hands
- mononeuritis multiplex (75%) of the time
(Asymmetric peripheral neuropathy appearing idiopathic)

Requires p-ANCA blood tests and high eosinophil levels to confirm

Question 16

Most common causes of mononeuritis multiplexes

Answer 16

1) leprosy
2) Diabetes
3) Vasculitis
4) sarcoidosis
5) amyloidosis
6) cancer
7) HIV infections

Question 17

Treatment of vasculitis

Answer 17

Varies based on disease stage, but all stages requires steroids

Early systemic w/ no vital organ damage = methotrexate and steroids

Generalized vasculitis w/ vital organ damage = cyclophosphamide w/ steroids

Severe renal or pulmonary vasculitis =
Cyclophosphamide/ azathioprine and steroids w/ plasma exchange if needed

Refractory vasculitis (vital organ damage w/ no response to therapy) = Rituximab

Question 18

Buerger Disease (thromboangiitis Obliterans)

Answer 18

Presents w/

• intermittent claudication of cyanotic extremities (worsens when warming up the affected regions)
• cyanotic extremities (worsens when cold)
• necrosis may be present
• ONLY found in heavy smokers

Tx: Stop smoking
- if you dont extremities become necrotic via dry gangrene

Question 19

Giant cell (temporal) arteritis

Answer 19

Presents with

• severe temporal headaches
• mild fevers
• age >50yrs old (almost never seen in younger patients)
• proximal upper muscle weakness
• blurry vision/ very short intermittent blindness
• intermittent jaw claudication
• tenderness over temporal arteries
• stupid high CRP/ESR levels
• biopsy confirms (treat before this step though)

Note: if girdle weakness w/ flu-like syndrome consider polymyalgia rheumatica (PMR) on top of giant cell arteritis

Tx: high steroid doses (even before biopsy)
Must get this correct or the patient will either go blind permanently or die

Question 20

Takayasu arteritis and Kawasaki disease

Answer 20

Presents w/:

• fever, malaise and weight loss
• massive HTN
• high proteinuria and hemouria
• elevated ESR and CRP rates

Takayasu will usually present w/ pulseless or super weakened pulses

Kawasaki is usually young patients

Question 21

IgA vasculitis (Henoch-Schonlein)

Answer 21

Presents only in children and has the following

• very palpable purpura in the extremities
• arthralgia
• GI and kidney arthralgia
• often follows an infections

Question 22

Possible causes for small cell vasculitis

Answer 22

Microscopic polyangitis (MPA)

Granulomatosis polyangitis (GPA)
- also know as Wegners 

Cryoglobinemic vasculitis

Churg Strauss vasculitis

Henloch-schonlein purpura