VASCULITIS

Question 1

Define vasculitis

Answer 1

Systemic disease, characterised by inflammation within the blood vessels, disrupting the internal elastic lamina.

• May involve one or many organs
• May involve vessels of one size or many vessel types

Question 2

Clinical features of systemic vasculitis

Answer 2

–Ischaemia to tissues supplied by damaged vessel
–Features of widespread inflammation
–Vessel rupture is extremely rare

Question 3

Classify vasculitis

Answer 3

•Primary – multisystem diseases of unknown aetiology
•Secondary – vessel inflammation due to
–Infection ; SBE, cystic fibrosis
–Drugs
–Malignancy
-radiation
-cryoglobulinaemia
-connective tissue disease

Question 4

Name types of primary vasculitis showing granulomata and no granulomata

Answer 4

Large vessel - Temporal Arteritis (Granulomata)

Medium vessel

• Church Strauss -(EPGA) - granulomata
• Polyarteritis Nodosa and Kawasaki syndrome - no granulomata

Small vessel

• Wegener’s granulomatosus (GPA) - granulomata
• Microscopic polyangiitis and Henoch Schonlein Purpura- no granulomata

Question 5

Mortality and morbidity a/w primary vasculitis due to ..?

Answer 5

Morbidity & mortality due to
–Organ failure – renal, pulmonary, neurological
–Effects of Immunosuppression

Question 6

Dx of primary vasculitis

Answer 6

•Serology-ANCA; anti PR3 , anti MPO
•Biopsy-Affected organ
Occasionally blind
•Angiography-Large vessel disease

Question 7

Define GPA

Answer 7

• Multisystem disease of unknown aetiology affecting small blood vessels
• Clinicopathological syndrome
• Most patients with active systemic disease are ANCA positive
• Relapsing, remitting course

Question 8

Clinical and lab features a/w GPA

Answer 8

Clinical Features
•Upper respiratory tract
•Lower respiratory tract
•Glomerulonephritis
•Any Organ

Lab 
•Granulomata
•Vasculitis
•Necrosis
•ANCA positive

Question 9

Explain ANCA dx in vasculitis

Answer 9

•Less specific than originally hoped
•50-70% ANCA positive subjects have vasculitis
False positives in
•Infection
•Inflammatory diseases
•Other, including lymphoma

Question 10

Describe Bacterial Endocarditis

Answer 10

• infection
  -acute/subacute
  •Immunological stimulation leads to additional tissue destruction
  •Immune complex mediated tissue damage

Question 11

Clinical features of subacute bacterial endocarditis

Answer 11

Symptoms & signs are due to
•Damage to valves
•Immune complex deposition
•Systemic illness associated with widespread inflammation

Question 12

Dx of SBE

Answer 12

• Clinical Suspicion – pyrexia unknown origin
• Blood cultures
• ECHO – vegetations
• Acute phase response

Question 13

Tx for SBE

Answer 13

Antibiotics –
•treating infection removes antigen
•Immune complexes no longer formed
•Very rarely need steroids for immune complex disease.

Question 14

Describe cryoglobulinaemia

Answer 14

•Cryoglobulins are immunoglobulins which precipitate in the cold
•Clinical manifestations are due to
Raynaud’s phenomenon
Vasculitis

Question 15

clinical features a/w cryoglobulinaemia

Answer 15

palpable purpura- skin 
Raynauds- loss of coloration in fingers 
joints (eg painful mononeuritis)  
-Renal manifestation 
-Neuropathy manifestation 
-GI involvement

Question 16

What is the hallmark for cryoglobinaemia

hint* triad

Answer 16

Meltzer’s triad

Purpura
Arthralgia
Weakness

Question 17

Dx of cryo

Answer 17

Serum taken and kept at 37’C until separated in lab.
•1 ml serum drawn into 1 ml syringe
•Keep at 4’C for 72 – 168 hours.
•If precipitate there – estimate percentage.
•Check that cryo goes back in solution when rewarmed to 37’C.
•When positive, Type & assess for causes.

Question 18

What can occur in testing for cryo

Answer 18

False -ve results due to
•The syringe is not warmed to 37 degrees C.
•The sample is not kept at 37 degrees C until clotting is completed
•The sample is centrifuged at temperatures below 37 degrees C
•The sample is not stored at 4 degrees C for 72 hours

Question 19

List the types of cryoglobins

Answer 19

• Type I – monoclonal cryoglobulin
• Type II – Mixed Cryoglobulin
• Monoclonal component & polyclonal Igs
• Type III – Polyclonal Crypglobulin
• Immune complexes

Question 20

What test can be done to determine cryoglobulin type?

Answer 20

Plasma protein electrophresis followed by immunofixation

Question 21

What disease is each type of cryoglobulin a/w?

Answer 21

Type I – monoclonal cryoglobulin
Myeloma,
Waldenstrom’s Macroglobulinaemia,
B cell leukaemias/lymphomas

•Type II – Mixed Cryoglobulin
•Monoclonal component & polyclonal Igs
Infection (Hep C)
Connective Tissue Disease
B cell Lymphoproliferative disease
Mixed essential cryoglobulinaemia

Type 3 - polyclonal - immune complexes
Connective tissue disease;
Chronic infection

Question 22

Tx for cryoglobulinaemia

Answer 22

• Treat underlying cause
• Steroids +/- immunosuppression
• Plasmapheresis if life or organ threatening

Question 23

How to distinguish CTDs from vasculitis

Answer 23

Ask the following questions

1. Could this be CTDs/Vasculitis?
2. What organs may be involved
3. Does it fit a classical pattern
4. Could this be a secondary vasculitis?
5. Is serology helpful?
6. What site for biopsy/angio?

Then do diagnostic tests 
•RF
•ANF, anti-dsDNA, Anti-ENA
•ANCA – anti-PR3 and anti-MPO
•Anti-cardiolipin, anti-b2glycoprotein 1, lupus anticoagulant
•Complement, Cryoglobulins
•Biopsy
•Angiography

Assess disease extent 
•Clinical Evaluation
•FBC & antibodies if cytopenias
•X-ray joints – involved & typical
•CXR/PFTs/ High Res CT
•U&E/ Urinalysis & micro/ GFR/ 24 hour prot
•CK/EMG/muscle biopsy
•Cardiac – ECG/ECHO/Holter/ enzymes
•Neurological – Imaging & Neurophysiology

Assess disease activity

• Inflammation
• Organ Function
• Immunology