22. Vasulitis Flashcards
What is vasculitis?
Inflammation within blood vessels which distrupts the internal elastic lamina
What kind of necrosis can be caused by vasculitis?
Fibrinoid
What are the features of widespread inflammation?
Temperature
Malaise
Weight loss
Fatigued
What are the causes of secondary systemic vasculitis?
Infection, drugs, malignancy
Cryoglobulinaemia
Connective tissue disease
It is common for vessels to rupture from vasculitis. T/F?
False
How is primary vasculitis classified?
Size of vessel
Whether or not there is granuloma formation
What type of primary granuloma occurs within large vessels?
Temporal arteritis
What type of primary granuloma occurs within medium vessels and is associated with granulomata?
EGPA
Eosinophilic Granulomatosis with Polyangitis
Which types of primary granuloma occur within medium vessels and are not associated with granulomata?
Polyarteritis nodosa
Kawasaki syndrome
What type of primary granuloma occurs in small vessels and is associated with granulomata formation?
GPA
Granulomatosis with Polyangiitis
What type of primary granuloma occurs in small vessels and is not associated with granulomata formation?
Microscopic polyangitis
Henoch Schonlein purpura
What are the causes of mortality in vasculitis?
Due to renal, pulmonary and neuro disease
Effects of immunosuppressive treatment
How is vasculitis diagnosed?
ANCA on serology
Biopsy of affected organ
Angiography in large vessel disease to show aneurysm formation
What conditions cause a false positive ANCA?
Infection
Inflammatory disease
Lymphoma
What organs are most common affected by GPA?
Respiratory tract
-nose bleeds
Glomerulonephritis
What is ANCA?
Collection of autoantibodies against neutrophils
What are the types of ANCA and what do they bind to?
cANCA binds to PR3
pANCA binds to MPO proteins in the cytoplasm of the neutrophil
Which type of ANCA is more closely associated with vasculitis?
cANCA
What types of infection can cause secondary vasculitis?
Subacute Bacterial Endocarditis
Cystic Fibrosis
What is the difference between acute and subacute bacterial endocarditis?
Acute directly due to infection
Subacute presentation is dominated by immune complex destruction
What are the features of subacute bacterial endocarditis?
Valve damage
Immune complex deposition
Widespread inflammation
How is subacute bacterial endocarditis diagnosed?
Pyrexia of unknown origin
3 blood cultures
Vegetations on echo
Acute phase response
What are cryoglobulins?
Igs which stick together in the cold
What causes the features of cryoglobulinaemia?
Vascular spasm
Vasculitis
What is the dermatological presentation of cryoglobulinaemia?
Palpable purpura
-Bumpy due to damage to the blood vessel
What is Raynaud’s phenomenon?
When exposed to the cold, vascular spasm causes fingers to turn white and blue
How does cryoglobulinaemia present?
Skin and Raynaud’s
Joint, renal, neuro and GI involvement
Vasculitis
What is Meltzer’s triad of cryglobulinaemia?
Purpura
Arthralgia
Weakness
How is serum tested for cryoglobulinaemia?
Must be kept at 37C until it is separated in the lab
Kept at 4C and read 3 days later
What causes type one cyroglobulinaemia?
Monoclonal
Myeloma, Waldenstrom’s, B cell lymphoma
What causes type two cryoglobulinaemia?
Mixed monoclonal and polyclonal
Infection, connective tissue disease, lymphoproliferative disease
What causes type three cryoglobulinaemia?
Polyclonal
Connective tissue disease, chronic infection
What is the treatment for cryoglobulinaemia?
Treat the underlying cause
Steroids/ immunosuppression for vasculitis
Plasmaphoresis if life threatening
Rituximab to deplete B cells
