Vasculitis Flashcards
What types of vasculitis affect the small vessels?
Granulomatosis with polyangitis (GPA)
Eosinophilic granulamotosis with polyangitis (EGPA; Churg-Strauss syndrome)
Microscopic polyangitis
Drug-induced vasculitis
What types of vasculitis affect the medium vessels?
Polyarteritis nodosa (PAN)
Kawasaki disease
Behcet’s Disease
What type of vasculitis affects the large vessels?
Giant cell arteritis (GCA) and polymylagia rheumatica
Takayasu arteritis
What organs are affected in GPA?
Upper and lower respiratory tract
Kidneys
What features are present in GPA?
ANCA-associated vasculitis features
Features specific to GPA: Epistaxis Nasal crusting Saddle-shape nose deformity Eye involvement e.g. proptosis Cranial nerve lesions
What investigations findings suggest a diagnosis of GPA?
cANCA +ve
CXR:
May display lung cavities
Urine dip:
Blood and protein in urine would suggest glomerulonephritis
Renal biopsy:
Epithelial crescents in Bowman’s capsule
What is the management for the ANCA-associated vasculitis conditions?
Steroids: Oral prednisolone IV hydrocortisone Nasal spray Inhaled if lung involvement e.g. EPGA
Immunosuppressants: Cyclophosphamide Methotrexate Azathioprine Rituximab and other monoclonal antibodies
Plasma exchange
What are the features of EGPA?
ANCA-associated vasculitis features
Features specific to EGPA: Asthma Paranasal sinusitis Multineuritis multiplex Eosinophilia of >10% of peripheral blood
What investigations findings suggest a diagnosis of EGPA?
pANCA +ve
FBC:
Elevated eosinophil level
CXR:
Lung cavities
Urine dip:
Blood and protein in urine would suggest glomerulonephritis
Renal biopsy:
Epithelial crescents in Bowman’s capsule
What common features do ANCA-associated vasculitis conditions have?
Renal impairment:
Rapid progressive glomerulonephritis - raised creatinine, haematuria, proteinuria
Respiratory symptoms:
SOB
Haemoptysis
ENT:
Sinusitis
Skin:
Vasculitis rash
Systemic symptoms:
Fatigue
Weight loss
Fever
What other conditions are associated with pANCA antibody?
Ulcerative colitis (70%)
Primary sclerosing cholangitis (70%)
Anti-GBM disease (25%)
Crohn’s disease (20%)
What features are present in microscopic polyangitis?
ANCA-associated vasculitis features
Mainly causes renal failure, SOB and haemoptysis
pANCA +ve in 50-75%
cANCA +ve in 40%
What condition is PAN associated with?
Hepatitis B
Can also occur in hep C and HIV
What are the features of PAN?
Livedo reticularis Fever, malaise, weight loss Arthralgia Hypertension Mononeuritis multiplex Sensorimotor polyneuropathy Testicular pain Haematuria Renal failure
pANCA in 20%
Hep B serology +ve in 30%
What are the features of Kawasaki disease?
Persistent fever lasting >5 days and is resistant to antipyretics
Conjunctival injection in eye
Bright red, cracked lips
Strawberry tongue
Cervical lymphadenopathy
Erythema and desquamation (skin peeling) of palms and soles
Erythematous rash
What is the management for Kawasaki disease?
High-dose aspirin
IV immunoglobulin
Echo to screen for coronary artery aneurysms
What is the key complication that can occur in Kawasaki disease?
Coronary artery aneurysms
What are the features of Behcet’s disease?
Classic triad:
Oral ulcers
Genital ulcers
Anterior uveitis
Thrombophlebitis and DVT
Erythema nodosum
Arthritis
Neurological involvement e.g aseptic meningitis
GI issue - abdominal pain, diarrhoea, colitis
What investigation is carried out in Behcet’s disease?
Pathergy test
What is the management for Behcet’s disease?
Topical steroids for mouth ulcers (e.g. soluble betamethasone tablets) Oral prednisolone Colchicine Lidocaine ointment for genital ulcers Immunosuppressants Biological therapy
What are the features of Takayasu’s arteritis?
Absent limb pulse due to occlusion of aorta
Systemic features of vasculitis e.g. malaise, headache
Unequel BP in upper limbs
Carotid bruits
Intermittent claudication
Aortic regurgitation (in 20%)
What condition is Takayasu’s arteritis associated with?
Renal artery stenosis
What is the management for Takayasu’s arteritis?
Steroids
