Vasculitides Flashcards
How is vasculitis classified
Large:
- Takayasu’s arteritis
- Giant cell arteritis
- Polymyalgia rheumatica
Medium:
- Polyarteritis nodosa
- Kawasaki disease
Small:
- Behcets disease
- Granulomatosis with polyangiitis (Wegener’s)
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
- Microscopic polyangiitis
- IgA vasculitis/Henoch-schonlein purpura
- Goodpasture’s/anti-GBM
- Cyroglobulinaemic vasculitis
- Anti-C1q vasculitis
Aetiology and associations for Takayasu’s arteritis
Involves the aorta and its branches
Middle-aged Asian/Japanese women
Symptoms and signs of Takayasu’s arteritis
Inflammatory phase: FLAWS, head or neck pain, tenderness over arteries
Carotid: CNS disease
Syncope on raising arms (subclavian steal)
Pulseless phase: weak/absent UL/LL pulses and upper limb claudication, tenderness over affected arteries
Investigations for takayasu’s arteritis
ESR/CRP: raised
MRA, CTGA - image aorta and arterial tree
FDG-PET: active inflammation
Management for Takayasu’s arteritis
High dose steroid e.g. prednisolone 20mgs PO
Steroid-sparing: cyclophosphamide, methotrexate
Biologics
Endovascular interventions
Aetiology and associations of polyarteritis nodosa
Idiopathic and associated with hepatitis B
GI, renal, coronary arteries affected
Symptoms and signs of polyarteritis nodosa
Constitutional (fever, malaise, lethargy)
Skin rash
Abdominal pain
Rectal bleeding
Peripheral neuropathy
Hypertension
*NO glomerulonephritis
Investigations for polyarteritis nodosa
Hep B serology
Angiography - Rosary sign
Management for polyarteritis nodosa
Mild: PO steroids
Systemic: IV steroids ± IV cyclophosphamide
Anti-virals for current hep B infection
Symptoms and signs of Kawasaki disease
Often <5yo
5 days fever
Conjunctivitis (non purulent, exudative)
Rash, polymorphic
Adenopathy, cervical
Strawberry tongue (prominent papillae and erythema)
Hands and feet swollen, red, tender
Other: Pericarditis with effusion, congestive HF, joint pain or oedema, neurological manifestations, GI manifestations, urological manifestations, dermatological manifestations
Investigations for Kawasaki disease
ECG
FBC: anaemia, leucocytosis/thrombocytosis
CRP/ESR: raised
Echo: Rule out coronary artery aneurysm
Management for kawasaki disease
<10 days from onset:
1. IVIg + high dose aspirin
2. Steroids e.g. IV methylpred or PO pred
3. Immunomodulation e.g. ciclosporin
> 10 days: low dose aspirin PO for 6-8 weeks
+ follow up echo
Association, symptoms and signs of Behcets disease
HLA-B51
TRIAD:
1. Recurrent oral ulcers
2. Genital ulcers
3. Uveitis, iritis, retinal vasculitis , optic neuropathy
Rash, arthritis, pericarditis, colitis, thrombophlebitis, DVT,
Investigations for Behcets disease
Clinical Diagnosis
CRP/ESR raised
Pathergy test - needle prick becomes inflamed and sterile pustule develops within 48 hours
Management for Behcets disease
Prednisolone
Colchicine
Azathioprine, Cyclosporin, Cyclophosphamide sometimes used
TNF alpha blockers
Type I IFN (IFN alpha) rarely
Aetiology, symptoms and signs of Granulomatosis with polyangiitis
c-ANCA (against proteinase 3)
- URT: epistaxis, rhinitis, saddle nose
- LRT: pulmonary haemorrhage → haemoptysis
- Renal: glomerulonephritis → haematuria
± sore eyes, ears, joints, gangrene
Investigations for Granulomatosis with polyangiitis
Urinalysis: haematuria, proteinuria, red cell clasts
cANCA
CRP/ESR raised
CXR shows cavitating lesions
Aetiology, symptoms and signs of Eosinophilic granulomatosis with polyangiitis
p-ANCA
TRI-PHASIC:
1. Allergic: Asthma/rhintiis
2. Eosinophilic: Tissue damage in lung and GIT
- Lung – eosinophilic pneumonitis – transient pulmonary infiltrates
- Skin – nodules
- Nervous system- mononeuritis multiplex
- Sinusitis
- Heart – myocarditis
3. Vasculitic: widespread organ damage and death
Aetiology, symptoms and signs for microscopic polyangiitis
P-ANCA presence
Men > women
Rapidly progressing glomerulonephritis
Pulmonary haemorrhage → haemoptysis
+ vasculitic skin rashes, myositis, mononeuritis multiplex
Aetiology of IgA vasculitis
Post-strep infection → nephritic syndrome 2 days after
Symptoms and signs of IgA vasculitis
Purpura on leg and buttocks
TRIAD
1. Purpuric rash on buttocks and extensors of lower limbs
2. Abdominal pain
3. Arthralgia
(oedema)
Investigations for IgA vasculitis
Raised ESR and CRP
Reduced complement C4 and C3 (sometimes)
IgA may be raised on blood test
Biopsy (skin, kidney) to demonstrate small vessel vasculitis with IgA immune complexes
Aetiology, symptoms and signs for Goodpasture’s disease
Anti-GBM
TRIAD
- Glomerulonephritis
- Haemoptysis (pulmonary haemorrhage)
- Anti-GBM antibodies
Investigations for Goodpasture’s disease
ESR/CRP: raised
CXR and pulmonary function test: pulmonary haemorrhage
Renal biopsy: glomerulonephritis + antibody deposition across the BM
Anti-GBM antibodies
Management for Goodpasture’s disease
High dose prednisolone
Cyclophosphamide
Plasmapharesis
Rituximab
Aetiology of cryoglobulinaemia
Disease due to immune complexes that precipitate at low temperatures
Type 1 – monoclonal antibody that is not a rheumatoid factor
Type 2 – monoclonal rheumatoid factor (mixed)
Type 3 – polyclonal rheumatoid factor (mixed)
Essential = no underlying disease
Secondary to lymphoma, CTD, hep C
Symptoms and signs of cryoglobulinaemia
Skin rash
Arthralgias
Glomerulonephritis
Interstitial lung disease
Neuropathy
Investigations for cryoglobulinaemia
Cryoglobulin within serum
Reduced complement C3, C4
Raised ESR
Investigate for underlying condition (eg hepatitis C serology, ANA, consider lymphoma)
Biopsy to demonstrate small vessel vasculitis with immune complex deposition
Management for cryoglobulinaemia
Treat underlying condition
Prednisolone
Azathioprine as steroid sparing agent
Plasmapharesis in severe disease (rarely)
Rituximab may be effective
Symptoms and signs of anti-C1q disease
Recurrent urticaria with lesions that persist >24 hours (urticarial vasculitis)
Purpura
Arthralgias
Glomerulonephritis
Ocular inflammation
Obstructive airways symptoms
Angiooedema
May occur in patients with SLE
Investigations for anti-C1q disease
Antibodies specific for C1q
Low complement C3 and C4 in more severe cases
Biopsy to show vasculitis
Management for anti-C1q disease
NSAIDs
Prednisolone
Azathioprine
Mycophenolate for severe disease
