Vasculitis Flashcards
Takayasu Arteritis
What? Who? Diagnosis? Treatment?
Granulatomatous inflammation of the aorta and it’s main branches –> aneurysmal dilatation or scarred stenosed vessel
Women, <40yrs, Asians
–> Claudication of extremities, decreased pulsation of brachial arteries, Bruits in subclavian arteries or abdo aorta
Diagnose with Angiography/CT/MRI
Treatment = pred + MTX or AZA and grafting for irreversible stenosis
Giant Cell Arteritis
What? Who? Diagnosis? Treatment?
Granulomatous inflammation and necrosis of the temporal artery
Age >50yrs F>M
Localised headache +/- vision changes
Tenderness or decreased pulse in the temporal artery
ESR >50
Diagnose with temporal artery biopsy - VZV Ag and gaint cells
Treatment = Prednisone
Treat early = better outcomes
Polyarteritis Nodosa
What is it?
Systemic necrotising vasculitis that typically affects the medium sized muscular arteries
20-30% are associated with Hepatitis B
ANCA negative
Does not effect the lungs
No GN on biopsy
No Granulomas
PAN
Presentation?
Fever and malaise Asymmetric peripheral neuropathy Arthralgia Livedo reticularlis or purpura AKI Abdominal pain HTN
PAN
Diagnosis and treatment?
Diagnosis:
- Biopsy of involved vessel
- Mesentric or renal arteriogram - multiple aneurysms and irregular constrictions and occulsion of smaller vessels
Treatment:
- Mild disease = pred
- Mod-severe = Cyclophosphamide and pred
- HBV positive = Antiviral not immunosuppression
Kawasaki’s Disease
What? Who? Diagnosis? Treatment?
Mucocutanous lymph node syndrome
Rare in adults - disease of childhood
Fever, cervical lymphadenopathy, conjunctival injection, rash, erythema of palms and soles with desquamation
–> MI in childhood due to thrombosis of inflammed coronary vessels
Treat with IVIG and aspirin
ANCA Associated small vessel vasculitis types
PAUCI-IMMUNE vasculitis
Granulomatosis with polyangitis = cANCA - PR3
Microscopic polyangitis = pANCA - MPO
Eosinophilic granulomatosis with Polyangitis = pANCA with eosinophilia
Granulomatosis with polyangitis
What organs?
Granulomatosis inflammation of the upper and lower airways with GN
Lung = 90% –> pulmonary haemorrhage or nodular lung lesions
ENT = 90% –> sinusitis, rhinitis, subglottic stenosis ans saddle nose deformity
Kidneys = 80% –> glomerulonephritis +/- proteinuria
Skin = 40% –> purpura, ulceration and necrosis
Granulomatosis with polyangitis
Diagnosis and Treatment
cANCA + in 90% - PR3
CXR and CT chest
Renal Biopsy = IF negative
Biopsy of other organ = granulomas
Acute treatment:
- Pulse methylpred –> pred and cyclophosphamide
- RPGN or haemoptysis = plasmapheresis
Maintenance:
- Pred and AZA or MTX
- Rituxamab
Microscopic polyangiitis
What organs?
Renal = 80% - Glomerulonephritis +/- proteinura
Lung = 40%
GIT = 40%
ENT = 35%
Neurologic system = 30%
Microscopic polyangiitis
Diagnosis and treatment
pANCA + in >80% - MPO
Renal biopsy = IF negative
Biopsy of other site = No granulomas
Acute treatment:
- Pulse methylpred –> pred and cyclophosphamide
- RPGN or haemoptysis = plasmapheresis
Maintenance:
- Pred and AZA or MTX
- Rituxamab
Eosinophilic granulomatosis with polyangiitis
What is it?
Asthma and peripheral eosinophilia with vasculitis effecting multiple organs and extravascular granulomas
Sequential phase:
Prodrome = 2nd3rd decade with atopic disease and asthma
Eosinophilic phase = pulmonary opacities, asthma and peripheral eosinophilia with eosinophilic infiltration of lungs and GIT
Vasculitic phase = 3rd-4th decade with vascular/extravascular gramulomatosis
Eosinophilic granulomatosis with polyangiitis
Diagnosis
Asthma >10% eosinophils in blood Mononeuropathy Migratory or transient pulmonary opacities Paranasal sinus abnormality Biopsy = eosinophils
Eosinphilic granulomatosis with polyangitis
Treatment
Methyl pred –> pred and cyclophosphamide for induction
Maintenance = pred and AZA
Cryoglobulinaemic Vasculitis Types
Type 1 = isolated monoclonal Ig - multiple myeloma or waldenstom’s macroglobulinaemia
Type 2 = Polyclonal Ig with a monoclonal Ig - HIV or HCV
Type 3 = Polyclonal Ig - Connective tissue disease - SLE
