Vasculitis Flashcards
Vasculitis overview- outline types of vasculitis
- *Large vessel vasculitis**
- Takayasu: affects aorta & other great vessels-> stenosis/thrombosis & aneurysm (HTN, +ve angiography, sBP differential, thrills/bruits, elevated acute phase reactants)
- GCA
- *Medium vessel vasculitis**
- Polyarteritis nodosa (cutaneous- without other Sx): aneurysm/stenosis, skin (livedo reticularis, tender SC nodules), myalgi, HTN, peripheral neuropathy/renal
- KD: fever >5d, bilateral conjunctivitis, oral mucosal changes/strawberry tongue, perineal/peripheral changes (desquamation), polymorphous rash, cervical lymphadenopathy (>1.5cm)
Small vessel vasculitis
pANCA (MPO) +ve
- Microscopic polyangiitis
- Churg-Strauss
cANCA (PR3) +ve
- Wegners/granulomatosis with polyangiitis: upper/lower resp tract- recurrent epistaxis, septum perforation/saddle deformity, sinusitis, , renal involvement - haematuria/proteinuria, casts/necrotising GN
Non-ANCA:
- HSP: lower limb purpura/petechiae, abdominal pain, arthralgia, possible IGA nephropathy/proteinuria
- Anti-GBM, isolated cutaneous leucocytoclastic vasculitis
- Hypocomplementemia urticarial vasculitis
Variable: Behcet, Cogan
What conditions does cANCA test for vs pANCA
ANCAs stain cytoplasm of nucleus, detected by immunofluorescence
Usually raised in small vessel vasculitis
Cytoplasmic anti-neutrophil cytoplasmic antibodies (cANCA)/PR3
- GPA/Wegners
Perinuclear anti-neutrophil cystoplasmic antibody (pANCA)/MPO
- polyarteritis nodosa
- MPA
- Kawasaki
- Churg-Strauss
- Lupus/SLE
- IBD
- CF
- Primary sclerosing cholangitis
ANCA vasculitis
Slow progressive or sudden symptom onset
Focal segmental necrotising/crescentic GN
Fevers, malaise, LOW
Joints, eyes, skin, heart, CNS can be involved
Ix:
- Immunofluorescence
- ELISA +ve if Ab directed against antigen present (MPO/pANCA, PR3/cANCA)
- CT (ground glass opacities, lymphadenopathy, cavitating lesions → lungs )
Rx: immunosuppression (steroids, cyclophosphamide, azathioprine rituximab), plasma exchange (if severe GN/pulmonary hemorrhage)
>80% mortality if untreated
Wegner’s/GPA (cANCA)
- Small vessel vasculitis
- Respiratory tract & kidney involvement
- necrotizing vasculitis, crescentic GN - no immune complexes
MPA (pANCA)
- Small vessel vasculitis
- Necrotising vasculitis, crescentic GN, no immune complexes
- No sinus/URT symptoms
Churg Strauss (pANCA)
- Small vessel vasculitis
- Asthma/eosinophilia, cartilage destruction, nerve/pericardium/skin/GT
- Necrotising vasculitis, crescentic GN, no immune complexes
BEHCET’s DISEASE
Common condition in Turkish populations (silk road countries Mediterranean -> Japan), thought to be due to vasculitis
Can involve small, medium & large vessels- arteries & veins
Patho: immune dysregulation/excessive Th1 activity, increased heat shock proteins
Genetics:HLA- B51 allele 8-12y/o
Features:recurrent ulcers (mouth/genitals) >3 episodes per year + 2 of uveitis,pathergy (blisters/pustular reaction 24-48h post sterile needle procedure), skin lesions- erythema nodosum/folliculitis/ulcers/purpura, oligoarthritis, CNS abnormalities (meningitis, psychosis, CN palsies), abdominal pain
Ulcers: oral mucosa, gingiva, lips & tongue, genitals often after puberty, last 3-10 days & heal without scar
Rx: limited evidence- colchicine, AZA, cyclosporine, tacrolimus
Prognosis: variable but low mortality
PAPA/FMF (periodic fever syndromes)
Familial Mediterranean Fever
Gene: MEFV (missense- i.e single bp leads to different a-a), autosomal recessive
Age onset 20yrs
Population: Jews, Armenians, Turkish, Arabs
Features:fever 1-3 days, abdominal pain, pleurisy, arthralgia, scrotal swelling, erysipeloid rash, amyloidosis (most serious complication)
Investigations: Mutational testing
Rx: Colchicine, IL-1 blockade
HSP
HSP
Most common childhood vasculitis
90% in children 3-10yrs
More common in winter months/after URTI
Linked to HLAB34/DRB101
Vasculitis of dermal capillaries/venules
Inflammatory infiltrate of neutrophils/monocytes in BV → IGA deposition in small vessels
Unknown pathogenesis but usually post illness
- *Features**
- Palpable purpura/petechiae/ecchymoses: bilateral, LL>UL
- 75% get arthralgia
- GI: N&V, diarrhoea, abdominal pain, ileus, melena, intussusception
- 50% renal involvement: haematuria/proteinuria, nephritis/nephrotic syndrome (4 days to 4 weeks after), focal segmental proliferative GN
- ICH, seizures, headache, behavior changes- rare
Ix:
elevated inflammatory markers, 90% have haematuria only 5% recurrent/persistent
Skin Bx = IGA deposition/C3, vascultis
Rx: supportive- steroids for analgesia (do not alter disease course), monitor BP, urinalysis
Natural history: most have acute/self limiting course, 30% experience recurrences 4-6weeks after dx, renal disease 1-2% of kids, 8% ESRD
Takayasu arteritis
10-40yrs, 20% diagnoses <19
F>M
Chronic large vessel disease - aorta & major branches
inflammation of vessel wall - T&B cell, NK & macrophage infiltration, granulomas formed in media
Leads to weakening/dilatation → aneurysm, scarring = occlusion/stenosis
Features: systemic (fever, malaise, LOW), HTN, abdominal pain, diminished pulses/asymmetrical sBP, claudiction, Reynauds, rash, arthritis, myocardiyis, splenomegaly
Dx: Angiography + 1 of decreased pulses, BP differences, bruits, HTN
Ix: angiography (MRA/CTA), USS doppler to assess pulses, inflammatory acute phase reactants
Mx: Steroids mainstay, MTZ, azathioprine
20% monophasic, other relapse
Polyarteritis nodosa (PAN, cPAN)
Rare in childhood, mean age Dx 9yrs, often post GAS infection/hep B
Necrotizing vasculitis
- infiltrates of monocytes/granulocytes in BV walls (small/medium)
Unclear detailed pathophysiology
Features: constitutional (LOW, fevers, malaise), abdominal pain, renovascular arteritis (HTN, hematuria, proteinuria), myocarditis/[pericarditis, arthralgias, stroke/TIA
Rash- livedo reticularis, palpable subcutaneous nodules
Ix: angiography- aneurysms
Rx: steroids, cyclophosphamide, treat underlying infection
