Approach to the Patient with

Suspected Vasculitis

Supervised by: Dr. Nabil al.jahawi

Persented by: Dr. Enas futaisi
 When to suspect vasculitis
 Multi-system involvement
 Unexplained fever, weight loss.
 Unexplained raised ESR or CRP
 Occlusive arterial disease or hypertension in young adults.
 Cerebrovascular/cardiovascular events in young.
 Unexplained proteinuria with or without casts.
 Splinter haemorrhages in nails
• Cutaneous lesions
- palpable purpura, erythema, subcutaneous nodules or urticaria.
 Sudden retinal vascular disease without hypertension or diabetes
 Sudden appearance of peripheral neuropathy
- wrist drop, foot drop.
 Unexplained finding of pulmonary nodular/cavitatory lesions.
 Persistent headache with sudden visual impairment (monocular blindness) in
elderly.
 Jaw claudication
 History
-chronicity (acute vs. chronic)
-exposures to drugs, vaccines,chemicals
– risk factors for Hep B/C/HIV
– history of valvular heart disease
- asthma Any :-
-sinus problems skin rash , photosensitivity
-nose bleeds mouth ulcers , hair loss
deafness dry / gritty eyes , dry mouth
haemoptysis Raynauds , pleuritic pain
pins / needles blood clots , miscarriages
• Any malignancy Drugs
-weight loss • Cocaine
-night Sweats
• Phenytoin
CTD?- photosensitivity, oral lesions, muscle
• Sulfa drugs
wkns
• Penicillins
-fevers
• Hydralazine
• Allopurinol
• Symptoms suggestive of systemic vasculitis
• Propylthiouracil
Systemic - malaise, fever, wt loss myalgia,
arthralgia Skin - purpura (palpable) • Thiazides
Physical Exam
 Diagnostic approach
1.Complete History & Physical Examination
ulceration, infarction
• ENT - epistaxis, crusting sinusitis, deafness
• Resp - cough, wheeze haemoptysis, dyspnoea
• Cardiac - chest pain, SOB
• GI - mouth ulcers, abdo pain, diarrhoea
• Neuro - sensory/motor impairment
• Many syndromes are based on clinical rather than lab criteria
• Principle historical & clinical features help to distinguish the major vasculitic
syndromes
Laboratory Investigations
Assessing Inflammation • Liver function
• Blood count & differential - often non specific
– total WCC - may also suggest viral
- leucocytosis consistent with infection & infection
primary vasculitis
-leucopaenia associated with CTD’s
- eosinophils
- elevated in CSS, drug reaction
• Acute phase response ESR/CRP
Assessment of organ involvement • Chest radiograph
• Urine analysis • Liver function
- proteinuria • Nervous system - NCS
- haematuria • Cardiac function - ECG - Echo
- casts • Gut - Angiography
• Renal function
- creatinine clearance
- 24hr prot ein excretion
- biopsy
 Immunological Tests
• Anti-neutrophil cytoplasmic antibodies
– proteinase 3
– myeloperoxidase
• Other autoantibodies
– Rheumatoid factor
– ANA nuclear antibodies
– Antibodies to extractable nuclear antigens
– Anti ds DNA
– Anticardiolipin
• Complement
- levels are low in SLE and infection but high in primary vasculitis
• Cryoglobulins
 Differential diagnosis
• Important to exclude infection and other conditions that may present as
multi-system disease & mimic vasculitis
• Blood cultures
• Viral serology
• Echo cardiography
 Specific Investigations
• Imaging of sinuses
• Biopsy of affected organs eg. skin/kidney/temporal artery
– necessary to confirm diagnosis
 Anti-Neutrophil Cytoplasmic Antibodies
• Diagnosis & classisification of vasculitis has been revolutionised by discovery &
characterisation of ANCA
• Two types of ANCA staining patterns are seen on immunofluorescence (IF),
namely cytoplasmic (c-ANCA) and perinuclear (p-ANCA).
• ELISA should then always be performed in patients with positive results on
immunofluorescence (IF) to identify the specific antigen targeted by ANCA
• Presence of c-ANCA with anti-proteinase 3 (anti-PR3) is highly suggestive of
Wegener’s granulomatosis while p- ANCA with anti-myeloperoxidase (anti-MPO) is
more often encountered in those Churg-Strauss syndrome and microscopic
polyangiitis
• 2 main staining patterns
– cytoplasmic C ANCA
– perinuclear P ANCA
• Highly specific markers for several systemic vasculidities
– Wegners Granulomatosis
– microscopic polyangitis
– Churg-Strauss syndrome
– idiopathic pauci-immune necrotising & cresenteric GN
• Only moderately sensitive in limited or localised disease
• c ANCA correlates with proteinase 3 specificity
– most frequently observed in WG – sensitivity for active WG approx 90%
– can be found in other systemic vasculitis although p ANCA more common
• Anti PR3 ANCA may have role in disease pathogenesis in WG probably by enhancing
disease expression
• In stable WG patients a rise in c ANCA may herald a clinical exacerbation
• p ANCA in most cases induced by antibodies against myeloperoxidase (MPO)
– less specific than c ANCA – Anti MPO antibodies occur in:-
• necrotising GN (65%)
• microscopic polyangitis (45%)
• CSS (60%)
• Wegners (10%)
– can also occur in other conditions but target antigen rarely MPO
• RA IBD
• Malignancy Infection
 The Biopsy
 Biospy newest lesion
 Document LCV
 size of vessels involved
 granulomatous inflamm (CSS orWG)
 lymphocyte rich infiltrate (CTD)
 immunofluorescence?
Cutaneous small vessel vasculitis (CSVV) showing
angiocentric segmental inflammation, endothelial
swelling, neutrophilic infiltrate with leukocytoclasia,
and fibrinoid necrosis of blood vessel walls.