Transcript Systemic Vasculitis

These are a heterogeneous group of diseases
characterized by inflammation and necrosis of
blood-vessel walls, with associated damage to skin,
kidney, lung, heart, brain and gastrointestinal tract.
There is a wide spectrum of involvement and
disease severity, ranging from mild and transient
disease affecting only the skin, to life-threatening
fulminant disease with
multiple organ failure
Classified according to the size of vessel involved
into:
1- Large vessel –giant cell arteritis ,Takayasu’s
arteritis
2-Medium vessel –classical polyarteritis nodosa ,
Kawasaki disease
3- Small vessel –microscopic polyangiitis
,wegner’s granulomatosis ,Churg-Strauss
syndrome ,Henoch –Schonlein purpura
,mixed essential cryoglobulinaemia



1.
2.
3.
4.
Drug induced Vasculitis
Serum sickness
Vasculitis associated with other primary
disorders
Infection –HBV ,HCV
Malignancy
Rheumatic diseases –SLE ,RA
Endocarditis
Constitutional symptoms –fever ,weight loss
,fatigue
 Skin –purpura , liviido reticularis ,digital
infarction
 Musculoskeletal –arthralgias ,arthritis
 Pulmonary –alveolar hemorrhage , pulmonary
nodules
 GIT –bowl ischemia /infarction
 Renal –GN ,nephrotic syndrome ,renovascular
involvement ,hypertension
 Neurological –mononeuritis multiplex ,visual
disturbances ,stroke ,lightheadedness
 CVS –pulselessness /bruits ,claudication
,aneurysms









Lab abnormalities –anemia ,eosinophilia , elevated acute phase
reactant ,renal insufficiency ,active urinary sediments
Tissue biopsy (skin ,nasal septum ,muscle)
Renal biopsy (RFT/GUE abnormality)
Visceral angiography
ANCA : c-ANCA - p-ANCA (Anti-neutrophil cytoplasmic antibody )
Which are a group of autoantibodies, mainly of the IgG type,
against antigens in the cytoplasm of neutrophil granulocytes (the
most common type of white blood cell) and monocytes. They are
detected as a blood test in a number of autoimmune disorders, but
are particularly
associated with systemic vasculitis.
 Clinical
syndrome of muscle pain and
stiffness and classically ,increased ESR
 Close association with GCA
 Prevalence is 20 per 100 000 (over 50)
 Mean age of onset is 70
 ♀:♂ ratio is 3:1
 ESR
is elevated above 40 mm/hour
 Normochromic ,normocytic anemia
 Elevated CRP (prior to ESR)
Oral corticosteroids
 Prednisolone 15 mg per day
 Dramatic response within 72 hours
 12 -18 months treatment
 Osteoporosis prophylaxis with
bisphosphonate
Steroid sparing agents (methotrexate
,azathioprine)
 Steroid can not be withdrawn at 2 years
 Dose greater than 7.5 mg per day
GCA should be treated promptly
 Chronic
inflammatory granulomatous
panarteritis of elastic arteries
 Aorta and its branches , carotid ,ulnar ,brachial
,radial and axillary arteries are most commonly
involved
 ♀:♂ ratio is 8 :1
 Typical age of onset is 25 -30 years
 Aetiology is unknown
 Thickened and inflammed intima
without fibrinoid degeneration
 Claudication
 Systemic
symptoms
On examination
 Loss of pulses
 Hypertension
 Bruits
 Aortic incompetance
• type 1: localised to the aorta and its
branches
• type 2: localised to the descending
thoracic and
abdominal aorta
• type 3: combines features of 1 and 2
• type 4: involves the pulmonary artery.
 High
ESR
 Normochromic normocytic anemia
 Angiography – coarctation ,occlusion ,
anuerysmal dilatation
 High
dose oral prednesolone
 Additional methotrexate or cyclophosphamide
is usually required
 Reconstructive vascular surgery (avoided
during active inflammation ) benefit
hypertension secondary to aortic or renal
lesion
 5 –year survival rate is 80%
Treatment
Aspirin
(5mg/kg for 14 days)
IV Gamaglobulin
400 mg/kg
daily for 4 days
 PAN
is a necrotising vasculitis characterised by
transmural inflammation of medium sized to
small arteries
 Annual incidence is 2 per million
 Peak incidence is 4th and 5th decade
 ♂:♀ ratio is 2:1
 HBV is a risk factor
 Myalgia
,arthralgia ,fever and weight loss
 Skin lesions –palpable purpura ,ulceration
,infarction and livedo reticularis
 Peripheral neuropathy (70%) –symmetrical
,sensory and motor
 Severe hypertension and/ or renal impairment
 Normochromic
normocytic anemia
 Mild to moderate leukocytosis
 Moderate to profound thrombocytosis
 Elevated ESR ,CRP
 RF ,ANF are negative
 GUE –hematuria , RBC cast
 Hepatitis B and C serology
 Angiography
–multiple anuerysims and
smooth narrowing of mesenteric , hepatic
or renal systems
 Tissue biopsy (muscle or sural nerve)
 HBV
related disease –antiviral therapy
 Idiopathic disease –corticosteroids and
cyclophosphamide
 Mortality
 Relapse
< 20%
–up to 50%
 The
annual incidence is 5 -10 per million
 ♂:♀ ratio is 1:1
 Can be seen at any age (rare before
adolescence)
 Mean age of onset is 40 years
It is a syndrome characterized by:
 Granulomatous inflammation involving the
respiratory tract
 Necrotizing vasculitis affecting small to
medium sized vessel
 Necrotizing GN is common




Upper airway involvement (95%) –epistaxis ,nasal
crusting ,sinusitis ,nasal mucosal ulceration, nasal
septal perforation and deafness (serous otitis media )
Pulmonary involvement(85% -90%) –asymptomatic
infiltrate ,cough
,hemoptysis ,dyspnea and
chest discomfort
Eye involvement(52%) –mild conjunctivitis
,episcleritis ,scleritis ,granulomatous
sclerouveitis , cilliary vessel vasculitis
,retroorbital mass lesion (proptosis
,diplopia ,loss of vision)
 Skin lesion –papule ,vesicle , palpable
purpura ,ulcerations or subcutaneous
nodules
 Renal disease (77%) -GN

 Demonstration
of necrotising
granulomatous vasculitis on tissue biopsy
in the presence of compatible clinical
features (pulmonary tissue offer the
highest diagnostic yield )
 When
biopsy specimens are non
diagnostic, ANCA assays provide
important adjunct to diagnosis
This is a vasculitis of unknown etiology
that characteristically targets small arteries and venules. It
is rare in Western Europe but more common in ‘Silk Route’
countries around the Mediterranean and Japan, where there
is a strong association with HLA-B51.
Oral ulcers are universal ,Unlike aphthous
ulcers, they are usually deep and multiple, and last for
10–30 days. Genital ulcers are
also a common problem,
occurring in 60–80% of cases.,
The usual skin lesions are
erythema nodosum or acneiform lesions, but
migratory
thrombophlebitis and vasculitis also occur.
Ocular
involvement is common and may include anterior
or
posterior uveitis or retinal vasculitis.
Neurological
involvement occurs in 5% and mainly involves
the
brainstem, although the meninges, hemispheres
 Recurrent
oral ulcerations plus 2 of the
followings:
 Recurrent genital ulceration
 Eye lesions
 Skin lesions
 Pathergy test



Recurrent oral ulceration –universal ,usually painful,
shallow or deep with central yellowish necrotic base
,singly or in croups ,anywhere in the oral cavity ,persist
for 1-2 weeks ,no scar formation.
Genital ulceration –less common, more specific ,don’t
affect the glance penis or urethra ,and produce scrotal
scars.
Skin involvement –folliculitis ,erythema nodosum
,acne-like exanthem ,and infrequently vasculitis.
 Leukocytosis
 Elevated
ESR
 Elevated CRP
 Autoantibodies may be found






Mucous membrane involvement –topical
glucocorticoid (mouth wash or paste)
Thalidomide –resistant oral and genital ulceration
Colchicine –erythema nodosum and arthralgia
Thrombophlebitis –aspirin 325 mg /day
Uveitis and CNS-Behcet’s –systemic glucocorticoids
and azathioprin
Interferon –very effective for CNS-Behcet’s and
refractory uveitis