Transcript systemic vasculitis

systemic vasculitis
are heterogeneous group of diseases
characterized by inflammation and
necrosis of blood vessel wall , often
associated with organ involvement
systemic vasculitis
 Large vessel:
 large cell arteritis
 Takayask’s arteritis
 Medium vessel:
 classical polyarteritis nodosa
 Kawasaki disease
 Small vessel:
 microscope polyangitis
 Wegener’s granulomatosis
 Churg - strrauss syndrome
 Henoch - schonlein purpura
 Mixed essential cryoglobulinaemia
polymyalgia rheumatica (PMR)
• Clinical syndrome of muscle pain and stiffness
and classically an increased ESR
• Predominantly disease of elderly means age
70
• Female 3: 1 male
Clinical features
 Muscle stiffness and pain, symmetrically
affecting the proximal muscle of the neck, upper
arm and less commonly the buttock and thigh
 Early morning stiffness, wt loss, fatigue,
depression and night sweating
 On examination there may be stiffness and
painful restriction of active shoulder movement
but passive movement are preserved
 Muscle tenderness may be elicited
Investigations
• Elevated ESR, CRP
• Normocitic, normochromic anemia
Management
• Prednisolone 15 mg /day, dramatic response
within 3 days
• Need steroids for 18 months
• Some patients require steroids sparing agents
as MTX or azathioprine
• 15-20 % of patients develop features of GCA
Giant cell arteritis
• Large vessel vasculitis predominantly affecting
branches of the temporal and ophthalmic
arteries
• Mean ages 70 years
• Female 4:1 male
Clinical features
• Abrupt onset, but may be insidious
• Headache localized to the temporal or
occipital region with scalp tenderness
• Jaw pain brought on by chewing or talking and
due to ischemia of the masseters
Clinical features
• Visual disturbance, damage to the optic nerve
result in loss of the visual acuity and field,
reduced color perception and pupillary defect,
complete blindness of one eye
• Anorexia, fatigue, wt loss, depression, and
malaise
• Neurological complication include TIA, brain stem infarct and hemi paresis
Investigations
• Elevated ESR (may be normal)
• Elevated CRP
• Temporal artery biopsy (fragmentation of the
internal elastic lamina with necrosis of the
media with mixed inflammatory cell infiltrate
(lymphocyte, plasma cell, eosonophils))
• Skip lesions are common and negative biopsy
does not exclude the diagnosis
Management
• Systemic steroids (60 mg /day) to prevent
visual loss, reduction should be guided by
symptoms and ESR, aiming for 10 mg daily for
6 weeks, and then decreased by 1 mg / month
• Maintenance therapy is required for at least
one year
• Relapse occur in 30 % and is an indication to
restart high dose steroid with additional
immunosuppressive agents typically
azathioprine or methotrexate
Classical polyarteritis nodosa (PAN)
• Is a necrotizing vasculitis characterized by
transmural inflammation of medium - sized to
small arteries
• All ages can be affected (peak incidence 40 50, f 1: 2 m)
• Hepatitis B is a risk factor for PAN
• Myalgia arthralgia, fever and wt loss,
associated with manifestations of
multisystemic disease
Classical polyarteritis nodosa (PAN)
• Palpable purpura, ulceration, inflammation ,
digital skin infarctions and livedo reticularis
• Symmetrical polyneuropathy due to arteritis
of the vasa nervorum (70 %)
• Severe hypertension and / or renal
impairment may occur due to multiple renal
infarctions, GN is rare
Digital skin infarctions
Digital skin infarctions with gangrene
Tissue necrosis
Wrist drop (mononeuritis multiplex )
Diagnosis by:
• Mesenteric, hepatic or renal angiography,
which shows multiple aneurysm and smooth
narrowing
• Tissue biopsy (muscle or sural nerve)
Treatment
• Steroids and cyclophosphomide are the
treatment of choice for idiopathic disease
• Antiviral therapy used for hepatitis B related
to remove the source of the antigen
• Mortality is less than 20 %, relapse occur in up
to 50 % of patients
• Small vessel disease of arterioles, venules and
capillaries:
Microscopic polyangitis (MPA)
is more common than PAN
• Rapidly progressive GN with alveolar
hemorrhage
• Cutaneous and GI involvement similar to PAN
• Neuropathy 15 %
• Pleural effusion 15 %
• Patient are usually P-ANCA (myeloperoxidase)
- positive
wegner's granulomatosis (WG)
 Upper airway involvement (typically epistaxis,
nasal crusting and sinusitis),saddle shape
deformity , haemoptasis, mucosal ulceration
and deafness due to serous otitis media
 Ocular involvement as proptosis due to
inflammation of the retro - orbital tissue,
diplopia, loss of vision due to optic nerve
compression
 Migratory pulmonary infiltrate and nodules
occur in 50 % of patients
 Minority of patients present with GN
 Patients usually C- ANCA - positive
Saddle shape deformity
ocular manifestations
pulmonary infiltrate and nodules with
cavitations
Churge - Strauss syndrome (CSS)
• Most patients have a prodromal period for
many years characterized by allergic rhinitis,
nasal polyposis and late onset asthma that is
often difficult to control
• Typically there is triad of skin lesion (purpura
or nodules), asymmetric mononeuritis
multiplex and eusinophilia or back ground of
resistant asthma
Typical skin lesions
Churge - Strauss syndrome (CSS)
• Pulmonary infiltrates and pleural or pericardial
effusion due to serositis may be present
• 50 % have abdominal symptoms due to
mesenteric vasculitis
• Either C-ANCA or P-ANCA is present in 40 % of
cases
Pulmonary infiltrate
Management
• The aim of treatment is to induce a remission
and then to maintain remission with minimum
drug toxicity
• Remission induced either with oral
Prednisolone (1 mg /Kg/day) and continuous
oral cyclophosphamide (2 mg/Kg/day) or with
bullous IV methylprednisolone (10 mg /kg)
and cyclophosphamide (15 mg/kg) initially
fortnightly and subsequently monthly
Management
 Cyclophosphamide is usually continued for 612 months followed by maintenance with
azathioprine
 Mensa is used with cyclophosphamide to
reduce the risk of hemorrhagic cystitis
 Co-trimoxazole is used for prophylaxis
against pneumocystis pneumonia
 Plasma exchange used in severe and resistant
cases
 IV gamaglobulin and anti T cell therapies are
consider
Henoch- Schonlein purpura (HSP)
• Occur in children and young adult
• Has good prognosis
• Suffered from purpura over the buttocks and
lower legs, abdominal symptoms (pain and
bleeding), arthritis (knee and ankle) following
an upper respiratory tract infection
Palpable purpura
Henoch- Schonlein purpura (HSP)
• Nephritis occur in 40 % of cases, 4 weeks after
the onset of symptoms
• The diagnosis confirmed by the demonstrating
IgA deposition within and around blood vessel
walls
• Bad prognosis signs: hypertension, abnormal
renal function and protein urea >1.5 g/day
Treatment
• Steroids for GI and joint involvement
• Pulse IV steroids and immunosupression for
nephritis
Behcet’s syndrome
• More common in Japan and eastern
Mediterranean countries where it has an
association with HLA-B51
• Characteristically targets venules
• Oral ulcers are universal, usually deep and
multiple, last for 10- 30 days
• Genital ulceration is less common 60- 80 %
• Skin lesions in form of erythema nodosum,
acneiform lesions, migratory thrombophlebitis
and vasculitis
Behcet’s syndrome
• Pathergy reaction is hyper reactivity at the site
of minor trauma (positive if a pustule
develops within 48 hours)
• Ocular involvement, anterior or posterior
uveitis or retinal vasculitis
• Neurological involvement in 5 %, cause
hemiparesis, recurrent thrombosis
• Renal involvement is rare
Criteria for the diagnosis of Behcet's
syndrome
 Oral ulceration: minor aphthous, major or
herpitiform ulceration at least 3 times in 12
month period
 Plus 2 of:
1. Recurrent genital ulceration
2. Eye lesion
3. Skin lesion
4. Positive Pathergy test
Treatment
• Topical steroid for oral ulceration
• Colchicine for erythema nodosum, arthralgia
and oral ulceration
• Thalidomide for oral and genital ulceration
• Oral steroid in combination with other
immunosuppressive drugs in systemic disease