1. systemic vasculitis
are heterogeneous group of diseases characterized by inflammation and necrosis of blood vessel wall , often associated with organ involvement

2. systemic vasculitis Large vessel: Medium vessel: Small 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

3. 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

4. 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

5. Investigations
Elevated ESR, CRP
Normocitic, normochromic anemia

6. 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

7. Giant cell arteritis
Large vessel vasculitis predominantly affecting branches of the temporal and ophthalmic arteries
Mean ages 70 years
Female 4:1 male

8. 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

9. 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

10. 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

11. 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

12. 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 , f 1: 2 m)
Hepatitis B is a risk factor for PAN
Myalgia arthralgia, fever and wt loss, associated with manifestations of multisystemic disease

13. 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

14. Digital skin infarctions

15. Digital skin infarctions with gangrene

16. Tissue necrosis

17. Wrist drop (mononeuritis multiplex )

18. Diagnosis by:
Mesenteric, hepatic or renal angiography, which shows multiple aneurysm and smooth narrowing
Tissue biopsy (muscle or sural nerve)

19. 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:

20. 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

21. 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

22. Saddle shape deformity

23. ocular manifestations

24. pulmonary infiltrate and nodules with cavitations

25. 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

26. Typical skin lesions

27. 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

28. Pulmonary infiltrate

29. 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

30. Management
Cyclophosphamide is usually continued for 6-12 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

31. 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

32. Palpable purpura

33. 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

34. Treatment Steroids for GI and joint involvement
Pulse IV steroids and immunosupression for nephritis

35. 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 days
Genital ulceration is less common %
Skin lesions in form of erythema nodosum, acneiform lesions, migratory thrombophlebitis and vasculitis

36. 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

37. 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:
Recurrent genital ulceration
Eye lesion
Skin lesion
Positive Pathergy test

38. 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