1. Vasculitis
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2. 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

3. Primary Vasculitis
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

5. Secondary Vasculitis Drug induced Vasculitis Serum sickness
Vasculitis associated with other primary disorders
Infection –HBV ,HCV
Malignancy
Rheumatic diseases –SLE ,RA
Endocarditis

6. Clinical Features 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

7. Investigations
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.

8. Polymyalgia Rheumatica (PMR)
Clinical syndrome of muscle pain and stiffness and classically ,increased ESR
Close association with GCA
Prevalence is 20 per (over 50)
Mean age of onset is 70
♀:♂ ratio is 3:1

10. Investigations ESR is elevated above 40 mm/hour
Normochromic ,normocytic anemia
Elevated CRP (prior to ESR)

11. Management Oral corticosteroids Prednisolone 15 mg per day
Dramatic response within 72 hours
months treatment
Osteoporosis prophylaxis with bisphosphonate

12. 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

13. Takayasu’s Arteritis
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 years
Aetiology is unknown
Thickened and inflammed intima without fibrinoid degeneration

14. Clinical Features Claudication Systemic symptoms On examination
Loss of pulses
Hypertension
Bruits
Aortic incompetance

15. classified into four types:
• 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.

16. Investigations High ESR Normochromic normocytic anemia
Angiography – coarctation ,occlusion , anuerysmal dilatation

17. Management and Prognosis
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%

21. Treatment
Aspirin
(5mg/kg for 14 days)
IV Gamaglobulin
400 mg/kg
daily for 4 days

22. Polyarteritis Nodosa
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

23. Clinical Features 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

24. Investigation 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

25. Diagnosis
Angiography –multiple anuerysims and smooth narrowing of mesenteric , hepatic or renal systems
Tissue biopsy (muscle or sural nerve)

26. Management and Prognosis
HBV related disease –antiviral therapy
Idiopathic disease –corticosteroids and cyclophosphamide
Mortality < 20%
Relapse –up to 50%

27. Wegner’s Granulomatosis(WG)
The annual incidence is per million
♂:♀ ratio is 1:1
Can be seen at any age (rare before adolescence)
Mean age of onset is 40 years

28. Definition It is a syndrome characterized by:
Granulomatous inflammation involving the respiratory tract
Necrotizing vasculitis affecting small to medium sized vessel
Necrotizing GN is common

29. Clinical features
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

30. 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

31. Diagnosis
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

32. Behcet’s Syndrome
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.,

33. 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 and cord can also be affected, causing pyramidal signs

34. Diagnosis Recurrent oral ulcerations plus 2 of the followings:
Recurrent genital ulceration
Eye lesions
Skin lesions
Pathergy test

35. Clinical Features
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.

36. Lab Finding Leukocytosis Elevated ESR Elevated CRP
Autoantibodies may be found

37. Treatment
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