Vasculitis د فاخر يوسف

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

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

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

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

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.

Polymyalgia Rheumatica (PMR) 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

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

Management 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

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 25 -30 years Aetiology is unknown Thickened and inflammed intima without fibrinoid degeneration

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

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.

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

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%

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

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

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

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

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

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

Wegner’s Granulomatosis(WG) 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

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

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

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

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

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.,

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

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

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.

Lab Finding Leukocytosis Elevated ESR Elevated CRP Autoantibodies may be found

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