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
Drug induced VasculitisSerum sicknessVasculitis associated with other primary disordersInfection –HBV ,HCVMalignancyRheumatic diseases –SLE ,RAEndocarditis
Constitutional symptoms –fever ,weight loss ,fatigueSkin –purpura , liviido reticularis ,digital infarctionMusculoskeletal –arthralgias ,arthritisPulmonary –alveolar hemorrhage , pulmonary nodulesGIT –bowl ischemia /infarctionRenal –GN ,nephrotic syndrome ,renovascular involvement ,hypertensionNeurological –mononeuritis multiplex ,visual disturbances ,stroke ,lightheadedness CVS –pulselessness /bruits ,claudication ,aneurysms
Lab abnormalities –anemia ,eosinophilia , elevated acute phase reactant ,renal insufficiency ,active urinary sedimentsTissue biopsy (skin ,nasal septum ,muscle)Renal biopsy (RFT/GUE abnormality)Visceral angiographyANCA : 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 ESRClose association with GCAPrevalence 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Steroid sparing agents (methotrexate ,azathioprine) Steroid can not be withdrawn at 2 years Dose greater than 7.5 mg per day GCA should be treated promptly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 arteriesAorta and its branches , carotid ,ulnar ,brachial ,radial and axillary arteries are most commonly involved♀:♂ ratio is 8 :1Typical age of onset is 25 -30 yearsAetiology is unknownThickened 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 andabdominal aorta• type 3: combines features of 1 and 2• type 4: involves the pulmonary artery.
High ESRNormochromic normocytic anemiaAngiography – coarctation ,occlusion , anuerysmal dilatation
High dose oral prednesoloneAdditional methotrexate or cyclophosphamide is usually requiredReconstructive vascular surgery (avoided during active inflammation ) benefit hypertension secondary to aortic or renal lesion5 –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 arteriesAnnual incidence is 2 per millionPeak incidence is 4th and 5th decade♂:♀ ratio is 2:1HBV is a risk factorMyalgia ,arthralgia ,fever and weight lossSkin lesions –palpable purpura ,ulceration ,infarction and livedo reticularisPeripheral neuropathy (70%) –symmetrical ,sensory and motorSevere hypertension and/ or renal impairment
Normochromic normocytic anemiaMild to moderate leukocytosisModerate to profound thrombocytosisElevated ESR ,CRPRF ,ANF are negativeGUE –hematuria , RBC castHepatitis B and C serology
Angiography –multiple anuerysims and smooth narrowing of mesenteric , hepatic or renal systemsTissue biopsy (muscle or sural nerve)
HBV related disease –antiviral therapyIdiopathic disease –corticosteroids and cyclophosphamideMortality < 20%Relapse –up to 50%
The annual incidence is 5 -10 per million♂:♀ ratio is 1:1Can 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 nodulesRenal 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 thereis a strong association with HLA-B51.Oral ulcers are universal ,Unlike aphthousulcers, they are usually deep and multiple, and last for10–30 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
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.