Vasculitis Philip Seo, MD, MHS Co-Director, the Johns Hopkins Vasculitis Center Compassionate Allowances Outreach Hearing on Autoimmune Diseases 16 March 2011 Vasculitis: Basic Facts Vasculitis affects all ages, although some types are restricted to certain age groups Vasculitis tends to affect Caucasians, although many African-Americans are affected Vasculitis has a genetic component, but is not heritable
Vasculitis is a chronic relapsing disease, although some patients experience prolonged remission Vasculitis: Definition Rheumatologist Inflammatory destruction of blood A clinicopathologic process characterized by vessels inflammatory destruction of Infiltration of vessel wall with blood vessels that results in inflammatory cells
occlusion or destruction of Leukocytoclasis the vessel and ischemia of Elastic membrane disruption the tissues supplied by that Fibrinoid necrosis of the vessel vessel. wall Systemic vasculitides Ischemia, occlusion, thrombosis Pathologist Aneurysm formation
Large-vessel vasculitis Aorta and the great vessels (subclavian, carotid) Claudication, blindness, stroke Medium-vessel vasculitis Arteries with muscular wall Mononeuritis multiplex (wrist/foot drop), mesenteric ischemia, cutaneous ulcers Small-vessel vasculitis
Capillaries, arterioles, venules Palpable purpura, glomerulonephritis, pulmonary hemorrhage ANCA-associated vasculitides Wegeners granulomatosis: granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small to mediumsized vessels Microscopic polyangiitis: Necrotizing vasculitis affecting the small vessels. Churg-Strauss Syndrome: Eosinophil-rich and granulomatous inflammation involving the medium-sized vessels, and associated with
asthma and eosinophilia Necrotizing Granuloma Pulmonary capillaritis Glomerulonephritis Sensory neuropathy Mononeuritis multiplex Sinusitis Subglottic stenosis Pulmonary nodules Orbital pseudotumor
Hypereosinophilia Asthma Pulmonary infiltrates Myocarditis Vasculitis: Diagnosis Diagnosis of a systemic vasculitis is often a diagnosis of exclusion, based on recognition of the clinical syndrome e.g. Churg-Strauss: adult onset asthma x 2 years, followed by atypical pneumonias, followed by peripheral nerve involvement
Biopsy of involved organ is the most straightforward method of establishing a diagnosis Biopsy may be helpful to exclude infection/malignancy Other tests may be suggestive, but not diagnostic
ESR, CRP CT: pulmonary hemorrhage, cavitary lesions Bronchoscopy: pulmonary hemorrhage (hemosiderosis) Urinalysis: for patients with kidney vasculitis ANCA (antineutrophil cytoplasmic antibodies) Angiogram (including MRA, CT-angiogram) Vasculitis: Treatment Remission induction: Cyclophosphamide 2mg/kg po qd x 3-6 months [or 15 mg/kg IV q 2 wk x3 then q 3 weeks x 6-12 months] Prednisone 1mg/kg po qd x 1 month, then taper
[Bactrim, Calcium, Vitamin D] Remission maintenance (minimum 2 years) Methotrexate 20-25 mg po q week + folate Azathioprine 2mg/kg po qd Mycophenolate mofetil 1.5 g po BID Leflunomide 20-30 mg po BID Vasculitis: Monitoring Large-vessel vasculitis MRI/MRA chest/abdomen/pelvis every 6-12 months Medium-vessel vasculitis Mesenteric angiogram to assess disease activity
EMG/NCV to monitor nerve damage Wound care for cutaneous ulcers Small-vessel vasculitis Chest CT every 6-12 months Blood and urine tests every 1-4 weeks Vasculitis: Burden of Disease 32 year old woman with Wegeners granulomatosis Drugs: cyclophosphamide, prednisone, TMP/SMX, Calcium+vitamin D Monitoring:
Lab tests every 1-4 weeks CT scans every 6-12 months PFTs with flow-volume loops, EMG/NCV Consultants: Otolaryngology: Sinusitis, subglottic stenosis
Blindness, Stroke Claudication: Angina of the arms Medium-vessel vasculitis Foot drop: inability to lift a foot Wrist drop: inability to lift a hand Cutaneous ulcerations Small-vessel vasculitis Oxygen dependence Renal insufficiency/failure
Vertebral fracture Fatigue Vasculitis-induced Loss of proprioception Glucocorticoid-induced Muscle loss, Weight gain Emotional liability Immunosuppression-induced Cognitive impairment Drug-associated fatigue Vasculitis: Summary
The systemic vasculitides are chronic diseases, characterized by relapse and remission Achieving remission requires intense monitoring by a multidisciplinary team with expertise in these diseases Even after achieving disease remission, patients will continue to suffer from the chronic, irreversible consequences of both the disease and its therapies Pain and fatigue are common consequences of vasculitis that are independent of disease activity and generally fail to respond to immunosuppression
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