Vasculitides (Vasculitis) Dr. Raid Jastania

Vasculitis Inflammation of the walls of the vessels Causes of inflammation: –Infectious, physical, chemical, immune, metabolic, ischemic Most important causes: Immune and infections May affect any site Symptoms: –Fever, Malaise, Weight loss… –symptoms specific to the site involved.

Classification of Vasculitis Direct infection –Bacterial: Neisseria –Fungal: mucor, aspergillous –Viral: Herpes zoster Immunologic: –A. Immune Complex Infection-induced: HepB, HepC SLE Drug-induced –B. Direct antibody Goodpasture syndrome Kawasaki disease (anti endothelial antibody) –C. ANCA-mediated Wegener granulomatosis Microscopic polyangiitis Churg-Strauss syndrome –D. Cell-mediated Rejection –E. Unknown: Giant cell arteritis Polyarteritis nodosa Takayaso arteritis

Classification of Vasculitis

Giant Cell (Temporal) Arteritis Most common vasculitis Acute, or chronic, Granulomatous Affect large-small arteries Common in: Temporal artery, vertebral arteries, ophthalmic artery

Giant Cell (Temporal) Arteritis Clinical presentation –Old >50 years –Fever, fatigue, weight loss –Facial pain, headache along the course of the temporal artery –Ophthalmic artery: visual disturbance, loss

Giant Cell (Temporal) Arteritis Segmental Process –Nodular thickening –Narrowing, thrombosis –Granulomatous inflammation –Affects the inner half of the media layer around the internal elastic lamina –Lymphocytes, giant cells –Healing by fibrosis, scarring

Giant Cell (Temporal) Arteritis Pathogenesis –Not clear –Suspect T-cell immune response –HLA-DR association –Presence of CD4+ T-cells

Takayasu Arteritis Granulomatous vasculitis Medium-large arteries Ocular disturbance Weak pulse of upper arms Fibrous thickening of the aorta Female <40 years ? Immune disease

Polyarteritis Nodosa Small-medium arteries Not arterioles or capillaries Renal vessels Not pulmonary circulation

Polyarteritis Nodosa Segmental transmural necrotizing inflammation Kidney, heart, liver, GI, pancreas, testes, muscle, CNS, skin Aneurysm, infarction, ischemia Fibrinous necrosis Neutrophils, lymphocytes Fibrous thickening

Polyarteritis Nodosa Clinical: –Young adult –Acute, subacute, chronic –Episodic –Fever, malaise, weight loss –Abdominal pain, melena –Muscle pain –neuritis

Kawasaki Disease Mucocutanous lymph node syndrome Acute febrile illness of infancy and childhood Large-medium-small arteries Involve coronary arteries Average age 4 years Conjunctival, oral, erythema, erosion Edema of hands and feet Erythema of palms and soles Lymph node enlargement

ANCA Antibodies against neutrophil cytoplasmic components Heterogenous group C-ANCA (cytoplasmic): –antibodies against porteinase-3, –Seen in Wegener granulomatosis P-ANCA (perinuclear): –antibodies against myeloperoxidase, –Seen in Microscopic polyangiitis, and Churg- Strauss disease

Microscopic Polyangiitis Necrotizing vasculitis Arterioles, capillaries and venules Skin, mucous membranes, lungs, brain, heart, GI, kidney, muscle Necrotizing glomerulonephritis Pulmonary capillaritis Hemoptysis, hematuris, proteinuria Cutaneous purpura P-ANCA in 80%

Churg-Strauss Syndrome Strong association with allergic rhinitis, bronchial asthma, eosinophilia Vessels in lung, heart, spleen, nerves, skin Granulomatous inflammation and eosinophils P-ANCA in 70% of cases

Wegener Granulomatosis Triad of: –Acute necrotizing granuloma of URT or LRT –Necrotizing or granulomatous vasculitis of small-medium vessels –Renal disease: crescentic glomerulonephritis C-ANCA in 95% of cases