vasculitis

VASCULITIS Definition • Vasculitis - characterised by inflammation and damage to blood vessels • • • • Common problem ALWAYS asked on “boards” Easy to confuse Ambiguity of clinical presentations Limited diagnostic tests Difficulty in obtaining diagnostic tissue Therefore, difficult to diagnose AND classify • The clinical picture is essentially dependent on the size and extent of vessel involvement Etiopathogenesis General clinical symptoms • General symptoms - fever, anorexia, weight - loss, weakness • Polymyalgia, arthralgia • Symptoms of downstream ischemia LARGE VESSEL VASCULITIS -Giant cell arteritis -Takayasu arteritis Aorta Vein MEDIUM SIZED VASCULITIS -Polyarteritis nodosa Kawasaki Disease SMALL VESSEL VASCULITIS -Wegeners granulomatosis -Henoch Schonlen purpura Giant cell / Temporal arteritis Age at disease onset  50 years New headache Temporal artery Elevated erythrocyte sedimentation rate: ESR>50mm/h • Abnormal arterial biopsy: granulomatous inflammation 3 criteria • • • • • Ophthalmic involvement – blindness • - emergency TAKAYASU ARTERITIS Pulseless disease Involves aortic arch and its branches Usually in females younger than 40 years Common carotid…visual defects , strokes Vertebral…dizziness Examination: Weaker pulses and lower pressure in upper limbs when compared to lower limbs Difference of BP > 10mm Hg between upper and lower limbs Wegener granulomatosis • • • • Definition A multisystem disorder Involvement of small vessels KIDNEY ,Upper and lower RESPIRATORY TRACT INVOLVEMENT Inflammation leads to necrosis, granuloma formation Polyarteritis nodosa Small and medium-sized artery inflammation involving the skin, kidney, peripheral nerves, muscles, gut Lungs - spared Henoch-Schönlein purpura The most common vasculitic syndrome of childhood. Generally a benign disorder that follows an intercurrent illness, usually of the upper respirastory tract. A self-limiting course in most children: systemic involvement or serious sequelae are infrequent. Henoch-Schönlein purpura • • • • Palpable purpura Abdominal pain Arthritis Haematuria Kawasaki disease An acute febrile disease occuring most commonly in infants and children under 5 years of age. Muco-cutaneous lymph node syndrome Vasculitis, especially of the coronary arteries, is the most serious and life-threatening complication of the disease. Diagnosis • • • • Clinical symptoms Raised ESR ANCA studies Arteriography • Serum from many patients with vasculitis react with cytoplasmic antigens of neutrophils• Anti - neutrophilic cytoplasmic antibodies (ANCA) • c-ANCA (cytoplasmic) • p-ANCA (peri nuclear) • Useful diagnostic markers Treatment • Corticosteroids • Immunosuppresants A fifty year old man comes to the office with severe pain in the right lower leg, and a petechial rash. He has a six month history of cough and nosebleeds. His urine also has red cell casts and protein. What is the probable type of vasculitis from these symptoms? Good luck You will need it!???