Vasculitides Premchand Anne, MD, MPH PGY II (aka the class that rocks) Internal Medicine/Pediatrics

Objectives basic principles of vasculitis cases/discussion/didactic questions

Vasculitis Inflammation of blood vessels and its consequences Classification Pathology of vasculitis Role of Immune complexes, complement, in vasculitis Presentation: purpura vs. nodule

Doctor, Doctor, my son has a rash for three days. 8 year old male who is very active in sports, presents with complaints of rash and swelling of the feet and ankles. Pt’s mother noticed a progressive bumpy red rash and the swelling over the past three days. Rash is also present on buttocks bilaterally and onset similar to that of the feet. Low grade fevers for a week, no nausea or vomiting but reported frothy urine and intermittent abdominal pain over the past few hours. History of a URI in the family, including the child, 2-3 weeks ago. No family history. No PMHx. QUESTIONS? DIFFERENTIAL DIAGNOSES? WORK UP?

Henoch Schonlein Purpura Self limited vasculitis of the small vessels Occurrence 9/100000 with male predominance 2-8 years of age, most common IgA and C3a mediated disease and can occur with other Renal involvement leads to nephrotic syndrome Clinical diagnosis Arthritis in knees and ankles Angioedema in dependent areas with palpable lesions Intermittent abdominal pain with possibility of intussusception (3%) with currant jelly stools.

Henoch Schonlein Purpura cont... Differential diagnosis PAN with neuro and cardiology involvement Meningococcemia for the rash Kawasaki disease-rash limited to LE, high fever JRA-salmon pink rash that is fleeting Flea and spider bites Labs: Def. Dx: bx of lesion shows leukoclastic angiitis, Bx of renal shows IgA nephropathy and segmental GN with crescents, elevated ESR, and elevated serum IgM and IgG Therapy: symptomatic; high dose steroids if significant GI, Neuro, and renal involvement and baby aspirin if hypercoaguble

Doctor, Doctor, my child has a high fever and a red tongue 3 year old Asian girl is brought in for persistent fevers of 102-104oF for more than two weeks. Patient was taken to primary pediatrician and was given antibiotics for a sorethroat 10days ago but no improvement in fevers, neck masses or the redness of the tongue and oral cavity. Fevers are improving but the girl has conjunctival injection and has periungual and perianal desquamation and incredibly irritable and poor appetite. No N/V/D/myalgias but reporting joint pains. QUESTIONS? DIFFERENTIAL DIAGNOSES? WORK UP?

Kawasaki Disease Aka MUCOCUTANEOUS LYMPH NODE SYNDROME febrile vasculitis of childhood cause is unknown unlikely in <4 months due to maternal Ab 80% are under 5 years of age Path: severe inflammation of medium sized vessels, with strong affinity for the coronary arteries, leading to destruction of the intimal layer (all 3 layers involved in severe cases) Cardiac disease: MOST COMMON, decreased ventricular function 2o myocarditis, pericarditis, and coronary aneurysms, thrombosis, stenosis, and sudden death.

Kawasaki disease cont... Clinical manifestations: Acute phase: 1-2 weeks with fevers upto 104F and unresponsive to Antibiotics Subacute phase: irritability, anorexia, conjuctival infection, desquamation of periungual and perineal regions and arthritis Convalescent stage: 6-8 weeks post onset of disease bilateral bulbar conjunctival injection without exudate strawberry tongue and oral erythema erythema and edema of arms and legs CERVICAL LYMPHADENOPATHY > 1.5 cm Rash Differential Diagnoses: Scarlet fever, TSSS, Measles, SJS, JRA, and RMSF

Kawasaki disease cont... Labs: 2D-Echo absolutely pertinent due to extensive coronary involvement; Ig profile due to elevations in subacute phase, coag studies. Follow up 2D absolutely recommended Therapy: IVIG: decreases CAD by 75% (2g/kg over 10 hours) ASA for hypercoagulable state due to thrombocytosis

Doctor, Doctor, I have painful marks on my legs 25 y/o asian indian female presents with complaints of nightsweats, anorexia, weight loss, fatigue, and myalgias of few weeks duration. She reports coming to the US 3 months ago. In addition to the above symptoms, pt noticed severely painful nodular lesions in the anterior aspects of her legs. Pain is unremitting. Physical exam reveals a thin women in distress secondary pain, (+) carotid bruit on the right, widened and laterally displaced PMI, and Left chest pain relieved with leaning forward. AVSS. (+) splenomegaly. LE exam with nodular lesions resembling erythema nodosum and tender to touch. QUESTIONS? DIFFERENTIAL DIAGNOSES? WORKUP?

TAKAYASU ARTERITIS Vasculitis affecting mostly large caliber vessels, mainly aortic arch branches predominant in Asian and Indian subcontinent peoples, women >>men predominance. 1/3 before the age of 20 Path: preference for aorta and major branches with significant renal disease (GN). Association with TB exposure and HLA-B Differential Diagnoses: Cogan syndrome Behcet’s disease Reiter syndrome Sarcoidosis

TAKAYASU ARTERITIS CONT... Clinical manifestations: pre-pulseless: nightsweats anorexia weight loss fatigue myalgia pulseless: splenomegaly erythema nodosum, syncope, amaurosis fugax dilated CM, myocarditis, and pericarditis (+) vascular bruit. Labs: ESR>60, microcytic hypochromic anemia Confirmation of Dx: angiography for occlusive and aneurysmal disease

TAKAYASU ARTERITIS CONT... Therapy surgical excision of stenotic lesions and management of dilated aorta if indolent disease Immunosuppressives: prednisone (1mg/kg/day) in early stage, cyclophosphamide and methotrexate if refractory to steroids

Doctor, Doctor, my child needs your help 10 year old child presented with complaints of persistent fevers with diagnosis of FUO, diarrhea, skin lesions, numbness and tingling with muscle weakness for the past few days. Physical exam with temp of 101.1, erythematous and necrotic skin lesions on the leg and lateral aspect of the ankle, similar to stasis ulcers, and Left foot drop QUESTIONS? DIFFERENTIAL DIAGNOSES? WORKUP?

POLYARTERITIS NODOSA Clinical manifestations FUO, mononeuritis multiplex depends on variety of vessels involved multiple systems affected, including peripheral nerves, mesenteric vessels, heart and brain often associated with p-ANCA in adults but classis PAN is ANCA negative. Diagnosis: Biopsy of tissue in symptomatic sites is highly sensitive and specific and ANGIOGRAPHY for dilatation of arteries. Differential diagnosis HSP Wegener’s good pasture’s Churg strauss

POLYARTERITIS NODOSA cont... Labs: ESR abnormal urine Hep B and C serology (cause 10-30%) increased B-cell lymphocytes hydralazine, allopurinol, penicillamine causes Therapy Steroids cytoxan prostacyclin

Doctor, Doctor, my teenage son coughed up blood 15 y/o male with hx of chronic sinusitis and congestion, here with complaints of hemoptysis and dyspnea but stable now. QUESTIONS? DIFFERENTIAL DIAGNOSIS? WORKUP

WEGENER’S GRANULOMATOSIS Differential diagnosis sarcoid TB Churg Strauss Epidemiology of WGN mostly kidney and lung with granuolomas adults (4th and 5th decades) >>>kids caucasians >>>noncaucasian initially with respiratory symptoms and then renal insufficiency

WEGENER’S GRANULOMATOSIS Diagnosis: Clinical findings of sinusitis and pulm finding, order high resolution CT. Check ANCA: (90% of WGN have the marker) with 70% sensitivity Bx: lung: granulomas renal: 10% with granulomas, segmental necrotizing GN RX: rapid therapy needed Steroids cyclophosphamide

OTHERS Behcet’s disease =>f/u CT scan of brain for ocular involvement. Cogan syndrome: autoimmune hearing loss, aortitis and keratitis Buerger’s Disease in a male <40 yrs smoker with claudication, rest pain SLE arteritis Temporal arteritis with polymyalgia rheumatica Churg-Strauss Syndrome

Behcet’s Cogan’s

References: Nelson’s Pediatrics Harrison’s Internal Medicine Google search for pictures AAP Board PREP CMDT 2004