Gen Med 1 MR

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Gen Med 1 MR

• Rozina Mithani, MD
• December 21,2006

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The Rash
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VASCULITIS

• Definition
• Latin "vasculum", vessel "- itis
• Presence of leukocytes in the vessel wall
• Reactive damage to mural structures
• Types
• Large vessel
• Medium vessel
• Small vessel

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Large Vessel Vasculitis

• Takayasu arteritis
• Aorta and its Primary branches
• Giant Cell arteritis
• Cranial branches orig from Aortic arch

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Medium Vessel Vasculitis

• Polyarteritis Nodosa
• Systemic necrotizing
• Small/Medium Muscular arteries
• Kawasaki Disease
• Esp. branches of coronary arteries
• Children
• Assoc. with mucocutaneous LN syndrome
• Isolated CNS vasulitis
• Diffuse area of CNS vessels

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Small Vessel Vasculitis

• Churg-Strauss arteritis
• Medium/small muscular arteries
• Vascular Extravascular granulomatosis
• Lung Skin
• Wegners granulomatosis
• Venules/Arterioles
• Respiratory tracts Pauci-immune GN
• ANCA
• Microscopic polyarteritis
• Capillaries/venules/arterioles
• Similar to Wegners with ANCA
• HSP
• Deposition of IgA immune complexes
• Form of Hypersensitivity

• Cryoglobulinemic vasculitis
• Most often due to HCV
• Immune complex deposition
• Hypersensitivity vasculitis
• Reaction to known or suspected substance
• Skin vasculitis palpable purpura/petechiae
• Postcapillary venules
• 2/2 Connective Tissue d/o
• Small muscular/arterioles/venules
• SLE, RA, Bechets, Relapsing polychondritis, etc.
• 2/2 Viral Infection
• Most often HCV/HBV
• Immune complex mediated
• Tx anti-viral

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Leukocytoclastic vasculitis

• Who white population other races
• What small vessel vasculitis
• When any age, children HSP
• Where skin but can affect other organs
• esp. kidneys
• Why many causes
• unknown cause in almost 50 cases
• How immune complex mediated

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Physical of LCV

• Palpable purpura - most frequent presentation
• Round and 1-3 mm
• May coalesce to form plaques
• May ulcerate
• Most often on the legs
• Urticarial lesions
• Longer duration, often 24 hrs
• Resolve with some residual pigmentation or
  ecchymosis
• Burning Itching
• Hypocomplementemic COPD risk thus examine heart
  lungs
• Nodular lesions
• Ulceration - more common in large vessel
  vasculitis
• Retiform purpura - linked to IgAassociated
  disease

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Identifying vasculitis

• Systemic symptoms
• Fatigue, weakness, fever, arthralgias, abd pain
• Palpable purpura
• Organ dysfunction
• HTN, Renal Insufficiency, Neuro dysfunction

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The Work-up

• History/Physical
• recent drugs, co-morbidities
• palpable purpura
• Labs
• Basics creatinine, muscle enzyme levels, LFTs,
  ESR/CRP, hepatitis panel, UA, CXR, EKG
• Specific ANA, complement, ANCA
• Others EMG, arteriography
• Tissue bx vascular perivascular infiltration
  PMNs w/formation of nuclear dust
  (leukocytoclasis), extravasation of erythrocytes,
  fibrinoid necrosis of the vessel walls

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Treatment

• Observation
• NSAIDs, Antihistamines, Colchicine, Dapsone
• Corticosteroids
• Immunosuppressive agents
• Cyclophosphamide
• Azathioprine
• MTX

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Goals of Tx

• Induce remission
• Titers often parallel course of vasculitis
• (i.e. ANCA)
• Prevent relapse
• Often with maintenance prednisone
• Prevent treatment related complications

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Complications

• Death
• Early active disease
• Late treatment s/e
• Vessel scarring/narrowing leading to signs of
  ischemia

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Happy Holidays!