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Vasculitis - large medium and small vessel disease


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Basic Facts:

Basic Facts Affects all ages , although some types are restricted to certain age groups Tends to affect Caucasians , although many African-Americans are affected Has a genetic component , but is not heritable It is a chronic relapsing disease , although some patients experience prolonged remission

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Inflammatory destruction of blood vessels Infiltration of vessel wall with inflammatory cells Leukocytoclasis Elastic membrane disruption F ibrinoid necrosis of the vessel wall Ischemia , occlusion, thrombosis A neurysm formation R upture, hemorrhage A clinicopathologic process characterized by inflammatory destruction of blood vessels that results in occlusion or destruction of the vessel and ischemia of the tissues supplied by that vessel. “Systemic vasculitides” Definition

You Should Suspect Vasculitis :

You Should Suspect Vasculitis Unexplained signs and symptoms Multisystem involvement Unexplained elevated ESR/CRP Skin lesions (palpable purpura) Ischemic vascular changes (Raynaud’s, gangrene, livedo , claudication) Glomerulonephritis Mononeuritis multiplex Intestinal angina Inflammatory ocular disease Arthralgia's/arthritis, myalgia's Sudden visual loss/headache

Symptoms of vasculitis:

Symptoms of vasculitis Fatigue Weakness Fever Abdominal pain Proteinuria , hematuria , casts Nerve problems (numbness , weakness) Skin rash


CLASSIFICATION Based on Size of Vessels involved Site of involvement Characteristic Features

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Large-vessel vasculitis Giant cell arteritis, Takayasu’s arteritis Behcet’s disease, Cogan’s syndrome Medium-vessel vasculitis Polyarteritis nodosa Buerger’s disease, Central nervous system vasculitis, Kawasaki’s disease, Rheumatoid vasculitis Small-vessel vasculitis Wegener’s Granulomatosis , microscopic polyangiitis, Churg-Strauss (ANCA associated) Cryoglobulinemic vasculitis, Henoch-Schönlein purpura (Non ANCA associated)



Giant Cell Arteritis:

Giant Cell Arteritis Can occur exclusively but often seen with PMR Rare: 15/100,000 Age >50 Cause unknown Involves the medium/large blood vessels of the head and neck including the blood vessels that supply the optic nerve


Pathophysiology Unknown trigger causes inflammatory response with the release of IL-1 and IL-6. This leads to systemic symptoms and the infiltration of inflammatory cells into the adventitia of the temporal and other involved arteries Typical histologic pattern: Giant Cells

Diagnostic Studies:

Diagnostic Studies Temporal Artery Biopsy is the gold standard Elevated ESR and CRP, usually levels higher than in PMR Anemia Elevated LFTs not uncommon


Treatment High dose Steroids (60 mg/day) is the only drug that works Slow taper over time usually 1-2 years. Some patients require low dose (<10 mg/day) chronically


Complications Blindness Scalp Necrosis Lingual Infarction Aortic Dissection/Aneurysm Complications from high dose steroids: osteoporosis, cataracts, elevated blood sugars, wt. gain etc.

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70 %

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Pathergy- An erythematous papule larger than 2 mm at the prick site 48 hours after the application of a 20- to 22-gauge sterile needle, which obliquely penetrated avascular skin to a depth of 5 mm as read by a physician at 48 hours

Polyarteritis Nodosa (PAN):

Polyarteritis Nodosa (PAN) Medium vessel vasculitis Can be caused by Hep B 5/million cases Peak incidence 50’s & 60’s, slightly more common in males


Pathophysiology In Hep B assoc cases immune complexes play significant role In non Hep B cases, the pathophysiology is less understood

Clinical Presentation:

Clinical Presentation Systemic: fever, fatigue, wt loss Abdominal pain due to mesenteric angina/ischemia Mononeuritis multiplex Myalgias/arthalgias/mild arthritis Renal: uremia, Hypertension Skin: livedo reticularis, palpable purpura, fingertip ulceration, subcutaneous nodules Testicular pain or tenderness

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Livedo reticularis


Complications Chronic renal failure Bowel perforation Stroke/cerebral hemorrhage due to HTN Foot/wrist drop


Investigations Elevation of acute phase reactants (ESR, CRP etc) Absence of ANCA Elevated transaminases, decreased albumin +/- Hep B Urine: proteinuria and hematuria without casts

Imaging Studies:

Imaging Studies Mesenteric and/or renal angiography is the test of choice Biopsies seldom done STRING OF PEARLS


Treatment High dose steroids and Cyclophosphamide Methotrexate or Azathioprine is used as steroid sparing agents later once the disease is controlled Treatment for Hep B with antivirals. Sometimes plasma exchange is used to remove immune complexes

PowerPoint Presentation:

Necrotizing Granuloma Hypereosinophilia Sinusitis Subglottic stenosis Pulmonary nodules Orbital pseudotumor Asthma Pulmonary infiltrates Myocarditis Pulmonary capillaritis Glomerulonephritis Sensory neuropathy Mononeuritis multiplex

Wegener’s Granulomatosis:

Wegener’s Granulomatosis Potentially fatal vasculitis involving small vessels Rare: 3-14/million, more common in whites, any age but rare in children Pathology shows necrotizing granulomas usually in upper airways, lungs and kidneys

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Clinical Presentation:

Clinical Presentation Variable, multisystem involvement


Investigations Presence of c-ANCA (cytoplasmic staining pattern antineutrophil cytoplasmic antibodies + clinical picture is often enough to make the diagnosis. It is + 80-90% of generalized WG. Tissue biopsy of lung or kidney Elevated CRP and ESR Anemia, leukocytosis, & thrombocytosis Elevated Cr Active urine sediment with red cell casts, hematuria and proteinuria

Clinical Course / Progression:

Clinical Course / Progression Prior to immunosuppression therapies, WG was uniformly fatal. Now survival rates almost 90% with aggressive treatment. High dose steroids and Cyclophosphamide are cornerstone of therapy. Methotrexate or Azathioprine sometimes used as steroid sparing agents.

Henoch-Schonlein Purpura:

Henoch-Schonlein Purpura


Investigations No specific diagnostic laboratory markers exist The plasma coagulation factor XIII is reduced in about 50% of patients Urinalysis reveals hematuria. Proteinuria may also be found CBC can show leukocytosis with eosinophilia and a left shift. Thrombocytosis is present in 67% of cases Serum IgA levels are increased in about 50% of patients during the acute phase of illness The antistreptolysin O (ASO) titer is elevated in 30% of cases


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


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

Long-term Damage:

Long-term Damage Large-vessel vasculitis 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

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