ppt presentation of vasculitis

Systemic vasculitis

Nov 13, 2014

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Systemic vasculitis. Dr. Overview. Introduction Classification Pathogenesis Clinical features Differential diagnosis Management Conclusions. Introduction. Systemic vasculitides are Complex Often serious group of disorders which,

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Systemic vasculitis Dr.

Overview • Introduction • Classification • Pathogenesis • Clinical features • Differential diagnosis • Management • Conclusions

Introduction • Systemic vasculitides are • Complex • Often serious group of disorders which, • While uncommon, require careful management in order to ensure optimal outcome • Primary systemic vasculitis has an incidence of • > 100 new cases per million Clinical and Experimental Immunology 2010;160:143–60

Introduction End Stage Renal Disease (ESRD)

Introduction • Despite a significant reduction in mortality as a result of • Standard immunosuppression, most patients experience • Poor quality of life, characterized by • Relapse, persisting low grade disease activity and increasing burden of drug toxicity Clinical and Experimental Immunology 2010;160:143–60

Vasculitis classification– the first step

Primary Systemic Vasculitis classification

Classification of systemic vasculitis

Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitis • Small vessel vasculitis • Wegener’s granulomatosis • Churg-Strauss syndrome • Microscopic polyangiitis • Henoch-Schonlein purpura • Essential cryoglobulinemic vasculitis • Cutaneous leukocytoclastic angiitis • Medium-sized vessel vasculitis • Polyarteritis nodosa • Kawasaki disease • Large-vessel vasculitis • Giant cell (temporal) arteritis • Takayasu arteritis

Classification of systemic vasculitis • 2 major groups based on clinical and histopathological features of vasculitis 1. Large vessel vasculitis: aorta and major branches • Giant cell arteritis / temporal arteritis • Takayasu arteritis 2. Medium-sized vasculitis: medium arteries • Polyarteritis nodosa (PAN) • Kawasaki disease

Classification of systemic vasculitis 2. medium-sized vasculitis: arterioles, capillaries and venules • Wegener’s granulomatosis (WG) • Churg-Strauss syndrome (CSS) • Microscopic polyangiitis (MPA) 3. small vessel vasculitis: venules, capillaries • Henoch Schonlein purpura (HSP) • Cryoglobulinaemic vasculitis

Epidemiology Clinical and Experimental Immunology 2010;160:143–60

Epidemiology • Begin during the 5th, 6th, 7th decades of life • Male predominance • Caucasians have greater incidence than African Americans • Suspicion that Wegener’s more frequent in colder compared to warmer climates, and that microscopic polyangiitis has opposite trend

Pathogenesis of vasculitis J Allergy Clin Immunol 2009;123:1226-36

Pathogenesis of vasculitis • Antibody mediated inflammation • Wegener’s granulomatosis (WG) • Churg-Strass syndrome (CSS) • Microscopic polyangiitis (MPA)

Pathogenesis of vasculitis • Immune complex-mediated inflammation • Henoch SchÖlein purpura (HSP) • Cryoglobulinaemic vasculitis • Polyarteritis nodosa (PAN) • Cell-mediated inflammation • Giant cell arteritis • Takayasu arteritis • Wegener’s granulomatosis (WG) • Churg-Strass syndrome (CSS)

Pathogenesis • All three associated with the presence in serum of autoantibodies against components of the cytoplasm of neutrophils: ANCA • ANCA activates neutrophils, which then adhere to endothelial cells and release mediators of inflammation and cell injury

Clinical features J Allergy Clin Immunol 2009;123:1226-36

Definition – large vessel vasculitis Clinical and Experimental Immunology 2010;160:143–60

Definition – medium vessel vasculitis Clinical and Experimental Immunology 2010;160:143–60

Definition – small vessel vasculitis Clinical and Experimental Immunology 2010;160:143–60

Small vessel pauci-immune vasculitis • Wegener’s granulomatosis: • Necrotizing granulomatous inflammation, most often affecting respiratory tract • Churg-Strauss syndrome: • Occurs in association with asthma, eosinophilia, and necrotizing granulomatous inflammation • Microscopic polyangiitis: • Pauci-immune systemic vasculitis occurring in the absence of asthma and eosinophilia with no evidence of granulomatous inflammation

Clinical Manifestations

General symptoms • Fever • Malaise • Anorexia • Weight loss • Myalgias • Arthralgias

Renal involvement *less frequent in Churg-Strauss *hematuria, proteinuria and renal failure *renal failure usually has characteristics of rapidly progressive glomerulonephritis

Skin *Purpura most common in lower extremities, occurs as recurrent crops. *Nodular lesions occur more frequently in Churg-Strauss and Wegener’s

Upper and lower respiratory tract *pulmonary hemorrhage *nodular or cavitary lesions in Wegener’s and Churg-Strauss *subglottic stenosis, sinusitis, rhinitis, otitis media, ocular inflammation most common in Wegener’s

Cardiac *identified in 50% of pts with Churg-Strauss *<20% in Wegener’s and microscopic polyangiitis *transient heart block, ventricular hypokinesis, infarction, myocarditis, endocarditis, pericarditis

Gastrointestinal *typically abdominal pain, blood in the stool, mesenteric ischemia and rarely perforation *can also mimic pancreatitis and hepatitis

Update on vasculitis: J Allergy Clin Immunol 2009

Differential Dx of Vasculitis • Fibromuscular dysplasia • Cholesterol emboli • Atrial myxoma with emboli • Infective endocarditis • Malignancies,ie lymphamatoid granulomatosis • Bacteremia • Rickettsial dz • Amyloid • SLE

Differential Diagnosis: Pulmonary-Renal Syndrome • Goodpasture’s disease • SLE • Henoch-Schoenlein purpura • Behcet’s syndrome • Essential mixed cryoglobulinemia • Rheumatoid vasculitis • Drugs: penicillamine, hydralazine, propylthiouracil • Acute renal failure with hypervolemia • Severe cardiac failure • Severe bacterial pna with renal failure • Hantavirus infection • Opportunistic infections • ARDS w/ renal failure in multi-organ failure • Paraquat poisoning • Renal vein/IVC thrombosis w/ PE

Differential of Pulmonary Renal Syndrome Goodpasture’s Disease Systemic Vasculitis Wegener’s Granulomatosis Microscopic Polyangiitis Churg-Strauss Syndrome Cryoglobulinemia Henoch-Schonlein Purpura Connective Tissue Disease Polymyositis/Dermatomyositis Progressive Systemic Sclerosis SLE Primary Glomerular Disease IgA Nephropathy Post-Infectious GN Membranoproliferative GN

Anti-neutrophil cytoplasmic autoantibodies • Serologic testing for ANCA is useful diagnostic test, but should be interpreted in the context of other patient characteristics • Testing should include both indirect immunoflourescence microscopy (IFA) and enzyme immunoassay (EIA) • Sensitivity for pauci-immune small vessel vasculitis and GN is 80-90% • ¼- 1/3 of patients with anti-GBM crescentic GN and ¼ of patients with idiopathic immune-complex crescentic GN are ANCA positive • Pts with concurrent ANCA and anti-GBM antibodies have worse prognosis than those with ANCA alone

Anti-neutrophil cytoplasmic autoantibodies

Pathologic diagnosis • Biopsy of involved site • Skin • Muscle • Nerve • Gut • Kidney

Size of vessel involvement • Glomerular vasculitis • Focal necrosis, crescents • Rapidly progressive glomerulonephritis • Extra-glomerular vasculitis • Arteritis of small/medium/large renal arteries

Takayasu’ s arteritis Polyarteritis nodosa

Crescentic glomerulonephritisClassification by immune deposits

Rapidly Progressive glomerulonephritis (RPGN) • Definition and disease associations • The kidney in ANCA vasculitis • Pathogenesis • Treatment and outcomes

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INTRODUCTION

The vasculitides are often serious and sometimes fatal diseases that require prompt recognition and therapy. Symptomatic involvement generally reflects and follows the pattern of affected organs. The distribution of affected organs may suggest a particular type of vasculitis.

This topic will review the nomenclature of the different vasculitides and provide an overview of the approach to the patient with suspected vasculitis. An overview of the treatment of these disorders and detailed discussions of the individual disorders are presented separately. (See "Overview of the management of vasculitis in adults" .)

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Among the notable changes in the 2012 CHCC was the preferential use and adoption of new names for several diseases, consistent with the trend of replacing eponyms with disease names that reflect an increased pathophysiologic understanding of these conditions. Among the name changes are: eosinophilic granulomatosis with polyangiitis, abbreviated EGPA, in place of Churg-Strauss syndrome; granulomatosis with polyangiitis, abbreviated GPA, in place of Wegener's granulomatosis; immunoglobulin A (IgA) vasculitis (Henoch-Schönlein), abbreviated as IgAV, in place of Henoch-Schönlein purpura (HSP); anti-C1q vasculitis as an alternative name for hypocomplementemic urticarial vasculitis, abbreviated HUV; and use of the term "cryoglobulinemic vasculitis" in place of "essential cryoglobulinemic vasculitis." Furthermore, the 2012 CHCC formally adopted the term antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) for the group of three disorders that include microscopic polyangiitis (MPA), GPA, and EGPA, with additional categories also named to describe variable-vessel vasculitis and secondary forms of vasculitis. This nomenclature system is not meant to substitute for classification criteria, which include clinical observations that classify a specific patient into a category for research purposes. (See 'Classification criteria' below.)

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Vasculitis - PowerPoint PPT Presentation

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Vasculitis Vasculitis arises when immune system mistakenly attacks blood vessels. What causes this attack isn't fully known, but it can result from infection or ... – PowerPoint PPT presentation

• Vasculitis arises when immune system mistakenly attacks blood vessels.
• What causes this attack isn't fully known, but it can result from infection or certain medications.
• Severe forms of vasculitis can be caused by the rare autoimmune diseases microscopic polyangiitis and granulomatosis with polyangiitis.
• People with these conditions produce harmful antibodies (anti-neutrophil cytoplasmic antibodies, or ANCAs) that attack immune cells known as neutrophils.
• Resulting inflammation in small- to medium-sized blood vessels can cause severe organ damage and sometimes death.
• Diseases characterized by inflammation and necorosis of artery
• Primary vasculitis damage
• Large vessels
• Medium vessels
• Small vessels
• Histological, patogenetic aspects and clinical feature
• Cranial arteritis
• prevalence 15-30/100 000
• - incidence 18/100 000
• Polyarteritis nodosa medium and small vessels
• Fever, fatigue, loss of weight, artritis, skin changes
• Myalgia, polyneuropathy
• Involvement of brain cca 20
• Wegener granulomatosis small vessels
• Nekrotisans granulomatomas compression of cranial nerves
• Meningitis, hydrocephalus
• Polyneuropathy, myelopathy, cerebrovascular diseases
• Kidney, lung
• Churg-Strauss syndrom
• CNS 6-8 pacients
• Stroke ischemic, hemorrhage, SAH
• Behcet disease
• Multisystem, chronic-relapsing vasculitis, damage predominantly venous system
• Oral ulcerations genital ulcerations, uveitis, erythema nodosum,
• 30 - CNS lesion of pyramidal tract, stroke, headache, venous sinuses thrombosis
• Combination of immunosuppressive drugs to control the inflammation.
• These drugs are commonly high dose steroids (prednisolone) and additional treatment with drugs such as cyclophosphamide or methotrexate may be given.
• The amount of steroid treatment will be reduced quickly over the first few weeks and then more slowly.
• The current standard of care for ANCA-related vasculitis requires daily doses of the harsh immunosuppressant drug cyclophosphamide for 3 to 6 months.
• Daily doses of another immunosuppressant, azathioprine, then follow for a year or more.
• This standard therapy usually clears the vasculitis, but relapse is common.
• In addition, this treatment suppresses the immune system in a non-specific way and has potentially severe side effects.
• Rituximab is specifically targeted to deplete the type of immune cells thought to produce ANCA.
• Multifocal or diffuse damage of CNS with remittent or progredient clinical course
• Leasion of the spinal cord
• CSF increased elements, proteins
• AG narroving and dilatation of the vessels
• MRI ischemic, hemoragic, tumor-like lesions, enhancement of meninges after gadolínium
• Brain biopsy
• Combined immunosuppressive therapy is the treatment of choice for PACNS.
• This therapy was initially proposed after its success in patients with systemic vasculitis such as Wegner granulomatosis and polyarteritis nodosa
• but is not supported by evidence from controlled trials in PACNS.
• An induction regimen for 9-12 weeks Cyclophosphamide 2.5 mg/kg/d coupled with intravenous methylprednisolone, 1 g/d for 3 days, then oral prednisolone, 60 mg/d, to be decreased by 10 mg at weekly intervals to reach a dose of 10 mg/d, if possible.
• A maintenance regimen for further 10 months Alternate day steroids (10-20 mg prednisolone) along with azathioprine, 2 mg/kg/d, substituted for cyclophosphamide.

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