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Vasculitis classification, secondary forms, mimickers
This document discusses vasculitis, including its classification, symptoms, and mimickers. Vasculitis is characterized by inflammation of blood vessels that can lead to ischemia. It is classified as either primary, where vasculitis is the principal feature, or secondary, where it is associated with another condition. Primary vasculitis is further divided based on vessel size affected. Symptoms depend on the vessel size involved and can include limb pain, rashes, organ dysfunction. Secondary causes include drugs, infections like hepatitis, and rheumatologic diseases. The document outlines features of different types and emphasizes vasculitis must be differentiated from conditions that can mimic its presentation.
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Vasculitis classification, secondary forms, mimickers
- 1. Vasculitis- Classification, secondary forms,mimickers Dr. Shinjan Patra
- 2. Introduction • Vasculitis- Aclinico-pathologic process characterized by inflammation of and damage to blood vessels. • Vessel lumen is usually compromised and associated with ischemia of the tissues. • May be the sole manifestation of a disease or may be a secondary component.
- 3. When to Suspecta Vasculitis • Unexplained ischemia: –Claudication, limb ischemia, angina, TIA, stroke, mesenteric ischemia, cutaneous ischemia –Especially in a young individual
- 4. Multi-organ dysfunction • Suggestivefeatures: –Glomerulonephritis –Peripheral neuropathy –Pulmonary hemorrhage –Sub-acute endocarditis –Skin manifestations- purpura, urticaria, livedo reticularis, vesiculo-bullous lesions.
- 5. Classification • Primary Vasculitissyndromes- Vasculitis is the principal feature of the disease. • Secondary vasculitis syndromes- Vasculitis is a secondary manifestation of a connective tissue disorder.
- 6. Primary Vasculitis syndromes •Predominantly Large Vessel (Aorta and its major branches & corresponding vessel in venous circulation)- –Giant cell arteritis –Takayasu’s arteritis –Cogan’s syndrome –Behcet’s disease
- 7. • Predominantly MediumVessel vasculitis- smaller than major aortic branches, yet enough to contain four elements (intima, elastic lamina, media, adventitia) –Polyarteritis Nodosa –Kawasaki’s disease –Buerger’s disease –Primary angitis of CNS
- 8. • Predominantly SmallVessel vasculitis (includes capillaries, post-capillary venules, arterioles- less than 500 μ in outer diameter) Immune Complex mediated- Good- pasture’s disease, HSP, cutaneous leucocytoclastic angitis, essential cryoglobulinemia. ANCA-associated disorders- Granulomatosis with polyangitis, Microscopic polyangitis, Churg-Strauss syndrome.
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- 10. Comparison of predominantC/F in these groups- Large-vessel vasculitis Medium-vessel vasculitis Small-vessel vasculitis Limb Claudication Cutaneous nodules + ulcers Purpura + urticaria Asymmetric blood pressures Digital gangrene Glomerulonephritis Absence/ Inequality of pulses Mononeuritis multiplex Alveolar hemorrhage Bruits Renovascular hypertension Uveitis/ scleritis/ splinter hemorrhages
- 11. Pathophysiologic mechanisms • Pathogenicimmune complex deposition- Lupus vasculitis, Hep-C/hep-B asso vasculitis. • Production of anti-neutrophilic cytoplasmic antibodies- Granulomatosis with polyangitis, microscopic polyangitis, churg-strauss syn. • Pathogenic T-lymphocyte responses and granuloma formation- Giant cell arteritis, Takayasu’s arteritis.
- 12. ANCA- Anti-Neutrophilic Cytoplasmic Antibodies •Antibodies directed against neutrophil granule constituents • c-ANCA – Stains cytoplasm (hence “c”) – Main target antigen: proteinase-3 – Highly specific (>90%) for Wegener’s • p-ANCA – Stains perinuclear (hence “p”) – Main target antigen: myeloperoxidase – A/w MPA and Churg-Strauss
- 13. Secondary Vasculitis syndromes •Specific etiology- - Drug-induced - Hep-C associated cryoglobulinemic vasculitis - Hep-B associated vasculitis - Malignancy associated • Associated with systemic disease- - Lupus vasculitis - Rheumatoid vasculitis - Sarcoid vasculitis
- 14. Drug Induced vasculitis •Examples- Hydralazine, propyl-thiouracil, Allopurinol, Thiazides, Gold, Sulfonamides, Phenytoin, Penicillin. • Palpable purpura- generalized/ limited to lower extremities/ urticarial lesions/ hemorrhagic blisters. • Glomerulonephritis/ pulmonary hemorrhage. • T/t- Glucocorticoids/ Cyclophosphamide
- 15. Hep-C associated cryoglobulinemic vasculitis •Type 2 & 3 cryoglobulinemia- monoclonal/polyclonal IgG and IgM. • C/f- fatigue, arthralgia’s, purpura, raynaud’s phenomenon, peripheral neuropathy & nephropathy. • T/t- Antivirals + Rituximab + low-dose IL-2
- 16. Hep-B associated Vasculitis •Associated with polyarteritis nodosa • Type 2 & 3 cryoglobulinemic vasculitis- fatigue, arthralgia’s, purpura, raynaud’s phenomenon, peripheral neuropathy & nephropathy. • T/t- Tenofovir/ Entacavir.
- 17. RA associated vasculitis •Incidence- less than 1% + hypocomplementemia • C/f- petechiae, purpura, digital infarcts, gangrene, livedo reticularis, painful lower extremity ulcers, sensorimotor polyneuropathy. • Bywater’s lesion- Isolated digital vasculitis with splinter like lesions in peri-ungual region. • T/t- Usual DMARD’s + cyclophosphamide + CS
- 18. Lupus associated vasculitis •Vasculitic rash in 20% of cases. • Ischemic events- TIA, CVA, AMI. • Associated atherosclerosis and dyslipidemia. • T/t- Immunosuppressive + statins.
- 19. Paraneoplastic peripheral neuropathies •Painful symmetric or asymmetric distal axonal sensorimotor neuropathy. • SCLC & lymphoma- commonest. • T/t- CS + cyclophosphamide.
- 20. Vasculitis Mimickers Infectious disease- Bacterial endocarditis- Janeway lesions, septic emboli with hemorrhage & infarction. Disseminated Gonococcal infection- Petechial lesions/ papular lesions/ pustular lesions. Pulmonary Histoplasmosis Coccidioidomycosis Whipple’s disease
- 21. Syphilis- Lymedisease Rocky mountain spotted fever Neoplasms- • Atrial myxomas • Lymphoma • Carcinomatosis
- 22. Coagulopathies- • Antiphospholipid syndrome •Thrombotic/ Immune thrombocytopenic purpura • Warfarin induced skin necrosis • DIC Drug toxicities- • Cocaine • Levamisole • Amphetamines Others- Sarcoidosis, Amyloidosis, Migraine.
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Editor's Notes
- #4 In particular, two types of presentation should alert the clinician to the possibility of systemic vasculitis: (1) unexplained ischemia, such as claudication, limb ischemia, angina, transient ischemic attack, stroke, mesenteric ischemia and cutaneous ischemia, particularly in a young patient or a patient without risk factors for atherosclerosis and (2) multiple organ dysfunction in a systemically ill patient, especially in the presence of other suggestive clinical features.
- #5 In particular, two types of presentation should alert the clinician to the possibility ofpura systemic vasculitis: (1) unexplained ischemia, such as claudication, limb ischemia, angina, transient ischemic attack, stroke, mesenteric ischemia and cutaneous ischemia, particularly in a young patient or a patient without risk factors for atherosclerosis and (2) multiple organ dysfunction in a systemically ill patient, especially in the presence of other suggestive clinical features.
- #13 They are antibodies directed against neutrophil granule constituents. Two main patterns of staining are recognized using indirect immunofluorescence: cytoplasmic (cANCA), a coarse granular staining of the cytoplasm, and perinuclear (pANCA), with staining chiefly around the nucleus, leaving the cytoplasm unstained. The main target antigen for cANCA is proteinase 3, whereas pANCA is usually associated with myeloperoxidase. The finding of cANCA in association with proteinase 3 antibodies is highly specific (>90%) for generalized Wegener's granulomatosis. pANCA associated with myeloperoxidase antibodies are typically found in microscopic polyangiitis and Churg-Strauss syndrome.