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Vasculitis syndromes
Vasculitis refers to inflammation of blood vessels. This document discusses vasculitis syndromes including definitions, classifications, clinical presentations, diagnostic evaluations, and management. Vasculitides are classified based on the size of vessels predominantly involved as large, medium, or small vessel. Common small vessel vasculitis includes Wegener's granulomatosis and microscopic polyangiitis. Treatment involves immunosuppressants such as cyclophosphamide, steroids, methotrexate, and biologics depending on severity and type of vasculitis. Prompt diagnosis and treatment is important to prevent organ damage from ischemia.
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Vasculitis syndromes
- 1. VASCULITIS SYNDROMES Dr.Sarath Menon.R DIVISIONOF RHEUMATOLOGY DEPT. OF MEDICINE MGM MEDICAL COLLEGE,INDORE
- 2. OUTLINE Definition Classification Diagnosis Clinical syndromes Laboratory evaluation Management
- 3. WHAT IS VASCULITIS? Vasculitis is a clinicopathologic process characterized by inflammation and damage to blood vessels,leading to compromise of the vascular lumen resulting in ischemia of the tissues supplied by the involved vessels.
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- 6. CLASSIFICATION PRIMARY VASCULITIS SYNDROMES PREDOMINANTLYLARGE VESSEL VASCULITIS GIANT CELL VASCULITIS TAKAYASUS ARTERITIS PREDOMINANTLY MEDIUM VESSEL VASCULITIS PAN KAWASAKIS DISEASE PREDOMINANTLY SMALL VESSEL VASCULITIS ANCA +VE--- c-ANCA +VE- WEGENERS GRANULOMATOSIS p-ANCA +VE MICROSCOPIC POLYANGITIS CHURG – STRAUSS SYNDROME ANCA -VE ESSENTIAL MIXED CRYOGLOBULINEMIA HENOCH SCHONLEIN PURPURA IDIOPATHIC CUTANEOUS VASCULITIS BECHETS SYNDROME SECONDARY VASCULITIS SYNDROMES DRUG INDUCED VASCULITIS HYDRALAZINE PROPYLTHIOURACIL ALLOPURINOL THIAZIDES SERUM SICKNESS INFECTIONS RICKETTSIAS SABE EBV HIV MALIGNANCIES LYMPHOMAS CTDs SLE RA SJOGRENS SYNDROME INFLAMMATORY MYOSITIS OTHER PRIMARY BILIARY CIRRHOSIS ULCERATIVE COLITIS ALPHA 1 ANTIITRYPSIN DEFICIENCY RETROPERITPNEAL FIBROSIS
- 7. PATHOGENESIS Immune complexproduction & deposition Production of ANCA T-Lymphocyte response and granuloma formation
- 8. APPROACH TO ACASE OF VASCULITIS Suspect diagnosis & exclude secondary causes History,clinical exam,lab.evaluation Syndrome recognition Confirmation of diagnosis Treatment
- 9. WHEN TO SUSPECTVASCULITIS?
- 10. Nonspecific systemic symptoms Fatigue Malaise Weakness Fever Anorexia Weightloss Skin involvement: palpable purpura, nodules, ulcers, cutaneous or nailfold infarctions Musculoskeletal: range from full–blown arthritis to aches in the joints without obvious swelling (arthralgias) GI: abdominal pain, bleeding Pulmonary symptoms: cough, dyspnea, hemoptysis Ocular symptoms: pain, redness, diplopia, visual loss Cardiac: chest pain, dyspnea Peripheral nerve symptoms: numbness, weakness, pain consistent with mononeuritis multiplex CNS symptoms: stroke, transient ischemic attack (TIA) Renal ds – HTN , hematuria
- 11. MEDICAL HISTORY Connective tissuedisease (SLE, Sjogren’ssyndrome, RA, Scleroderma, Dermatomyositis ) Malignancy ( Lymphoma, leukemia) TTP Bronchial asthma HIV ds
- 12. DRUG HISTORY Hydralazine Propylthiouracil Allopurinol Thiazides Gold Sulphonamides Phenytoin penicillin
- 13. GENERAL EXAMINATION Pallor Lymph nodes Pulses Blood pressure Skin Eyes Nasal cavity Oral cavity
- 14. SYSTEMIC EXAMINATION Respiratorysystem - cavitatory lesion - fleeting infiltrates CVS - CHF GIT - abdominal tenderness - malaena CNS - mononeuritis multiplex - visual loss - TIA,stroke
- 15. SYSTEMIC EXAMINATION Renal -hematuria - oliguria - hypertension
- 16. LABORATORY EVALUATION Routineblood counts S.electrolytes RFT LFT Urinalysis Rheumatoid factor Blood culture ESR,CRP ANA, C3,C4 HIV,HBsAg, anti- HCV
- 17. LAB.TESTS ANCA CXR USG abdomen 2D Echo Angiography/MRA Biopsy
- 18. CLUES TO CLASSIFYTYPE OF VASCULITIS Small vessel vasculitis - palpable purpura,ulcers - glomerulonephritis - GI bleeding - pulmonary hemorrhage,infiltrates, cavities - peripheral neuropathy
- 19. Medium vesselvasculitis - nodules,livedo reticularis, - digital ischemia,infarcts,gangrene - mesentric ischemia - ischemic renal failure - testicular pain,tenderness Large vessel vasculitis - stroke - jaw claudication
- 20. EPIDEMOLOGY OF SYNDROMES Henoch schonlein purpura - M=F, children Kawasaki syndrome - M>F, children Behcets disease - M=F, young adults Takayasu arteritis - F>M, young adults
- 21. Wegeners granulomatosis,CSS,PAN M>F, Middle age Giant cell arteritis F>M , Elderly
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- 26. CHURG STRAUSS SYNDROME MEAN AGE OFONSET – 48 YRS F:M= 1.2:1 Laboratory features C/F
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- 30. FEVER IS THEMOST COMMON CONSTITUTIONAL SYMPTOM KAWASAKIS DISEASE
- 31. HENOCH SCHONLEIN PRUPURA Children Small vessel vasculitis Palpable purpura athralgia Git symptoms- pain,malena,intussusception Renal-glomerulonephitis Myocardial involvement – rare- adults Skin biopsy- leukocytoclastic venulitis with IgA & C3 deposition
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- 36. SPECIFIC THERAPY-IMMUNOMODULATORS Cyclophosphomide Indication: - ANCA + vasculitis- multisystem inv. & life threatening condition - steroid non responsive CSS,PAN Dosage: - 2mg/kg/d- Rx of choice - IV cyclophosp. Intermittent bolus - 15mg/kg thrice infusion every 2 wks, then every 3 wks
- 37. S/e- - BMsuppression - cystitis - bladder cancer - infertility, GI intolerance - pulmonary fibrosis - myelodysplasia
- 38. Methotroxate Indication: - limited WG- non- life threatening - cyclophosphomide toxicity - maintaining remission Dosage - start -0.3mg/kg/week ,inc . by 2.5mg/wk - max.dose- 20-25mg/wk - maintain remission level - 2 yr post remission ,taper down by 2.5 mg /mnt and discontinue
- 39. S/E- - GIintolerance, - hepatotoxicity - stomatitis - BM suppression - teratogenicity
- 40. Azathioprine &mycophenolate mofetil Indication : - alternative to MTx. - maintaining remission Dosage - azathioprine-2mg/kg/d - mycophenolate- 1gm bd
- 41. ROLE OF STEROIDS Indication for first line therapy -severe ulcerative,necrotic cutaneous lesion - GI bleeding - a./c glomerulonephritis - peripheral neuropathy with impending palsy - primary Rx in CSS,GCA,TA Dosage -1mg/kg/d x 1 mnth, then alternate days - taper down & discontinue in 6-9 mnths
- 42. ROLE OF IVIG Indications: Kawasaki disease- - Rx of choice - 2 g/kg single dose infusion over 10 hr with high dose aspirin - prevents aneurysmal formation Henoch schonlein purpura - cutaneous - renal - git involvement
- 43. ROLE OF ASPIRIN Kawasaki disease - high dose 10mg/kg/d X 14 d,then - 3-5mg/kg/d for several weeks - reduce coronary abnormalities Giant cell arteritis - reduce cerebral ischemic complication
- 44. ROLE OF ANTIVIRALTHERAPY Hep .C related cryoglobulinemia - IFN –alpha preffered drug - 3 million IU thrice weekly X 12-18 Months - 60-80% improvement renal,cutaneous,joint - relapse 90%- ribavarin can be added Hep .B related PAN -IFN a + vidarabine + lamivudine in combination with plasma exchange
- 45. ROLE OF SURGERY Takayasu arteritis - surgical/angioplasty for stenosis - reduce risk of stroke - correct HTN due to renal artery stenosis - improves blood flow to viscera & limbs
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- 47. Q1 20 yrold woman presented with bilateral conductive deafness,palpable purpura on legs,hemoptysis.for duration of 1 month Lab- TLC- 12000/mm3 S.Creat- 3mg/dl CXR-
- 49. DIAGNOSIS Henoch-schonlein purpura Polyateritis nodosa Wegeners granulomatosis Disseminated TB
- 50. Q2 A 45yr old male presented to opd c/o dyspnoea athralgia for 2 week. The patient has h/o asthma for 5 yrs on Rx. o/e- P- 82/mt BP- 122/82 mmHg Rt.arm pallor+,no icterus,LN,jvp nasal polyposis + Urinalysis- rbc casts+++,protein++
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- 52. DIAGNOSIS Churg strausssyndrome Allergic broncho pulmonary aspergillosis Wegeners granulomatosis SLE
- 53. Q2 B-WHICH MARKERWILL BE POSITIVE P –ANCA RA factor ds – DNA C-ANCA
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- 55. HSP SLE PAN Cryoglobulinemia
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