Vasculitis syndromes

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  • Dear Dr.Sarath Menon, I am impressed by your clear understanding of rational diagnostic steps in such complicated issue as vasculitides. I have worked with vasculitides for 10 years. The problem is that you should keep in mind a big list of nosologies and criteria. I like your solution of this problem show in your presentation. I work with this issue too. I would be grateful if you look through my Quick Reference Guide on Diagnosis of vasculitides. My idea is to place all necessary data on two pages of A4 sheet. Please if you find time see http://www.slideshare.net/MikhailValivach/diagnosis-of-vasculitides-and-pseudovasculitides-a-quick-reference-guide-mikhail-valivach-pavlodar-2015 Mikhail Valivach, M.D., Kazakhstan, Pavlodar Regional Diagnostic Center
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Vasculitis syndromes

  1. 1. VASCULITIS SYNDROMES Dr.Sarath Menon.R DIVISION OF RHEUMATOLOGY DEPT. OF MEDICINE MGM MEDICAL COLLEGE,INDORE
  2. 2. OUTLINE  Definition  Classification  Diagnosis  Clinical syndromes  Laboratory evaluation  Management
  3. 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.
  4. 4. CLASSIFICATION
  5. 5. CLASSIFICATION PRIMARY VASCULITIS SYNDROMES PREDOMINANTLY LARGE 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
  6. 6. PATHOGENESIS  Immune complex production & deposition  Production of ANCA  T-Lymphocyte response and granuloma formation
  7. 7. APPROACH TO A CASE OF VASCULITIS  Suspect diagnosis & exclude secondary causes  History,clinical exam,lab.evaluation  Syndrome recognition  Confirmation of diagnosis  Treatment
  8. 8. WHEN TO SUSPECT VASCULITIS?
  9. 9. Nonspecific systemic symptoms Fatigue Malaise Weakness Fever Anorexia Weight loss 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
  10. 10. MEDICAL HISTORY Connective tissue disease (SLE, Sjogren’ssyndrome, RA, Scleroderma, Dermatomyositis ) Malignancy ( Lymphoma, leukemia) TTP Bronchial asthma HIV ds
  11. 11. DRUG HISTORY  Hydralazine  Propylthiouracil  Allopurinol  Thiazides  Gold  Sulphonamides  Phenytoin  penicillin
  12. 12. GENERAL EXAMINATION  Pallor  Lymph nodes  Pulses  Blood pressure  Skin  Eyes  Nasal cavity  Oral cavity
  13. 13. SYSTEMIC EXAMINATION  Respiratory system - cavitatory lesion - fleeting infiltrates  CVS - CHF  GIT - abdominal tenderness - malaena  CNS - mononeuritis multiplex - visual loss - TIA,stroke
  14. 14. SYSTEMIC EXAMINATION  Renal - hematuria - oliguria - hypertension
  15. 15. LABORATORY EVALUATION  Routine blood counts  S.electrolytes  RFT  LFT  Urinalysis  Rheumatoid factor  Blood culture  ESR,CRP  ANA, C3,C4  HIV,HBsAg, anti- HCV
  16. 16. LAB.TESTS  ANCA  CXR  USG abdomen  2D Echo  Angiography/MRA  Biopsy
  17. 17. CLUES TO CLASSIFY TYPE OF VASCULITIS  Small vessel vasculitis - palpable purpura,ulcers - glomerulonephritis - GI bleeding - pulmonary hemorrhage,infiltrates, cavities - peripheral neuropathy
  18. 18.  Medium vessel vasculitis - nodules,livedo reticularis, - digital ischemia,infarcts,gangrene - mesentric ischemia - ischemic renal failure - testicular pain,tenderness  Large vessel vasculitis - stroke - jaw claudication
  19. 19. 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
  20. 20.  Wegeners granulomatosis,CSS,PAN M >F, Middle age  Giant cell arteritis F>M , Elderly
  21. 21. SYNDROME RECOGNITION
  22. 22. WEGENERS GRANULOMATO SIS Laboratory features C/F Age- 40 yrs M:F = 1:1
  23. 23. CHURG STRAUSS SYNDROME MEAN AGE OF ONSET – 48 YRS F:M= 1.2:1 Laboratory features C/F
  24. 24. C/F Laboratory features POLYARTERITIS NODOSA
  25. 25. GIANT CELL ARTERITIS FEMALE PREPONDERANCE AGE>50 YRS Laboratory features C/F
  26. 26. FEVER IS THE MOST COMMON CONSTITUTIONAL SYMPTOM KAWASAKIS DISEASE
  27. 27. 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
  28. 28. TREATMENT
  29. 29. 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
  30. 30.  S/e- - BM suppression - cystitis - bladder cancer - infertility, GI intolerance - pulmonary fibrosis - myelodysplasia
  31. 31.  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
  32. 32.  S/E- - GI intolerance, - hepatotoxicity - stomatitis - BM suppression - teratogenicity
  33. 33.  Azathioprine & mycophenolate mofetil  Indication : - alternative to MTx. - maintaining remission Dosage - azathioprine-2mg/kg/d - mycophenolate- 1gm bd
  34. 34. 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
  35. 35. 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
  36. 36. 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
  37. 37. ROLE OF ANTIVIRAL THERAPY  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
  38. 38. 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
  39. 39. QUIZ
  40. 40. Q1  20 yr old 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-
  41. 41. DIAGNOSIS  Henoch-schonlein purpura  Polyateritis nodosa  Wegeners granulomatosis  Disseminated TB
  42. 42. Q2 A  45 yr 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++
  43. 43. CXR
  44. 44. DIAGNOSIS  Churg strauss syndrome  Allergic broncho pulmonary aspergillosis  Wegeners granulomatosis  SLE
  45. 45. Q2 B-WHICH MARKER WILL BE POSITIVE  P –ANCA  RA factor  ds – DNA  C-ANCA
  46. 46. Q3- IDENTIFY CONDITION?
  47. 47.  HSP  SLE  PAN  Cryoglobulinemia
  48. 48. THANK U….

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