1. VASCULITIS SYNDROMES
Dr.Sarath Menon.R
DIVISION OF 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.

5. CLASSIFICATION

6. 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

7. PATHOGENESIS
 Immune complex production & deposition
 Production of ANCA
 T-Lymphocyte response and granuloma
formation

8. APPROACH TO A CASE OF VASCULITIS
 Suspect diagnosis & exclude secondary causes
 History,clinical exam,lab.evaluation
 Syndrome recognition
 Confirmation of diagnosis
 Treatment

9. WHEN TO SUSPECT VASCULITIS?

10. 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

11. MEDICAL HISTORY
Connective tissue disease (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
 Respiratory system
- 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
 Routine blood 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 CLASSIFY TYPE OF VASCULITIS
 Small vessel vasculitis
- palpable purpura,ulcers
- glomerulonephritis
- GI bleeding
- pulmonary hemorrhage,infiltrates,
cavities
- peripheral neuropathy

19.  Medium vessel vasculitis
- 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

22. SYNDROME RECOGNITION

23. WEGENERS
GRANULOMATO
SIS
Laboratory
features
C/F
Age- 40 yrs
M:F = 1:1

26. CHURG
STRAUSS
SYNDROME
MEAN AGE OF ONSET – 48
YRS
F:M= 1.2:1
Laboratory
features
C/F

27. C/F
Laboratory
features
POLYARTERITIS
NODOSA

28. GIANT CELL
ARTERITIS
FEMALE
PREPONDERANCE
AGE>50 YRS
Laboratory
features
C/F

30. FEVER IS THE MOST 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

35. TREATMENT

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-
- BM suppression
- 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-
- GI intolerance,
- 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 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

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

46. QUIZ

47. 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-

49. DIAGNOSIS
 Henoch-schonlein purpura
 Polyateritis nodosa
 Wegeners granulomatosis
 Disseminated TB

50. 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++

51. CXR

52. DIAGNOSIS
 Churg strauss syndrome
 Allergic broncho pulmonary aspergillosis
 Wegeners granulomatosis
 SLE

53. Q2 B-WHICH MARKER WILL BE POSITIVE
 P –ANCA
 RA factor
 ds – DNA
 C-ANCA

54. Q3- IDENTIFY CONDITION?

55.  HSP
 SLE
 PAN
 Cryoglobulinemia

56. THANK U….