project

2.  Vasculitis- Inflammation of blood vessels
characterised by leucocytic infiltration of the
vessel walls
 Different patterns of vessels’ involvement in
different entities
 Vessel lumen compromisedischemia of the
corresponding organ

3. • 3 main groups of pathogenetic mechanisms
behind vasculitis-
1. Immune complex formation
2. ANCA mediated
3. T lymphocyte mediated with Granuloma
formation

4. • Henoch Schonlein purpura- IgA mediated
• SLE & other collagen vascular diseases-
ANA
• Serum sickness
• Polyarteritis Nodosa- Hepatitis B ag
• Essential Mixed Cryoglobinemia- Hepatitis
C virion
*deposition of immune complexes in the blood
vesselsactivation of
complementsdestruction of vessel wall
(acute & chronic inflammation)

5. • P-ANCA (anti-proteinase 3)- Wegener’s
• C-ANCA (anti-MPO)
- Churg Strauss vasculitis
- Microscopic Polyangiitis
- Wegener’s granulomatosis
* Aberrant expression of proteinase 3 and MPO
over the surface of the neutrophilsformation
of antibodiesdestruction of
neutrophilsvessel wall damage

7. • Giant cell arteritis
• Takayasu’s arteritis
• Wegener’s granulomatosis
• Churg Strauss vasculitis
*classical granuloma formation (giant cells and
epitheloid cells in a backround of fibrinoid
necrosis) can be demonstrated in the
corresponding vessel biopsy

9. “LEARN TO RECOGNISE
VASCULITIS”

10.  Palpable purpura (cutaneous vasculitis)
 Pulmonary infiltrates
 Glomerulonephritis (microscopic hematuria)
 Mononeuritis multiplex
 Unexplained ischemic events- Myocardial
Infarction, Stroke, Raynaud’s phenomena,
Digital gangrene, Mesentric Ischemia

14. RULE OUT SECONDARY CAUSES OF
VASCULITIS!!
i.e- diseases where vasculitis is one of the clinical
manifestations of the respective disease

15.  Infections
 Malignancies
 Thrombotic Microangiopathies
 Drugs
 Others

16. • Bacterial endocarditis
• Gonococcal Infection
• Syphilis
• Rickettsial diseases
• Histoplasmosis
• Coccidiomycosis
• Whipple’s
• Lyme’s

17.  Atrial Myxomas
 Carcinomatosis
 Lymphomas
Thrombotic Microangiopathies
• TTP
• HUS

18. • Cocaine
• Phenytoin
• Sulfa drugs
• Penicillins
• Hydralazine
• Allopurinol
• Propylthiouracil
• Thiazides

19.  SLE
 Amyloidosis
 Sarcoidosis
 Migraine
 Atheroembolic Disease

20. THE PATTERN OF VESSEL INVOLVEMENT
(Large vessel, Medium vessel, Small vessel)

21.  Giant cell arteritis
 Takayasu’s arteritis

22.  Poly Arteritis Nodosa
 Kawasaki’s vasculitis

23. Pauci-immune (ANCA mediated)
Wegener’s Granulomatosis
Churg Strauss vasculitis
Microscopic Polyangiitis
Immune complex mediated
Henoch Schonlein Purpura
Essential Mixed Cryoglobulinemia
SLE and other collagen c=vascular diseases related
vascultis

24.  Thromb Angiitis Obliterans
 Behcet’s disease
 Idiopathic Cutaneous vasculitis
 Isolated Vasculitis of CNS
 Relapsing Polychondritis
 Polyangiitis overlap syndromes (features of
more than 1 vasculitis)

25. Learn the characteristic presentations of each vasculitis !

26.  Temporal arteritis
 Elderly persons more than 50 yrs. of age
 Non specific symptoms, Headache, Elevated
ESR
 BLINDNESS-most serious complication
 Jaw claudication, Scalp pain, Scalp Tenderness
 Polymyalgia Rheumatica- different end of the
spectrum of Giant Cell Arteritis

28.  Pulseless Disease
 Middle aged females
 Aorta and its branches mainly involved
 Subclavian vessels, Carotid vessels, Mesentric
vessels
 Chronic and Relapsing course

30.  Renal arteries most commonly involved
leading to renovascular hypertension
 Pulmonary vessels NEVER involved
 Association with patients of
o Hepatitis B
o Hairy cell leukemia

31.  MucoCutaneous Lymph node syndrome
 Children < 5 years of age mostly
 Desquamative erythematous rashes involving
the skin, mucus membranes, cervical
lymphadenopathy
 25 % develop coronary artery aneurysms in the
convalescent stage of the illness

34. Usually Pulmonary capillaritis PLUS
Glomerulonephritis
•Granulomas +, Asthma +  Churg Strauss
•Granulomas +, NO asthma  Wegener’s
•NO granulomas, NO asthma  Microscopic
Polyangiitis

35. • Classical triad  URT + LRT + renal
• Chronis sinusitis, Pulmonary nodules,
Pulmonary cavities, Rapidly Progressive
Glomerulonephritis
• Cutaneous vasculitis, Eye lesions may be
present
• Non specific symptoms may predominate

37. • Asthma, Eosinophilia with pulmonary infiltrates ,
glomerulonephritis
• Myocardial involvement  most common cause of
death
Microscopic Polyangiitis
• Pulmonary alveolar capillariitis,
glomerulonephritis

38.  2nd decade
 Palpable purpura over lower limbs,
 Gastrointestinal complaints (abd.colicky pain,
blood in stools),
 Fever, polyarthralgia
 Increased IgA levels in blood

40.  5 % of Chronic Hepatits C pts. Have EMC
 Cryoglobulins formed agianst HCV RNA
 Pulmonary, renal ( MPGN ), cutaneous
vasculitis
Thromb Angiitis Obliterans
• Chronic heavy Smokers
• Inflammation of arteries, veins, nerves
• Upper and lower limb gangrene, Instep
claudication, rest pain

41.  Behcet’s disease (Recurrent OculoOroGenital
ulcerations with vasculitis)
 Idiopathic Cutaneous vasculitis
 Isolated Vasculitis of CNS
 Relapsing Polychondritis
 Polyangiitis overlap syndromes (features of
more than 1 vasculitis)

42.  Step 1- Recognise vasculitis
 Step 2- Rule out Sec. Vasculitis
 Step 3- Study the pattern of vessels involved in
the patient
 Step 4- Remember the characteristic
presentations of each primary vasculitis

43. How to diagnose vasculitis???

44. • Mild Anemia – Anemia of Chronic Disease
• Differential Leucocyte Count:
Predominant eosinophils- Churg Strauss, HSP
ESR
• Non specific
• But useful test to suggest presence of
underlying inflammatory process

45. • Acute Phase Reactants
Highly sensitive C reactive Protein, Alpha 2
globulin
• Chest X ray / HRCT thorax:
-Pulmonary infiltrates- small vessel vasculitis
-Pulmonary cavities- Wegener’s granulomatosis
• Xray Para Nasal Sinuses
-Sinusitis of Wegener’s

47. • Urine routine- RBCs with active sediments
suggest Glomerulonephritis (Renal
involvement of small vessel vasculitis)
• Viral Markers
- Hep. B Poly Arteritis Nodosa
- Hep.C Essential Mixed Cryoglobulinemia

48. • Immunoglogulin levels (IgG, M, A)
- Usually hyper gammaglobulinemia seen
- Elevated IgA levelsHenoch Sconlein Purpura
• Cryoglobulins- Essential Mixed Cryoglobulinemia
• Rheumatoid Factors
-To detect secondary vasculitisRheumatoid Arthrits
-Significantly raised in Essential Mixed
Cryoglobulinemia also

49. • Complement levels (reduced in immune compex
mediated diseases)- EMC, HSP
• ANCA
 P-ANCA: Wegener’s Granulomatosis
 C-ANCA: Microscopic polyangiitis, Churg Strauss,
Wegener’s vasculitis
• ANA
-screening of SLE, collagen vascular disorders in
suspicion of secondary vasculitis

50. • Renal Biopsy- to detect glomerulonephritis
especially in small vessel vasculitis
 RPGN- seen in pauci immune vasculitis
 MPGN- seen in EMC
• Skin Biopsy- to detect “leukocytoclasis” in
cutaneous vasculitis all small vessel and
secondary vasculitides

51. • Temporal Artery Biopsy- Giant Cell Arteritis
• Pulmonary tissue Biopsy- Small vessel vascultides
• Upper Airway biopsies- Wegener’s Vasculitis
* Main purpose of biopsy is to study presence of
leukocytoclasis, characterisitc pathological
alterations in tissues, GRANULOMAS
* Immunofluorescence also helps to study immune
complex deposition, IgA deposition, Complement
deposition

52.  Helps specially in in arteries that cannot be
biopsied easily like Aorta, Coronary artery,
Mesentric vessels
 Presence of vascular patency, Aneurysms
• Aortic Angiography- Takayasu’s
• Cerebral Angiography- Isolated CNS vascultis
• Renal Angiography- PAN
• Coronary Angiography- Kawasaki’s
• Lower limb arteriography-Buerger’s Disease (TAO)

54. TREATMENT

55. • Immuno Suppression
Glucocorticoids- oral / IV methyl prednisolone
Cyclophosphamide
Methotrexate
Azathioprine
Cyclosporine
Rituximab- anti CD 20 ab
AntiTNF therapies- Infliximab, Adalimumab,
Etanacerpt, Certulizumab

56. • Choice of therapy depends on
 Severity of organ damage
 Extent of Multi System Involvement
 The vascular bed involved (renal, ocular,
coronary)
• Cyclophosphamide + Glucocorticoid therapy
preferred for severe / serious complications
• Glucocorticoids alone will suffice for isolated
mild vascultis like “idiopathic cutaneous
vascultis”

57.  Wherever possible secondary causes
(infections, malignancies) should be sought
and treated
 Anti viral therapy (HCV, HBV)
 ASPIRIN therapy – Kawasaki’s, Giant cell
arteritis
 Intravenous Immunogloguloin Therapy- Prevents
coronary aneurysms in Kawasaki’s

58. • Major toxic side effects of all prescribed drugs
need to be kept in mind
(Osteoporosis, growth retardation, bone marrow
suppression, hepatic toxicity, renal toxicity,
bladder cancer, cystitis …)
• Long term toxicities need to be prevented
• Long term prescription of a single group of
drug to be avoided change over to a drug
with lesser toxicity profile as soon as symptoms
are controlled

59. • Regular Monitoring of Blood Counts, Renal
and hepatic functions
• Most of the Primary vasculitides have one
thing in common
“Chronic, Responsive to treatment, But
Notoriously Relapsing”

60.  Step 1- Recognise vasculitis
 Step 2- Rule out Sec. Vasculitis
 Step 3- Study the pattern of vessels involved in
the patient
 Step 4- Remember the characteristic
presentations of each primary vasculitis
 Step 5- How to Diagnose
 Step 6- Principles of treatment