2. Introduction
• Vasculitis- Inflammation of blood vessels
characterised by leucocytic infiltration of the
vessel walls
• Different patterns of vessels’ involvement in
different entities
• Vessel lumen compromisedischemia of the
corresponding organ
3. Pathogenesis
• 3 main groups of pathogenetic mechanisms
behind vasculitis-
1.Immune complex formation
2.ANCA mediated
3.T lymphocyte mediated with Granuloma
formation
4. Immune complex formation
• 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 vesselsactivation of
complementsdestruction of vessel wall
(acute & chronic inflammation)
5. ANCA
• 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 neutrophilsformation
of antibodiesdestruction of
neutrophilsvessel wall damage
6.
7. Granuloma formation
(T lymphocyte mediated)
• 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
23. Small vessel Vasculitis
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. Other primary vasculitides
• 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. STEP 4
Learn the characteristic presentations of each vasculitis !!!
26. Giant cell arteritis
• 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
27.
28. Takayasu’s Arteritis
• Pulseless Disease
• Middle aged females
• Aorta and its branches mainly involved
• Subclavian vessels, Carotid vessels, Mesentric
vessels
• Chronic and Relapsing course
29.
30. Poly Arteritis Nodosa
• 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. Kawasaki’s Vasculitis
• 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
32.
33.
34. Pauci immune Vasculitis
Usually Pulmonary capillaritis PLUS
Glomerulonephritis
•Granulomas +, Asthma + Churg Strauss
•Granulomas +, NO asthma Wegener’s
•NO granulomas, NO asthma Microscopic
Polyangiitis
35. Wegener’s Granulomatosis
• 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
36.
37. Churg Strauss Vasculitis
• Asthma, Eosinophilia with pulmonary infiltrates ,
glomerulonephritis
• Myocardial involvement most common cause
of death
Microscopic Polyangiitis
• Pulmonary alveolar capillariitis,
glomerulonephritis
38. Henoch Schonlein Purpura
• 2nd decade
• Palpable purpura over lower limbs,
• Gastrointestinal complaints (abd.colicky pain,
blood in stools),
• Fever, polyarthralgia
• Increased IgA levels in blood
39.
40. Essential Mixed Cryoglobulinemia
• 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. Other primary vasculitides
• 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. Summary of 4 steps
• 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
44. Common Blood Counts
• 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
46.
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 levelsHenoch Sconlein Purpura
• Cryoglobulins- Essential Mixed Cryoglobulinemia
• Rheumatoid Factors
-To detect secondary vasculitisRheumatoid
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. BIOPSY
• 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. BIOPSY
• 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. ARTERIOGRAPHY
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)
55. Principles of Treatment
• Immuno Suppression
Glucocorticoids- oral / IV methyl prednisolone
Cyclophosphamide
Methotrexate
Azathioprine
Cyclosporine
Rituximab- anti CD 20 ab
AntiTNF therapies- Infliximab, Adalimumab,
Etanacerpt, Certulizumab
56. Principles of Treatment
• 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. Principles of Treatment
• 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. Principles of Treatment
• 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. Principles of Treatment
• 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. SUMMARY OF STEPS
• 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