Vasculitis is inflammation of blood vessels that can involve different vessel sizes and patterns. It is characterized by leukocytic infiltration of vessel walls. The main causes are immune complex formation, ANCA mediated, and T lymphocyte mediated processes. Diagnosis involves evaluating clinical features, lab tests like ANCA and complement levels, and biopsy of affected tissues. Treatment focuses on immunosuppression with glucocorticoids and other agents depending on severity and organ involvement. Vasculitis often follows a chronic relapsing course.

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