1. Vasculitis is defined as inflammation of blood vessel walls and can be classified by the size of vessels involved - large, medium, or small. 2. Some examples of large vessel vasculitis include giant-cell arteritis, which commonly involves the temporal artery in older patients, and Takayasu arteritis, which usually occurs in younger patients. 3. Medium vessel vasculitis encompasses diseases like polyarteritis nodosa, characterized by necrotizing inflammation of renal arteries, and Kawasaki disease, an arteritis that can cause coronary aneurysms in children. 4. Small vessel vasculitis includes Wegener's granulomatosis, Churg-Stra

1. VASCULITIS
DR. SHANMUGASAMY.K
ASST PROFESSOR

2. Content :
• Introduction
• Classification of vasculitis
• Pathogenesis
• Large vessel vasculitis.
• Medium sized vasculitis.
• Small vessel vasculitis.

3. Introduction:
• It is defined as inflammation of vessel walls.
• Most commonly affects small vessels like
arterioles, capillaries and venules.
• FACTORS:
- Vessel size,
- Role of immune complexes,
- Presence of specific autoantibodies,
- Granuloma formation and
- Organ specificity.

4. Blood vessels

5. CLASSIFICATION
LARGE SIZED VESSELS
– Temporal arteritis
– Takayasu’s arteritis
MEDIUM SIZED VESSELS
– Polyarteritis nodosa
– Kawasaki’s disease
SMALL SIZED VESSELS
– Wegener’s granulematosis
– Churg-Strauss syndrome
– Microscopic polyangiitis

8. Pathogenesis:
CAUSES
INFECTIOUS IMMUNOLOGICAL
- Immune complex deposition
- antineutrophil cytoplasmic ab
- anti–endothelial cell ab

9. 1. Giant-Cell (Temporal) Arteritis
• Most common type of vasculitis
• Patients >50, F:M = 2:1.
• Chronic, granulomatous
inflammation of large to small
arteries, especially in head
particularly the branches of the
carotid artery (temporal a. and
branches of the ophthalmic a.)
• Involvment is segmental, acute
and chronic.

10. Clinical Features
 Symptoms :
fever, facial pain or headache, often most intense
along the course of the superficial temporal artery,
Thickened and painful temporal artery
Jaw pain
Visual problems and acute vision loss
 The diagnosis depends on biopsy and histologic
confirmation.
 Treatment: corticosteroids

12. Giant-Cell (Temporal) Arteritis:
morphology
 Granulomatous inflammation
of the blood vessel wall
 Giant cells
 Disruption and fragmentation
of internal elastic lamina
 Proliferation of the intima with
associated occlusion of the
lumen.
 The healed stage reveals
collagenous thickening of the
vessel wall and the artery is
transformed into a fibrous
cord

13. Takayasu arteritis
• Granulomatous vasculitis of medium and
larger arteries characterized principally by
ocular disturbances and marked weakening of
the pulses in the upper extremities -Pulseless
disease .
• Takayasu arteritis manifests with transmural
fibrous thickening of the aorta—particularly
the aortic arch and great vessels—and severe
luminal narrowing of the major branch
vessels.

14. • Histological changes range from adventitial
mononuclear infiltrates with perivascular
cuffing of the vasa vasorum, to intense
mononuclear inflammation in the media, to
granulomatous inflammation, replete with
giant cells and patchy medial necrosis.

15. Takayasu arteritis

16. Takayasu arteritis

17. Polyarteritis Nodosa
• Disease of young adults.
• there is segmental necrotizing inflammation of arteries of
medium to small size, in any organ (esp kidney) except the
lung.
• Most frequently kidneys, heart, liver, and gastrointestinal
tract.
• Polyarteritis nodosa has been associated with hepatitis B or
hepatitis C

18. Polyarteritis Nodosa
Clinical manifestations result from ischemia and
infarction of affected tissues and organs.
 Weakening of the arterial wall due to the
inflammatory process may cause aneurysmal dilation
or localized rupture.
Fever, weight loss, abdominal pain and melena (bloody
stool), muscular pain and neuritis.
Renal arterial involvement is often prominent and is a
major cause of death.

19. Polyarteritis Nodosa
Ischemic ulcers

20. Particularly characteristic of PAN is that all the
different stages of activity ( i.e. active and
chronic stages) may coexist in same artery or
in different vessels at the same time.
Fatal if untreated, but steroids and
cyclophosphamide are curative.

21. Polyarteritis nodosa with segmental inflammation and fibrinoid necrosis and occlusion of the lumen of
this artery. Note that part of the vessel wall at the left side is uninvolved.

23. Kawasaki disease
• It is an acute febrile, usually self-limited illness of
infancy.
• Usually affects large to medium-sized, and even
small, vessels.
• It is the leading cause of acquired heart disease in
children.
• Coronary artery involvement- coronary arteritis
can cause aneurysms that rupture or thrombosis.

24. Kawasaki disease- Morphology
• It exhibits pronounced inflammation affecting
the entire thickness of the vessel wall.
• Fibrinoid necrosis is usually less prominent.
• Also leads to aneurysm formation with
thrombosis .
• Obstructive intimal thickening.

25. • Kawasaki disease is also known as
mucocutaneous lymph node syndrome.
• It presents with conjunctival and oral erythema
and erosion, edema of the hands and feet,
erythema of the palms and soles, a desquamative
rash, and cervical lymph node enlargement.
• Approximately 20% of untreated patients
develop cardiovascular sequelae- myocardial
infarction, and sudden death.

26. Wegener granulomatosis
is a necrotizing vasculitis characterized by the triad of
1) necrotizing granulomas of the upper and lower respiratory
tract
2) necrotizing or granulomatous vasculitis of small to medium-
sized vessels
3) renal disease in the form of necrotizing, crescentic,
glomerulonephritis.

27. Wegener Granulomatosis
 Males are affected more often than females, at an average age of
about 40 years
 C-ANCAs(antineutrophilic cytoplasmic antibodies) is positive in
serum of more than 95% of patients.
 Persistent pneumonitis , chronic sinusitis , mucosal ulcerations of the
nasopharynx , and evidence of renal disease.
 Untreated: fatal - may lead to death within 2 years if not treated.

28. Wegener granulomatosis: palatal ulceration

29. Wegener granulomatosis: palatal destruction

30. Wegener’s Granulomatosis
Orbital and nasal granulomaPulmonary nodes
And cavitation

31. WG

32. WG

33. WG

34. Churg-Strauss syndrome
• Eosinophil-rich and granulomatous
inflammation involving the respiratory tract
and necrotizing vasculitis affecting small
vessels
• Associated with asthma and blood
eosinophilia
• Associated with p-ANCAs.

35. Microscopic polyangitis
• Necrotizing small-vessel vasculitis with few or
no immune deposits.
• necrotizing arteritis of small and medium-
sized arteries can occur.
• Necrotizing glomerulonephritis and
pulmonary capillaritis are common.
• Associated with MPO-ANCAs

36. Microscopic polyangitis

37. THROMBOANGIITIS OBLITERANS
(BUERGER DISEASE)
• It leads to vascular insufficiency.
• it is characterized by segmental, thrombosing,
acute and chronic inflammation of medium-sized
and small arteries.
• Tibial and radial arteries, with occasional
secondary extension into the veins and nerves of
the extremities.
• Affects heavy cigarette smokers, usually before
the age of 35.

38. • Cigarette smoking - Direct endothelial cell toxicity by
some component of tobacco, or an idiosyncratic
immune response to the same agents.
• Microscopically, there is acute and chronic
inflammation, accompanied by luminal thrombosis.
• Typically, the thrombus contains small microabscesses
composed of neutrophils surrounded by
granulomatous inflammation.
• The inflammatory process extends into contiguous
veins and nerves (rare with other forms ofvasculitis),
and in time all three structures become encased in
fibrous tissue.

39. TAO

40. TAKE HOME MESSAGE:
1. LARGE VESSEL VASCULITIS:
s.no Disease Description
1. Giant-cell (temporal) arteritis Granulomatous inflammation; frequently
involves the temporal artery. Usually occurs in
patients older than age 50.
2. Takayasu arteritis Granulomatous inflammation usually occurring in
patients younger than age 50

41. TAKE HOME MESSAGE
• 2. MEDIUM VESSEL VASCULITIS:
s.no Disease Description
1. Polyarteritis nodosa Necrotizing inflammation typically involving renal
arteries but sparing pulmonary vessels
2. Kawasaki disease Arteritis with mucocutaneous lymph node syndrome;
usually occurs in children. Coronary arteries can be
involved with aneurysm formation and/or thrombosis.

42. TAKE HOME MESSAGE
• 3. SMALL VESSEL VASCULITIS:
s.no Disease Description
1. Wegener granulomatosis Granulomatous inflammation involving the
respiratory tract and necrotizing vasculitis affecting
small vessels, including glomerular vessels.
Associated with PR3-ANCAs.
2. Churg-Strauss syndrome Eosinophil-rich granulomatous inflammation
involving the respiratory tract and necrotizing
vasculitis affecting small vessels. Associated with
asthma and blood eosinophilia. Associated with
MPO-ANCAs.
3. Microscopic polyangiitis Necrotizing small-vessel vasculitis.
Necrotizing glomerulonephritis and pulmonary
capillaritis are common.