This document discusses vasculitis, which is inflammation of blood vessels. It defines vasculitis and describes the different types including large vessel, medium vessel, and small vessel vasculitis. Specific conditions are discussed such as giant cell arteritis, granulomatosis with polyangiitis, Churg-Strauss syndrome, Behcet's disease, thromboangiitis obliterans, and infectious vasculitis. The pathology, clinical features, morphology, and treatment of some of these conditions are summarized. Images are also included showing histological features.

1. VASCULITIS
DR IMRANA TANVIR

2. Vasculitis
 Vasculitis is a general term for vessel wall
inflammation.
 Vessels of any type in virtually any organ can
be affected,but most vasculitides affect small
vessels ranging in size from arterioles to
capillaries to venules.
 The two common pathogenic mechanisms of
vasculitis are immune-mediated inflammation
and direct invasion of vascular walls by
infectious pathogens.

3. Vasculitis: Definition
 Pathologist
 Inflammatory destruction of blood
vessels
 Infiltration of vessel wall with
inflammatory cells
 Leukocytoclasis
 Elastic membrane disruption
 Fibrinoid necrosis of the vessel
wall
 Ischemia, occlusion, thrombosis
 Aneurysm formation
 Rupture, hemorrhage
 Rheumatologist
 A clinicopathologic process
characterized by inflammatory
destruction of blood vessels that
results in occlusion or destruction
of the vessel and ischemia of the
tissues supplied by that vessel.
 “Systemic vasculitides”

5. Vacuities: Classification
 Large-vessel vasculitis
 Giant cell arteritis, Takayasu’s arteritis
 Behcet’s disease, Cogan’s syndrome
 Medium-vessel vasculitis
 Polyarteritis nodosa
 Buerger’s disease, Central nervous system vasculitis,
Kawasaki’s disease, Rheumatoid vasculitis
 Small-vessel vasculitis
 Wegener’s, microscopic polyangiitis, Churg-Strauss
 Cryoglobulinemic vasculitis, Henoch-Schönlein purpura,

6. Vasculitis: Classification
 Large-vessel vasculitis
Aorta and the great vessels (subclavian, carotid)
Claudication, blindness, stroke
 Medium-vessel vasculitis
Arteries with muscular wall
Mononeuritis multiplex (wrist/foot drop), mesenteric
ischemia, cutaneous ulcers
 Small-vessel vasculitis
Capillaries, arterioles, venules
Palpable purpura, glomerulonephritis, pulmonary
hemorrhage

7. Giant Cell (Temporal) Arteritis
 It is a chronic inflammatory disorder of large to
small-sized arteries that principally affects
arteries in the head—especially the temporal
arteries—but also the vertebral and
ophthalmic arteries. Ophthalmic arterial
involvement can lead abruptly to permanent
blindness; consequently, giant cell arteritis is a
medical emergency requiring prompt
recognition and treatment.

8.  T-cell–mediated immune response against
one of handful of vessel wall antigens that
drives
subsequent proinflammatory cytokine production
(particularlyTNF).
 Anti-endothelial cell and anti-smooth muscle
cell antibodies can also be demonstrated in
roughly two thirds of patients, although it is
unclear whether these are causal or a
consequence of other immune injury.

9.  A cellular immune etiology is supported by the
characteristic granulomatous response, a
correlation with certain MHC class II
haplotypes, and a prompt therapeutic
response to steroids.

10. MORPHOLOGY
 Intimal thickening (with occasional thromboses) that
reduces the luminal diameter.
 Classic lesions exhibit medial granulomatous
inflammation centered on the internal elastic lamina that
produce elastic lamina fragmentation;
 There is an infiltrate of T cells (CD4+ CD8+) and
macrophages. Although multinucleated giant cells are
seen in approximately 75% of adequately biopsied
specimens
 Granulomas and giant cells can be rare or absent.

11.  Inflammatory lesions are only focally
distributed along the vessel and long
segments of relatively normal artery may be
interposed.
 The healed stage is marked by medial
attenuation and scarring with intimal
thickening, typically with residual elastic tissue
fragmentation and adventitial fibrosis.

12. Clinical Features.
 Rare before age 50
 Symptoms may be only vague and
constitutional—fever,fatigue, weight loss—or
there may be facial pain or headache, most
intense along the course of the superficial
temporal artery, which can be painful to
palpation.
 Ocular symptoms (associated with
involvement of the ophthalmic artery) appear
abruptly in about 50% of patients;

14. ELASTIN STAIN

15. Doctor, Doctor, my teenage son
coughed up blood
 15 y/o male with hx of chronic sinusitis and
congestion, here with complaints of hemoptysis
and dyspnea but stable now.

17. Granulomatosis with Polyangiitis
 Previously called Wegeners granulomatosis
 Differential diagnosis
 sarcoid
 TB
 Churg Strauss
 Epidemiology of WGN
 mostly kidney and lung with granuolomas
 adults (4th and 5th decades) >>>kids
 caucasians >>>noncaucasian
 initially with respiratory symptoms and then renal insufficiency

18. Granulomatosis with Polyangiitis
 Diagnosis: Clinical findings of sinusitis and pulm finding,
order high resolution CT. Check ANCA: (90% of WGN
have the marker) with 70% sensitivity
 Bx:
 lung: granulomas with geographic patterns of central
necrosis and accompanying vasculitis
 renal: 10% with granulomas, segmental necrotizing GN
 RX: rapid therapy needed
 Steroids
 cyclophosphamide

19. Showing features characteristic of granulomatosis with polyangiitis - a vasculitis and
granulomas with multi-nucleated giant cells.

20. Churg-Strauss Syndrome
 Churg-Strauss syndrome is a small-vessel
necrotizing vasculitis classically associated with
asthma, allergic rhinitis,lung infiltrates, peripheral
hypereosinophilia, and extravascular necrotizing
granulomata.
 Multisystem diseases with cutaneous involvement
(palpable purpura)gastrointestinal tract bleeding,
and renal disease (primarily as focal and
segmental glomerulosclerosis). Myocardial
involvement may give rise to cardiomyopathy

21.  Lung and extrapulmonary
sites (skin, heart, nervous
system, GI) have prominent
eosinophilic infiltrate,
granulomatous reaction
around necrotic foci with
radially arranged histiocytes
and pallisading giant cells
near small arteries or
arterioles, eosinophilic
vasculitis
 ● May have fibrin-rich edema,
lymphocytes, sarcoid-like
granulomas, focal fibrosis and
eosinophilic microabscesses

22. Behçet Disease
 Behçet disease is a small- to medium-vessel
neutrophilic vasculitis that classically presents
as a clinical triad of recurrent oral aphthous
ulcers, genital ulcers, and uveitis.

23. Thromboangiitis obliterans
Buerger’s Disease
 Thromboangiitis obliterans or Buerger's disease is a
segmental occlusive inflammatory condition of arteries
and veins, characterized by thrombosis and
recanalization of the affected vessels.
 It is a non-atherosclerotic inflammatory disease affecting
small and medium sized arteries and veins of upper and
lower extremities.
 The etiology of thromboangiitis obliterans is unknown,
but use or exposure to tobacco is central to the initiation
and progression of the disease.

24. MORPHOLOGY
 focal acute and chronic vasculitis of small-
and medium-sized arteries,predominantly of
the extremities.
 Acute and chronic inflammation, accompanied
by luminal thrombosis. The thrombus can
contain small microabscesses composed of
neutrophils surrounded by granulomatous
inflammation

25. Clinical Features
 Early manifestations include cold induced Raynaud
phenomenon (see later), leg pain induced by exercise
that is relieved on rest (intermittent claudication), instep
foot pain induced by exercise (instep claudication), and
a superficial nodular phlebitis.
 Chronic extremity ulcerations develop, progressing over
time to frank gangrene. Smoking abstinence in the early
stages of the disease can often ameliorate further
attacks; however, once established, the vascular lesions
typically do not respond to smoking abstinence.

26. Infectious Vasculitis
 Arteritis can be caused by the direct invasion
of infectious agents, usually bacteria
(Pseudomonas being the classic example) or
fungi, in particular Aspergillus and Mucor
species.
 Vascular invasion can be part of a localized
tissue infection (e.g., bacterial pneumonia or
adjacent to abscesses).

27.  Vascular infections can weaken arterial walls
and culminate in mycotic aneurysms (see
earlier), or can induce thrombosis and
infarction.

28. References:
 Nelson’s Pediatrics
 Harrison’s Internal Medicine
 Google search for pictures
 AAP Board PREP
 CMDT 2004