Pathology

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Lecture (6) :
Vasculitis

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▪VERY IMPORTANT
▪Extra explanation
▪Examples
▪Diseases names: Underlined
▪Definitions *It always seems impossible, until it’s done..
 • Know the common causes of
Objectives :

vasculitis with special

emphasis on the clinic-
pathological features and
mechanism of :
1. Giant cell arteritis.
2. Polyarteritis nodosa.
3. Wegener's granulomatosis.
4. Leukocytoclastic vasculitis.
 * Try to focus on the red text, all of it is important.

INTRODUCTION
Vasculitis : It is the inflammation of vessel walls with many possible
symptoms.
Causes:
1. It is usually immune-mediated :
• Immune complex deposition
• Antineutrophil cytoplasmic antibodies (ANCAs)
• Anti-endothelial cell antibodies
• Autoreactive T cells
2. It also can be caused by infection, physical or chemical injury.

Quick look on Vasculitis:

Vessel Disease Features

>50 years. Arteries of the
Giant-cell arteritis
head.
Large
Female <40. “Pulseless
Takayasu arteritis
disease”
Polyarteritis nodosa Young adults. Widespread.
Medium
<4. Coronary disease.
Kawasaki disease
Lymph nodes.
Wegener granulomatosis Lung, kidney, c-ANCA.
Lung, Eosinophils, Asthma,
Churg-Strauss syndrome
p-ANCA.
Small Microscopic polyangiitis Lung, kidney, p-ANCA.
Idiopathic, infectious,
Cutaneous Leukocytoclastic drugs, chemicals, cancer
vasculitis and systemic disease like
HNP.
GIANT CELL (TEMPORAL) ARTERIES
• Most common type of vasculitis.
• Affect patients more than 50 years of age (only one occurs in
elderly)
• Female to male ratio is 2:1 respectively.
• Chronic, granulomatous inflammation of large to small
arteries, especially in the head, particularly the branches of
the carotid artery (temporal artery (headache) and branches
of the ophthalmic artery (blindness)).
• Involvement is segmental, acute and chronic. by segmental
it means it only affects sections of the arteries rather than
the whole artery
Clinical Features:
v Symptoms :
• Fever. general symptom from inflammatory response
• headache. often most intense along the course of the superficial
temporal artery
• Thickened and painful temporal artery. thickened From the
inflammation and painful from the inflammatory mediators which
cause pain Extra picture:-
• Facial pain or Jaw pain. when the facial artery is involved
• Visual problems and acute vision loss. when the ophthalmic
artery is involved

• more specific symptoms occur when the organ supplied lacks

blood supply due to the inflammation of the artery supplying the
organ
v The diagnosis depends on biopsy and histologic confirmation.
v Treatment : corticosteroids. It weakens the Immune response
Morphology:
that is causing the inflammation v Granulmatous
Mnemonic: Giant old Head = affects Arteries in the head
inflammation of the blood
vessel wall.
v Giant cells.
v Disruption and
fragmentation of internal
elastic lamina.
vThe healed stage reveals
collagenous thickening of
the vessel wall and the
artery is transformed into a
fibrous cord.
v Proliferation of the intima
with associated occlusion of
the lumen.
POLYARTERITIS NODOSA
• Cutaneous only or systemic (can affect any organ).
• Disease of young adults.
• There is segmental necrotizing inflammation of arteries of medium to
small size, in any organ (especially the kidneys and skin) except the lung.
Mnemonic: Polyarteritis Nodosa = Pulmonary Are Not damaged
• Polyarteritis nodosa has been associated with hepatitis B or hepatitis C.

Fever general symptom from inflammatory

response
Results from ischemia and infarction of weight loss, abdominal pain and melena
affected tissues and organs. (bloody stool), muscular pain and neuritis.
All result from the inflammation and necrosis
of the arteries supplying the organ

Clinical Manifestations

Particularly characteristic of PAN is that all

the different stages of activity (i.e. active
Renal arterial involvement is often and chronic stages) may coexist in same
prominent and is a major cause of death. artery or in different vessels at the same
time.

v Fatal if untreated, but steroids and cyclophosphamide are curative.

Steroids are used to inhibit wbc from migrating and causing necrosis
Cyclophosphamide is used to damage the dna of Immune cells that cause the
damage

• 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.
 WEGENER GRANULOMATOSIS (NEW NAME
IS GRANULOMATOSIS WITH POLYANGITIS)

Necrotizing
granulomas of the
upper and lower
respiratory tract.
• Males are affected more (respiratory
syptoms)
often than females, at
an average age of about
40 years
WG is a
• C- ANCAs necrotizing
(antineutrophilic vasculitis
characterized
cytoplasmic antibodies) by the triad of:
is positive in serum of necrotizing or
Renal disease in granulomatous
more than 95% of the form of vasculitis of
necrotizing,
patients. crescentic,
small to
glomerulonephritis. medium-sized
• Persistent pneumonitis, vessels.
chronic sinusitis,
mucosal ulcerations of
the nasopharynx , and
evidence of renal
disease. Wegener
Granulomatosis involves
damage to the Respiratory
tract and Kidney only

• Untreated: fatal - may

lead to death within 2
years if not treated.

Extra picture:- Crescentic

Palatal Palatal Ulceration

Destruction
THROMBOANGIITIS OBLITERANS:
(BUERGER’S DISEASE)
v Distinct disorder that results in severe vascular insufficiency and
gangrene of extremities. It is characterized by areas of inflammation
of medium and small sized arteries. It is associated with thrombosis
and can extend to nearby veins as well as nerves.
v Abstinence of cigarette smoking in early stages of disease brings
relief from further attacks

Extra picture:-
Features: Buerger’s sounds
like Burgers

§ Affects medium/small arteries

§ Hands and legs
§ Associated with heavy smokers. Before age of 35
§ Pain at rest due to nerve damage
§ Pain if affected part induced by exercise (instep claudication)
claudication=‫اﻟﻌرج‬
§ Chronic ulcerations in hand and foot can progress to gangrene

Luminal
thrombosis

Lymphocytic rim (acute

& chronic inflammation)
CUTANEOUS LEUKOCYTOCLASTIC
OR HYPERSENSITIVITY VASCULITIS
v Necrotizing vasculitis that affects capillaries as well as small arterioles
and venules. Vessels of the skin, mucous membranes, lungs, brain,
heart, gastrointestinal tract, kidney and muscle can be involved.

Features:
§ Most common vasculitis seen in clinical practice
§ Inflammation of small blood vessels (usually, post capillary venules in
the dermis)
§ Palpable purpura (purple discolored spots cause by bleeding under skin)
§ Can be cutaneous or systemic disease
§ Effects many organs, most notably the skin
§ Characterized by Leukocytoclasis: karyorrhexis (fragmentation) of
neutrophils in and around the vessels. (leading to purpura)
v Causes:
Ø Idiopathic v Diagnosis:
Ø Immune response towards: skin biopsy
• Drugs: Penicillin v Features:
• Infections : streptococcal Infiltration of vessel wall
• Food products/ toxic with neutrophils which then
chemicals become fragmented
• Tumor antigens in cancer resulting in leukocytoclasia
cells or nuclear dust
Ø Part of systemic disease:
• collagen vascular diseases:
ü Lupus erythematosus
ü Rheumatoid arthritis
• Henoch-Schönlein purpura

Fibrinoid
necrosis

Fragmented
neutrophils
 HENOCH-SCHONLEIN PURPURA (HSP)

• HSP is an IgA-mediated, autoimmune systemic small vessel

leukocytoclastic hypersensitivity vasculitis of childhood. It
causes skin purpura, arthritis, abdominal pain,
gastrointestinal bleeding, orchitis and nephritis.
• The aetiology remains unknown.
• Immunoglobulin A (IgA) and complement component 3 (C3)
are deposited on arterioles, capillaries, and venules.
• Serum levels of IgA are high in HSP.
• Skin biopsy will show necrotizing leukocytoclastic vasculitis
of capillaries in the dermis.

Summary Important!
Cases from Dr. Alhumeidi:

Case 1:
Elderly patient with headache and visual disturbances.
Diagnosis: Giant-cell (temporal) vasculitis
Giant cell vasculitis is almost the only vasculitis that affects the
elderly (old age is a hint)

Case 2:
A young adult that is a heavy smoker presents with pain in his
feet while playing soccer
Diagnosis: thromboangiitis obliterans: buerger’s disease
Smoking and exercise induced pain are hints

Case 3:
A pediatric patient presents with skin lesions, abdominal pain,
hematuria, and blood in stool
Diagnosis: Leukocytoclastic vasculitis
The age of the patient and these clinical manifestations like
skin lesions (pupura) are hints
Q1) What is the most common cause of vasculitis?
A. Immune-mediated
B. Infections
C. Congenital defect
D. Drugs

Q2) Patient came to the hospital having fever and headache, what artery is most
likely thickened or painful?
A. Carotid artery
B. Ophthalmic artery
C. Brachiocephalic artery
D. Temporal artery

Q3) Regarding (Q2), what can be found in the patient’s biopsy?

A. Disruption and fragmentation of internal elastic lamina
B. Segmental inflammation and fibrinoid necrosis
C. Crescentic inflammation of the arteries
D. Leukocytoclasia

Q4) A Patient came to the hospital and the doctor noticed an inflammation in a
medium sized vessels. The patient has fever lost some weight, and he has a
bloody stool. Which of the following organs cannot be affected?
A. Kidney
B. Heart
C. Lung
D. Liver

Q5) A 34 year old man came to the clinic with ulceration of the palate, what is the
most likely diagnosis in this case?
A. Polyarteritis Nodosa
B. Wegener granulomatosis
C. Burger disease
D. Cutaneous leukocytoclastic
Q6) Regarding (Q5), what is the best test to confirm your diagnosis?
A. C-ANCA
B. ECR
C. Blood culture
D. X ray

Q7) Increased neutrophils infiltration and the presence of karyorrhexis of

the neutrophils indicates?
A. Polyarteritis Nodosa
B. Wegener granulomatosis
C. Burger disease
D. Hypersensitivity vasculitis

Q8) In case of Thromboangiitis obliterans, which statement can be true?

A. Smoking is a risk factor
B. Pain without any activity
C. Gangrene
D. All of the above

Answers :
Q1 – A
Q2 – D
Q3 – A
Q4 – C
Q5 – B
Q6 – A
Q7 – D
Q8 – D
 ‫‪Females:‬‬ ‫‪Males:‬‬
‫ﻓﺎطﻣﺔ ﺑﺎﻟﺷرف ‪-Leader :‬‬ ‫ﻣﻧﺻور اﻟﻌﺑرة ‪-Leader :‬‬
‫رﯾﻨﺎد اﻟﻐﺮﯾﺒﻲ‬ ‫ﺧﺎﻟد اﻟﻌﻘﯾﻠﻲ‬
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‫ﻧورة اﻟﻘﺎﺿﻲ‬ ‫أﺣﻣد اﻟﺻﺑﻲ‬
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GOOD LUCK ! J

*references:
- Robbins Basic Pathology

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