AN INTRODUCTION TO
VASCULITIS
DR NASIR FAROOQ BUTT
ASSISTANT PROFESSOR
DEPARTMENT OF MEDICINE
KING EDWARD MEDICAL UNIVERSITY
MAYO HOSPITAL LAHORE
INTRODUCTION TO VASCULITIS
• DEFINITION
• CAUSES
• CLASSIFICATION
• CLINICAL
PRESENTATION
• CASE SCENARIOS
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
Clinical
• 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”
VASCULITIS
“Angiitis” and “Arteritis” are both
synonyms for vasculitis, literally
meaning “inflammation within blood
vessels” or “inflammation in
arteries.” Because there are so many
types of vasculitis, the group is
sometimes referred to in the
plural: vasculitides.
Causes Of Vasculitis
• Unknown
• Genetic factors (different genes) appear be somewhat
important in the disease
• Autoimmune disease -- body comes under attack by its
own immune system. In vasculitis, the immune system
attacks blood vessels.
• Drugs e.g. penicillin, cephalosporin, sulfonamide, loop
and thiazide-type diuretics, phenytoin and allopurinol.
• Infections e.g. hepatitis C, hepatitis B virus, HIV
• Vasculitis can be a part of other rheumatic diseases,
mainly including systemic lupus erythematosus,
rheumatoid arthritis and Sjögren’s syndrome
CLASSIFICATION OF VASCULITIS
According to the size of the vessel involve:
• Large vessel vasculitis: Vasculitis affecting large
arteries more often than other vasculitides. Large
arteries are the aorta and its major branches. Any size
artery may be affected.
• Medium vessel vasculitis: Vasculitis predominantly
affecting medium arteries defined as the main visceral
arteries and their branches. Any size artery may be
affected
• Small vessel vasculitis: Vasculitis predominantly
affecting small vessels, defined as small
intraparenchymal arteries, arterioles, capillaries, and
venules. Medium arteries and veins may be affected.
Names for vasculitides adopted by the 2012
International Chapel Hill Consensus Conference on the
Nomenclature of Vasculitides
Large vessel vasculitis (LVV)
Takayasu arteritis (TAK)
Giant cell arteritis (GCA)
Medium vessel vasculitis (MVV)
Polyarteritis nodosa (PAN)
Kawasaki disease (KD)
Names for vasculitides adopted by the 2012
International Chapel Hill Consensus Conference on the
Nomenclature of Vasculitides
Small vessel vasculitis (SVV)
• Antineutrophil cytoplasmic antibody (ANCA)–associated
vasculitis (AAV)
 Microscopic polyangiitis (MPA)
 Granulomatosis with polyangiitis (Wegener’s) (GPA)
 Eosinophilic granulomatosis with polyangiitis (ChurgStrauss) (EGPA)
• Immune complex SVV
 Anti-glomerular basement membrane (anti-GBM)
disease
 Cryoglobulinemic vasculitis (CV)
 IgA vasculitis (Henoch-Schönlein) (IgAV)
 Hypocomplementemic urticarial vasculitis (HUV) (antiC1q vasculitis)
Names for vasculitides adopted by the 2012 International
Chapel Hill Consensus Conference on the Nomenclature of
Vasculitides
Variable vessel vasculitis (VVV)
• Behcet’s disease (BD)
• Cogan’s syndrome (CS)
Single-organ vasculitis (SOV)
• Cutaneous leukocytoclastic
angiitis
• Cutaneous arteritis
• Primary central nervous system
vasculitis Isolated aortitis
• Others
Vasculitis associated with systemic
disease
• Lupus vasculitis
• Rheumatoid vasculitis
• Sarcoid vasculitis
• Others
Vasculitis associated with
probable etiology
• Hepatitis C virus–associated
cryoglobulinemic vasculitis
• Hepatitis B virus–associated
vasculitis
• Syphilis-associated aortitis
• Drug-associated immune
complex vasculitis
• Drug-associated ANCAassociated vasculitis
• Cancer-associated vasculitis
• Others
Vasculitis: Primary Or
Secondary?
Primary:
Vasculitis is the principal feature of the
disease
Secondary:
Vasculitis is the complication of other disease
or Toxin (e.g. RA, Infection, Malignancy)
TYPICAL CLINICAL MANIFESTATIONS OF VASCULITIS
Constitutional symptoms:
• Fever
• Weight loss
• Malaise
Arthralgias/arthritis
TYPICAL CLINICAL MANIFESTATIONS OF VASCULITIS
Large Vessel Vasculitis
• Limb claudication (Development and worsening of
fatigue and discomfort in muscles of one or more
extremities while in use, especially the upper extremities)
• Asymmetric blood pressures (Difference of >10
mmHg in systolic blood pressure between arms)
• Absence of pulses
• Bruits (audible on auscultation)
• Aortic dilation
CLAUDICATION
Claudication is defined as a pain or discomfort in a group of muscles, usually
the legs, thighs, or buttocks, that is worsened by exercise (ie, walking) and
relieved with rest.
Types Of Claudication:
VASCULAR
• cramping pains in the buttock or leg muscles, caused by poor circulation
of the blood to the affected area - Peripheral artery disease - DM
• fatigue and discomfort in muscles of one or more extremities while in use,
especially the upper extremities due to Vasculitis
• Jaw claudication is pain in the jaw or ear while chewing. This is caused by
insufficiency of the arteries supplying the jaw muscles, associated with
giant cell arteritis.
NEUROGENIC (SPINAL)
• caused by nerve root compression or stenosis of the spinal canal, usually
from a degenerative spine, most often at the "L4-L5" or "L5-S1" level. This
may result from many factors, including bulging disc, herniated disc,
osteophytes. In most cases neurogenic claudication is bilateral, i.e.
Symmetrical .
TYPICAL CLINICAL MANIFESTATIONS OF VASCULITIS
Medium Vessel Vasculitis
•
•
•
•
•
•
•
Cutaneous nodules
Cutaneous Ulcers
Livedo reticularis
Digital gangrene
Mononeuritis multiplex (wrist/foot drop)
Microaneurysms
Mesenteric ischemia
Healing subcutaneous ulcer over thigh (a)
and leg (b)
Livedo reticularis refers to a condition in which there is mottled
discolouration of the skin. It is described as being reticular (net-like,
lace-like) and cyanotic (reddish blue discolouration). The discolouration
surrounds pale central skin. Livedo reticularis occurs mostly on the legs
but can extend to arms and trunk. It is more pronounced in cold
weather.
Digital gangrene
Mononeuritis multiplex
• Mononeuritis multiplex is the simultaneous malfunction
of two or more peripheral nerves in separate areas of the
body. It causes abnormal sensations and weakness.
• Multiple mononeuropathy typically affects only a few
nerves, often in different areas of the body. In contrast,
polyneuropathy affects many nerves, usually in about the
same areas on both sides of the body. However, if
multiple mononeuropathy involves many nerves, it may
be difficult to distinguish from polyneuropathy.
Mononeuritis multiplex
A MICROANEURYSM is a tiny aneurysm, or swelling, in the
side of a blood vessel. In people with diabetes,
microaneurysms are sometimes found in the retina of the
eye. These miniature aneurysms can rupture and leak blood.
MESENTERIC ISCHEMIA is a medical condition in which injury
of the small intestine occurs due to not enough blood supply. It can
come on suddenly, known as acute mesenteric ischemia, or
gradually, known as chronic mesenteric ischemia.
 Symptoms of acute mesenteric artery ischemia:
Sudden severe abdominal pain
Bloody Diarrhea
Vomiting
 Symptoms of chronic mesenteric artery ischemia:
Abdominal pain 1 to 2 hours after eating
Changes in the frequency of bowel movements
Bloating, nausea and vomiting
TYPICAL CLINICAL MANIFESTATIONS OF VASCULITIS
Small Vessel Vasculitis
• Palpable purpura
• Vesiculobullous lesions
• Urticaria
• Glomerulonephritis
• Alveolar hemorrhage
• Scleritis
• Cutaneous extravascular necrotizing granulomas
• Splinter hemorrhages
• Uveitis
• Episcleritis
Palpable purpura is a condition where purpura, which constitutes
visible non-blanching hemorrhages, are raised and able to be touched
or felt upon palpation.
Vesiculobullous lesions
• A vesicle is a raised hemispherical lesion less than 0.5
cm in diameter,contain clear fluid while a bulla is raised
hemispherical lesion more than 0.5 cm in diameter
contain clear fluid.a bulla can contain pus when it is
termed as pustular bulla.
Urticaria – also known as hives, welts or nettle rash – is a
raised, itchy rash that appears on the skin
Alveolar Hemorrhage
Bleeding into the alveolar spaces of the lungs due to disruption of the
alveolar-capillary basement membrane.
Caused by injury or inflammation of the arterioles, venules, or alveolar
septal (alveolar wall or interstitial) capillaries.
Characterized clinically by the presence of cough, dyspnea, and
hemoptysis.
Glomerulonephritis
It is a disease of the kidney, characterized by inflammation
of the glomeruli.
Glomerulonephritis signs and symptoms may include:
• Pink or cola-colored urine from red blood cells in your
urine (hematuria)
• Foamy urine due to excess protein (proteinuria)
• High blood pressure (hypertension)
• Fluid retention (edema) with swelling evident in your
face, hands, feet and abdomen
• Fatigue from anemia or kidney failure
Scleritis
Scleritis is a disorder in which the sclera becomes severely
inflamed and red. The condition can be very painful.
Symptoms:
• Severe eye pain aggr. with eye movements.
• deep-seated headaches
• double vision
• lacrimation, or excessive tearing
• decreased vision
• photophobia, or sensitivity to light
Cutaneous Extravascular Necrotizing
Granulomas
Splinter hemorrhages (or haemorrhages) are tiny blood clots that
tend to run vertically under the nails.
Uveitis
 Uveitis is the inflammation of the uvea, the pigmented
layer that lies between the inner retina and the outer
fibrous layer composed of the sclera and cornea.
 Acute anterior uveitis presents as follows:
• Pain, generally developing over a few hours or
days except in cases of trauma
• Redness
• Photophobia
• Blurred vision
• Increased lacrimation
 Posterior uveitis presents as follows:
• Blurred vision and floaters
• Absence of symptoms of anterior uveitis (ie, pain,
redness, and photophobia)
Episcleritis
Episcleritis is a benign, self-limiting inflammatory disease
affecting part of the eye called the episclera. The episclera
is a thin layer of tissue that lies between the conjunctiva
and the connective tissue layer that forms the white of the
eye (sclera).
CASE SCENARIO 01
A 25-year-old male cricketer notes that his
arms now ache after bowling two or three
consecutive overs during net practice
sessions, and he is unable to continue. He has
had night sweats and a 10-lb weight loss.
Pulses in the upper extremity are difficult to
palpate. Systolic Blood Pressure Recorded in
Left arm is 20 mm Hg higher than right arm.
What is your diagnosis?
CASE SCENARIO 01
Patient has:
• Limb claudication
• Asymmetric blood pressures (Difference of >10
mmHg in systolic blood pressure between arms)
• Absence of pulses
CASE SCENARIO 01
Answer:
LARGE VESSEL VASCULITIS (LVV)
Takayasu arteritis (TAK)
• Limb claudication
• Asymmetric blood pressures (Difference of >10
mmHg in systolic blood pressure between arms)
• Absence of pulses
• Bruits (audible on auscultation)
• Aortic dilation
CASE SCENARIO 02
A 60-year-old man presents with a 2-month history of
weight loss (approx 5 kg), fever and intermittent
abdominal pain. On examination he has net like rash
on legs, left foot drop and gangrene of tip of left big
toe. His blood Pressure is 150 /105.
He develops peritoneal signs and at laparotomy is
found to have an area of infarcted bowel.
What is your diagnosis?
CASE SCENARIO 02
Patient has:
• Livedo reticularis
• Digital gangrene
• Mononeuritis multiplex (wrist/foot drop)
• Mesenteric ischemia
CASE SCENARIO 02
Answer:
MEDIUM VESSEL VASCULITIS (MVV)
Polyarteritis nodosa (PAN)
•
•
•
•
Livedo reticularis
Digital gangrene
Mononeuritis multiplex (wrist/foot drop)
Mesenteric ischemia
• Microaneurysms
• Cutaneous nodules
• Cutaneous Ulcers
CASE SCENARIO 03
• A 11-year-old boy presented with fever for 5 days. After a day, he
developed an erythematous, nonpruritic rash which progressed
proximally from both feet to thighs and upper extremities including
palms and soles. He is also complaining of pain in knee and ankle
joints.
• On physical examination he has palpable nonblanching purpuric
rash involving both upper and lower extremities with non-pitting
pedal edema. There was no truncal involvement. Nails show splinter
hemorrhages. Two days later, the patient developed abdominal pain
involving the right and left upper quadrant which was constant,
colicky in nature, severe in intensity, aggravated with meals. He is
also c/o breathlessness and hemoptysis.
• Laboratory tests showed WBC: 16,900/microL); Hb: 14 g/dL;; Serum
Creatinine: 2.1 mg/dL; Urinalysis: Blood ++ or protein ++; ESR:
58 mm/Hr;
• What is your diagnosis?
CASE SCENARIO 03
Patient has:
• Palpable purpura
• Glomerulonephritis
• Alveolar hemorrhage
• Splinter hemorrhages
CASE SCENARIO 03
Answer:
SMALL VESSEL VASCULITIS (SVV)
IgA vasculitis (Henoch-Schönlein) (IgAV)
•
•
•
•
Palpable purpura
Glomerulonephritis
Alveolar hemorrhage
Splinter hemorrhages
• Vesiculobullous lesions, Urticaria, Cutaneous
extravascular necrotizing granulomas
• Uveitis, Scleritis, Episcleritis