Vasculitis

Dr. Shwan Kader Media

Consultant Rheumatologist and Medical
Rehabilitation, sports
medicine.

07.11-2020
 Vasculitis

Inflammation of
blood vessel
walls (artery, vein
and capillary).
Vasculitis
• Is inflammation of blood vessels ( arteries, Veins and capillaries). It causes
changes in the blood vessel walls, including thickening, weakening (note:
fragile), narrowing (note: and thus causing distal ischemia) or scarring. These
changes can restrict blood flow, resulting in organ and tissue damage (note:
and potentially necrosis).
• There are many types of vasculitis, and most of them are rare. Vasculitis
might affect just one organ, or several organs of the body.
• May affect anyone (children, adult, female and male), may disappear without
treatment or lasts for long time with multiple organ affection like kidneys, lungs,
brain or eyes, nerves and skin.
• Note: hence, the severity of vasculitis can range from self limiting to multiorgan
failure and fatality
• Note: since vasculitis can affect many different organs, it can present to various
specialties.
• Note: there are two types of symptoms in this inflammatory condition: systemic
symptoms of inflammation and local symptoms depending on the vessels
affected.
• Note: following COVID-19, the incidence of vasculitides has increased,
especially in children (Kawasaki disease, which was previously rare) [Dr.
Shwan]
Types of Vasculitis
• Behcet's disease
• Giant cell arteritis
• Takayasu's arteritis
• Buerger's disease
• Churg-Strauss syndrome
• Cryoglobulinemia
• Granulomatosis with polyangiitis (previously called Wegner’s granulomatosis)
• Note: Initial symptoms usually occur in the upper respiratory tract and resemble those
associated with a severe common cold, including a persistent runny nose (rhinorrhea),
nasal crusting and nasal obstruction or congestion. More serious symptoms include
nosebleeds, ulcerations of the mucous membranes in the nose with secondary bacterial
infection, sinus pain, inflammation of the sinuses (paranasal sinusitis), and hoarseness.
They thus tend to present to ENT specialists and the condition may be misdiagnosed as an
infection which does not respond to antibiotics[1].
• Henoch-Schonlein purpura
• Kawasaki disease (note: now more common following COVID-19)
Note: polymyalgia rheumatica

• Polymyalgia rheumatica is not included on this list of types of

vasculitis; it's always mentioned with the vasculitides but it's not a
vasculitis itself, though PMR is closely linked with giant cell arteritis
(GCA)
• 50% of GCA patients have polymyalgic symptoms
• About 10% of people with polymyalgia rheumatica have giant cell
arteritis
 Skin and joints

• the most classic of which is “palpable purpura” –purplish–red spots,

usually found on the legs.
Joints as arthritis or arthralgia.
 Lungs:
Granulomatosis with Polyangiitis.

l Inthis image of a CAT scan of the

lungs of a 73 year–old woman
complaining for shortness of breath,
and bloody sputum. The patient
also had glomerulonephritis, a
positive P–ANCA.
l The diagnosis of microscopic
polyangiitis was made.
Kidnys: Glomerulonephritis.
• Note: vasculitis, particularly granulomatosis with polyangiitis, can
affect the kidneys. A urinalysis should be done.
• Red blood cells (usually invisible to the naked eye), clumps of red
blood cells (known as “casts”, also invisible to the naked eye), and
loss of protein in the urine.
• May lead to renal insufficiency and the requirement of dialysis.
Eyes:
Behcet’s disease, GCA and Lupus.
• May affect either blood vessels to
the eyes, causing the sudden loss
of vision, or small blood vessels
within the eyes, leading to retinal
problems
• Inflammation within the eye’s
different chambers, Uveitis and
conjunctivitis.
• This picture is an example of retinal
vasculitis in a patient with systemic
lupus erythematosus (SLE).
Brain: Cerebral vasculitis (note: e.g.,
Behcet’s disease. An MRI is indicated)
• Headaches, strokes, changes in mental
status, difficulty with coordination.
• This (MRI) imaging study of the brain in
central nervous system vasculitis
demonstrates an intra–cerebral
hemorrhage (bright area).
Nerves

• Vascular neuropathy which is inflammation of blood vessels in the

brain, spinal cord, and peripheral nerves
• Numbness and pains in the arms and legs.
• Asymmetrical weakness (weakness that involves one side of the body
more than the other).
• Note: vasculitis can affect nerves directly (neuritis) or indirectly by
causing compression/entrapment (?? Dr. Shwan) (in GPA, for
example, perineural vasculitis or direct compression on nerves by
granulomatous lesions can cause cranial and peripheral neuropathies
[1]
)
 Abdominal: (Mesenteric Arteritis) PAN.

Abdominal pain,
bloody diarrhea,
perforation of the
intestines.
 Other symptoms:

l Sinus, Nose & Ears l Buerger’s disease

l Chronic sinus congestion and l involves
the fingers and
“infections” that persist for longer (toes).
than they should; hearing loss; Gangrene can result from a
inflammation of the nasal septum profound lack of blood flow.
(saddle nose)
Causes of vasculitis:
l The exact cause of vasculitis isn't fully understood.
l A person's genetic makeup and immune systems.

Note: an underlying cause is not always found.

1. Infections, such as hepatitis B and hepatitis C
2. Blood cancers
[Link] Immune diseases, such as rheumatoid
arthritis, lupus and scleroderma
4. Reactions to certain drugs
Differential diagnosis:

• Vasculitis mimic diseases

• viral and bacterial infections (note: suspect vasculitis if no response

to treatment)
• various malignancies
• autoimmune disorders, all of which may have similar clinical
presentation
• Drug-related disorders (note: allergy)
Diagnosis:

• Taking medical history and performing a physical exam.

• Blood tests. level of CRP, ESR and CBC, specific tests such as ANCA (anti-
neutrophil cytoplasmic antibodies)
• Urine tests. Urine may contains red blood cells or has too much protein.
• Imaging tests. Noninvasive imaging techniques can help determine what
blood vessels and organs are affected and monitoring the response to
treatment.
• Imaging includes doppler ultrasound, computerized tomography angiography
(CTA), magnetic resonance imaging (MRI) and positron emission tomography
(PET).
• Note: Transcranial Doppler (TCD) ultrasound provides rapid, noninvasive, real-time
measures of cerebrovascular function (intracranial vessels)
• Biopsy. By taking a small sample of tissue from the affected organ (e.g., kidney,
bowel, temporal artery, etc.) for histopathological study.
• Note: you may need to start treatment even before biopsy results come back in some
conditions such as GCA as the condition can be organ/life-threatening.
Principles of treatment:

• depending on the cause and severity

• Treatment focuses on controlling the inflammation with medications
and resolving any underlying disease that triggered vasculitis.
• Two treatment phases:
1. first stopping the inflammation (note: induction therapy) and then
2. preventing relapse (maintenance therapy).
• Both phases involve prescription drugs.
• Steroid, t-DMARDs (t= traditional), b-DMARDs (b = biological;
monoclonal antibodies) (note: usually combined with other agents/t-
DMARDs[1]; it’s added to them, doesn’t replace them),
cyclophosphamide and others.
Vasculitis

l GiantCell Arteritis
l Polymyalgia rheumatica l
Takayasu’s Arteritis and l
Behcet’s disease
 Giant Cell Arterietis (GCA) &
Takayasus Arterietis (TA)

GCA & TA
l Both of the diseases are
prototypes of large vessel
vasculitis, tending to involve
the aorta and its branches.
l GCA predominantly affects
aorta and often extracranial
arteries of the head.
Vasculitis

• Both GCA and TA are associated with granulomatous inflammation

within the blood vessel wall (note: The histological features of TAK
and GCA are often almost indistinguishable[1])
• In both GCA and TA, clinical symptoms of vascular inflammation and
vascular insufficiency are usually accompanied or preceded by a
systemic inflammatory process.
 Giant Cell Arteritis (GCA)

l Occursexclusively among individuals who are

50 years of age or older.

l Themean age at diagnosis onset is

approximately 72.
GCA

l Visual loss is the most feared complication of

GCA .
l Visual loss may occur through the syndrome of
ischemic anterior optic neuropathy, caused by
narrowing of the posterior ciliary artery and
other vessels to the eye.
Clinical Features of GCA

Presents with two major symptomatic complexes

1- Signs of vascular insufficiency resulting from

impaired blood flow and
2- Signs of systemic inflammation.
Clinical Features GCA

• Local symptoms with severe unilateral headache, loss of vision.

Note: GCA vs migraine
• Biopsy
• Features of aura in migraine but not GCA
• Temporal artery tenderness in GCA
 In a patient with a background of migraine, is their current headache the
same as their previous ones (another attack of migraine) or different (GCA)?
• Note: Jaw claudication refers to pain or discomfort in the jaw that
typically occurs while chewing. It is a classic symptom of giant cell
arteritis (GCA)
• A generalized inflammatory syndrome with fever, night sweating,
malaise, anorexia, weight loss and diffuse muscle pain.
GCA diagnosis
• History
• Physical examination
• Doppler US of temporal artery (note: nowadays, it can be used to more easily diagnose GCA
[Dr. Shwan]; It may be possible for color Doppler ultrasound (CDUS), if performed by
experienced operators, to substitute for temporal artery biopsy as a diagnostic procedure in
the appropriate clinical setting[1])
• Temporal artery biopsy
• The diagnostic procedure of choice is the histological verification with the superficial
temporal artery biopsy.
• Note: Temporal artery biopsy (TAB) is useful in assisting with giant cell arteritis (GCA) diagnosis but
lacks sensitivity[1]; A positive biopsy result is proof of GCA, but a negative result does not rule GCA out.
In 10% to 25% of cases the biopsy result is a false negative. This may be because the biopsy sampled a
non-inflamed segment (Dr. Shwan: this goes for other vasculitides as well) (extra: or because it was
carried out too long after the start of treatment).
• Note: As a rule, however, GCA can be demonstrated by temporal artery biopsy up to at least 14 days
after the start of treatment, and therefore steroid therapy, if indicated, should not be delayed until
after planned biopsy or imaging[1] (Dr. Shwan: a biopsy taken for up 2 weeks after starting steroids may
still be positive and you can take a biopsy even after you've started steroid treatment)
Treatment of GCA.

Note: corticosteroids[1] and DMARDs (?) are the cornerstone of treatment

• Glucocorticoids 0.8 to 1mg/kg/day (note: hence large doses are used [Dr. Shwan])
orally are effective in suppressing the inflammations.
• Since the introduction of glucocorticoids, the rate of GCA-related blindness has
reduced.
• Glucocorticoids induce relief within 2-3 days (note: if no response in 72 hours to
steroids, then the dose may be inadequate[1] or the diagnosis may be wrong [?? Dr.
Shawn])
• Note – Dr. Shwan: steroids are continued for 2 weeks as inflammation may not have subsided
yet, and then the dose is tapered afterwards
• (extra) dose reduction should be started after 1-2 weeks under careful monitoring of ESR and CRP. A dose of 15
mg must be reached within 2 months. Thereafter, the prednisone dose may be reduced with 2.5 mg/day every
month until a maintenance dose of 5 mg (range: 2.5-7.5 mg) is reached. Maintenance therapy could be stopped
if patients are asymptomatic for 6 months on a maintenance dose of 2.5 mg/day [1].
• DMARDS: Like Methotrexate, Azathioprine (note: effective for vasculitis [Dr. Shwan])
and Biological agents are also effective.
• Prevention of osteoporosis ( Vit.D3 + Ca) (note: since high dose steroids are being
used[1])
Polymyalgia Rheumatica (PMR).

l Painand stiffness in the

muscles of the neck,
shoulder girdle, and
pelvic girdle of at least 4
weeks duration
PMR
• The proximal muscle pain and stiffness are combined with signs of
systemic inflammation manifesting clinically as malaise, weight loss,
sweating, low-grade fever and anemia.
• Elevated ESR, elevated CRP.
• Note: may have features of GCA
• Note – how it differs from polymyositis: polymyositis causes
decreased power in the proximal muscles without (extra: or with)
pain, while PMR causes pain and stiffness in the proximal muscles.
• Note: involvement of the pelvic girdle produces an abnormal gait that
may be mistaken for a neurological condition [?? Dr. Shwan]
Treatment of PMR

• PMR responds dramatically to Prednisolone tablet

10-15 mg daily doses within 72 hours.
• Note: most people require a maintenance dosage
of prednisone for 6 months to 2 years (Dr. Shwan:
rarely) that is gradually tapered to the lowest
effective dose required to control symptoms[1].
• Note: if not associated with GCA, taper as soon as
possible [Dr. Shwan]
• Note: DMARDs (steroid-sparing agents) are
occasionally used (for resistant or relapsing
cases??)
TAKAYASU’S ARTERITIS (TA)
(pulsless disease)
• Note: histopathologically similar to GCA but affects
different arteries.
• TA is vasculitis of the large arteries, specifically the
aorta and its main branches of upper limbs.
• The disease may also affect the coronary and
pulmonary arteries.
• Complete occlusion of upper extremity arteries
results in the loss of palpable pulses.
Epidemiology of TA

• Takayasu’s arteritis is a rare disease of

adolescent girls and young women.
• Note: the disease has most often been
reported in young East Asian women
• The diagnostic criteria include an age of less
than 40 years at disease onset (note: a
patient may be older than 40 but in them TA
started before the age of 40 [Dr. Shwan])
Clinical Features TA

• A generalized inflammatory syndrome with fever, night

sweating, malaise, anorexia, weight loss and diffuse
muscle pain often dominates initial manifestations of TA.
• These symptoms are frequently misdiagnosed as
infection.
• Note: Takayasu arteritis is a rare cause of cardiovascular
morbidity in the Western world. As consequence,
vasculitis may be misdiagnosed and treated as
atherosclerotic cardiovascular disease (Dr. Shwan:
Takayasu cases may be mistaken for IHD and may be
referred to cardiology and may even receive PCI with no
response)
Diagnosis TA

• Medical history taking.

• Physical examination, loss of radial artery pulse.
• Blood tests: high ESR, CRP and CBC.
• Doppler US of affected vessels (note: sufficient for diagnosis)
• Angiography reveals long, smooth tapering of involved vessels, with a
remarkable web of collateral blood vessels in advanced cases.
Treatment of TA

• Most patients have progressive or relapsing/remitting disease and

require long-term immunosuppressive treatment .
• Glucocorticoids are drug of choice for management of this disease at
doses between 0.8 to 1mg/kg body weight daily (note: hence large
doses are used [Dr. Shwan])
• Note – Dr. Shwan: steroids are continued for 2 weeks as inflammation may
not have subsided yet, and then the dose is tapered afterwards.
• Vitamin D3 + Calcium supplements to prevent osteoporosis.
• DMARDs like MTX, Azathioprine, Ciclosporin.
Behcet's disease

• Behcet's disease, is a rare disorder that causes inflammation in

blood vessels.
• Note: Behcet's disease may not be as rare as written in
textbooks; it’s more common here in the middle east than it is in
western countries. The prevalence of Behçet disease is highest
in Middle Eastern and Japanese populations.
• Note: affects young adults
• Leads to numerous symptoms that may initially seem unrelated.
• The signs and symptoms of Behcet's disease which may include mouth
and genital sores, eye inflammation, skin rashes, bowel symptoms and
musculo skeletal symptoms.
• Note: Apthous ulcers (mouth sores) (and genital ulcers?) are often the first
symptom. In some cases, eye abnormalities may be the first symptom of Behçet’s
syndrome (may cause uveitis, unilateral or bilateral, and present to an
ophthalmologist)
• Note: can also affect the kidneys
Diagnosis

• Medical history, local and systemic symptoms.

• Examination: ulcerations, arthritis, eye symptoms and skin lesions.
• Investigations: high ESR, HLA-B51 positive.
• Pathergy test: erythematous induration of the skin (note: The
pathergy test is a nonspecific hypersensitivity skin reaction induced
by needle prick that is performed to look for evidence of this
phenomenon) (Dr. Shwan: introduce distilled water into the skin??)
Treatment

• Colchicine 1mg tablets daily to control inflammation

• Note: colchicine is very effective for Behcet's disease, especially for mouth
and genital ulcers and arthritis [? Dr. Shwan]
• Glucocorticoid (given according to severity)
• DMARDS and biological agents.
• Mouth rinses.
• Eyedrops.
• Skin creams and ointments.
• Ca + Vit D3 supplements (osteoporosis) (note: if steroids are given)
Case 1

• A 26 years old man presented with decreased visual acuity,

headache, oral and genital ulcers
• ESR 50 mm/hr.
• Temp. 37.0

• Investigations? ESR, HLA-B51, ophthalmology referral

• Diagnosis? Behcet's disease
Case 2

• A 75 year old female patient started to complain of occasional jaw

claudication 4 months ago. Started complaining of right side
headache 3 weeks ago and decreased visual acuity since yesterday.
• Palpable and tender temporal artery on right side.
• ESR 85 mm/hr.

• Investigations? CBC, ESR, General urine, ophthalmology referral,

Doppler US, and start treatment immediately
• Diagnosis? GCA
Case 3

• A 52 year old lady presented to you in outpatient clinic with pain and
tenderness in the muscles around shoulders and pelvic girdle for 6
weeks duration.
• No headache or mouth ulceration.
• ESR = 40

• Diagnosis? Polymyalgia rheumatica

• Treatment? Steroids (low dose)
Case 4

• 25 year old female presented with chest pain typical of angina, mild
fever and malaise.
• No pulse of left radial artery.
• ESR 75 mm/hr.
• Normal cardiac enzymes and ECG.
• MRI angio?

• Diagnosis? Takayasu arteritis

• Treatment? See relevant slide