Systemic Vasculitis - University of Pretoria · Systemic Vasculitis Dr. Christa Visser MBChB MMed...

Systemic Vasculitis

Dr. Christa VisserMBChB MMed (Med Phys)

Diploma in Orthopaedic MedicineMember: Society of Orthopaedic Medicine, UK

XR

Cartilage

Primary: DIPPIP,hip,kneespine, 1 MTPSecondary:

OA

Synovial fluidXR

Urate level

CartilageSynoviumSoft tissue

Bone

GoutPseudogout

Crystal

RFXR

SynoviumJoints + C-spine

>> Systemic

RA

XRHLA B27

SynoviumEntheses

Axial + peripheraljoints>> systemic

ASPsoriatic

EnteropathicReactive

SpA

ANFENA

Organ Fx tests

SynoviumSystemic >>Synovium

SLEPSS

PM/DMMCTD

CTD

ANCAHistologyImaging

SynoviumSystemic >>Synovium

PAN,WegenersTakayasu,GCA etc

Vasculitis

Arthritis

Takayasu’s arteritis

• Takayasu’s arteritis is a chronic inflammatory disorder of unknown etiologyprimarily affecting the aorta and its major branches.

• Occurs most commonly in females under 40 years of age.

Takayasu’s arteritis: clinical features

• Systemic phase: malaise, fever, night sweats and fatigue.

• Occlusive phase: upper limb claudication, headaches, postural dizziness and visual disturbances.

• Reduced or absent upper limb pulses.• Arterial bruits over the carotid, abdominal

and subclavian vessels.

Aortic arch arteriogram in a patient with Takayasu’s arteritis. Smooth taperedstenosis of bilateral common carotid arteries (upper arrows) and of the right subclavian artery (lower arrow) can be seen. There is poststenoticdilatation beyond the left common carotid narrowed segment. Total involvement is seen along the length of the right common carotid artery.

Giant Cell Arteritis

• A vasculitis of unknown etiology occurring primarily in the elderly. Other terms commonly used include temporal arteritis, cranial arteritis and granulomatous arteritis.

• Early recognition and treatment can prevent blindness and other complications due to occlusion or rupture of involved arteries.

Giant Cell Arteritis: Clinical Features

• There are a wide range of symptoms, but most patients have clinical findings related to involved arteries.

• Frequent features: fatigue, headaches, jawclaudication, loss of vision, scalp tenderness,polymyalgia rheumatica and aortic arch syndrome.

• Unlike other forms of vasculitis, giant cell arteritisrarely involves the skin, kidneys and lungs.

• The ESR is usually highly elevated but may be normal or only slightly increased in up to 2% of patients with active disease.

Photograph of dilated temporal arteries in a patient with giant cellarteritis.

A fundus photograph showing optic atrophy secondary to giant cellarteritis.

Polymyalgia Rheumatica

• A clinical syndrome of the middle aged and elderly characterized by pain and stiffness in the neck, shoulder and pelvic girdles, often accompanied by constitutional symptoms.

• The clinical response to small doses ofcorticosteroids can be dramatic.

Polymyalgia Rheumatica: Clinical features

• The musculoskeletal symptoms are usually bilateral and symmetrical.

• Stiffness is usually the predominant feature; it is particularly severe after rest and may prevent the patient getting out of bed in the morning.

• Muscular pain is often diffuse and is accentuated by movement; pain at night is common.

• Muscle strength is unimpaired although the pain makes interpretation of muscle testing difficult.

Polymyalgia Rheumatica: Clinical features 2

• Systemic features include low-grade fever, fatigue, weight loss and an elevated ESR.

• Corticosteroid treatment is usually required for at least 2 years. Most patients should be able to stop taking corticosteroids after 4–5 years.

Polyarteritis Nodosa

• Small and medium-sized artery inflammation involving the skin, kidney, peripheral nerves, muscle and gut.

• Involvement of other organs is rare.

Polyarteritis Nodosa: Clinical Features

• Constitutional symptoms – fever, anorexia, weight loss.

• Skin involvement – palpable purpura, infarctions,livedo reticularis.

• Arthralgia and arthritis. • Peripheral neuropathy. • Renal involvement – red blood cells and red blood

cell casts, proteinuria, renal insufficiency. • Gut involvement – abdominal pain, liver function

abnormalities.

Palpable purpura.

Digital tip infarctions.

Polyarteritis involving the gallbladder artery showing pleomorphicinflammatory cell infiltration andfibrinoid necrosis.

Visceral angiogram inpolyarteritis showing areas of segmental narrowing andaneuryms.

Wegener’s Granulomatosis

• A multisystem disease of unknown etiology with the following clinicopathologic complex:

• Necrosis, granuloma formation and vasculitis of the upper and lower respiratory tracts.

• Glomerulonephritis developing in 75% of patients.• Variable degrees of small and occasionally

medium-sized vessel vasculitis.

Wegener’s Granulomatosis: Clinical features

• Inflammatory destructive lesions often affect the eyes, ears, nose, throat, lungs, and kidneys. Although musculoskeletal features are common, they tend to be mild and not destructive. About 25% of cases may have peripheral or central nervous system disease.

• The greatest morbidity results from airway, renal, auditory and ocular disease.

• Immunosuppressive therapy may be very effective, but may be associated with serious complications.

Chronic bilateral maxillary sinusitis. This has required several surgical procedures to remove abnormal tissue and facilitate drainage ofmucopurulentsecretions. Bacterialsuperinfection is a common sequelae.

Wegener’s granulomatosis: lung disease. The lung disease may be unilateral or bilateral, and consist of infiltrates, nodules and/or hemorrhage.

Ocular abnormalities in Wegener’s granulomatosis. Retro-orbital pseudotumorhas resulted inproptosis, the position of the left eye being fixed and unable to deviate in conjunction with the right eye.

Nasal deformity inWegener’s granulomatosis. This has resulted from chronic inflammation and destruction of nasal cartilage.

Behçet’s Syndrome

• A systemic vasculitis of unknown cause with mucocutaneous and frequent ocular and musculoskeletal involvement.

• A marked geographic distribution is characterized by highest prevalence in Turkey, Iran and Japan.

Behçet’s Syndrome: Clinical features

• Recurrent oral and/or genital aphthous ulceration.• Chronic relapsing uveitis leading to blindness in

10% of all cases.• A variety of skin manifestations, including the

‘pathergy’ phenomenon.• Musculoskeletal, neurologic, major artery and

vein involvement.• An undulating course that generally abates in

intensity with the passage of time.

Genital ulcerations that are typically on the scrotum and leave scars.

Hypopyon uveitis. A precipitate of white cells is formed in the anterior chamber of the eye.

Takayasu’s arteritis

• Takayasu’s arteritis is a chronic inflammatory disorder of unknown etiologyprimarily affecting the aorta and its major branches.

• Occurs most commonly in females under 40 years of age.

Takayasu’s arteritis: clinical features

• Systemic phase: malaise, fever, night sweats and fatigue.

• Occlusive phase: upper limb claudication, headaches, postural dizziness and visual disturbances.

• Reduced or absent upper limb pulses.• Arterial bruits over the carotid, abdominal

and subclavian vessels.

Aortic arch arteriogram in a patient with Takayasu’s arteritis. Smooth taperedstenosis of bilateral common carotid arteries (upper arrows) and of the right subclavian artery (lower arrow) can be seen. There is poststenoticdilatation beyond the left common carotid narrowed segment. Total involvement is seen along the length of the right common carotid artery.

Kawasaki Disease

• An acute febrile eruptive disease occurring most commonly in infants and children under 5 years of age.

• Vasculitis, especially involving coronary arteries, is a serious complication.

Kawasaki Disease: Clinical Features

• Fever of unknown etiology lasting 5 days or more.• Bilateral conjunctival congestion.• Dry and red lips, reddening of oral cavity.• Acute nonpurulent swelling of the cervical lymph

nodes.• Polymorphous exanthema of the trunk without

vesicles or crusts.• Red palms and soles.

Bleeding and crust formation on the lips and cervical lymphadenopathy in Kawasaki disease.

Polymorphous exanthema on the limbs and trunk of an infant with Kawasaki disease.

Membranous desquamation of the fingertips.

Coronary angiography. Leftcoronary artery aneurysm.