Vasculitis

Vasculitis Pathology Video

Vasculitis is inflammation of the blood vessel wall.

The arterial wall is made of three layers which include:

• Tunica intima (endothelial intima)
• Tunica media (smooth muscle)
• Adventitia (connective tissue adventitia)

Most instances of vasculitis have an unknown etiology.

Most instances of vasculitis are not contagious.

Clinical features associated with vasculitis include:

• Fever
• Fatigue
• Muscle pain (myalgia)
• Weight loss

Some vasculitides may cause organ ischemia due to thrombosis or luminal constriction of inflammatory vessels.

Vasculitis is classified into:

• Large vessel vasculitis
• Medium vessel vasculitis
• Small vessel vasculitis

The aorta and its major branches are affected by large vessel vasculitis.

Muscular arteries that supply organs are involved in medium vessel vasculitis.

Arterioles, capillaries, and venules are all involved in small vessel vasculitis.

Large Vessel Vasculitis

Temporal (Giant Cell) Arteritis

Temporal (giant cell) arteritis is a granulomatous vasculitis that typically affects branches of the carotid artery (i.e. the temporal artery).

Temporal (giant cell) arteritis affects females more than males.

Temporal (giant cell) arteritis usually affects individuals that are considered elderly.

Symptoms include of temporal (giant cell) arteritis classically include:

• Headache
• Jaw claudication
• Vision problems (ophthalmic artery involvement)
• Frequent flu-like symptoms
• Joint pain
• Muscle pain

Lab findings of temporal (giant cell) arteritis include:

• Elevated erythrocyte sedimentation rate (ESR)

Temporal (giant cell) arteritis lesions are segmental, and long segments of vessels must be biopsied for the diagnosis.

Temporal (giant cell) arteritis is not excluded by a negative biopsy result.

Histologic assessment of the temporal artery of a patient with temporal (giant cell) arteritis classically shows an inflammatory artery wall with giant cells and intimal fibrosis.

Temporal (giant cell) arteritis is treated with corticosteroids.

Blindness is a risk factor of temporal (giant cell) arteritis, especially if it is not treated.

Takayasu Arteritis

Aortic arch branch points are typically affected by granulomatous vasculitis in Takayasu arteritis.

Takayasu arteritis is also known as pulseless disease.

Takayasu arteritis is a condition that typically affects young Asian female adults.

Symptoms of Takayasu arteritis includes:

• Visual issues
• Neurological symptoms
• Weak or nonexistent pulse in the upper extremities

Lab findings of Takayasu arteritis include:

• Elevated erythrocyte sedimentation rate (ESR)

Takayasu arteritis is treated by corticosteroids.

Medium Vessel Vasculitis

Polyarteritis Nodosa

Polyarteritis nodosa is a multisystemic disorder characterized by widespread inflammation, small and medium vessel damage.

Organs commonly involved by polyarteritis nodosa include:

• Skeletal muscles
• Nervous systems
• Gastrointestinal system
• Kidneys
• Cardiovascular system

The lungs are typically spared in polyarteritis nodosa.

Symptoms of polyarteritis nodosa typically include:

• Hypertension due to involvement of the renal arteries
• Stomach pain with melena due to involvement of the mesenteric vessels
• Neurologic abnormalities
• Skin lesions

Polyarteritis nodosa is associated with serum HBsAg.

Histopathology of polyarteritis nodosa shows transmural inflammation and fibrinoid necrosis early on.

Fibrosis may present later on as a ‘string-of-pearls’ appearance on imaging.

Polyarteritis nodosa is treated with corticosteroids and cyclophosphamide.

Polyarteritis nodosa is lethal if not treated.

Kawasaki Disease

Kawasaki disease causes vascular inflammation of medium sized vessels.

Kawasaki disease typically affects children of Asian descent.

Symptoms of Kawasaki disease include:

• Fever
• Conjunctivitis
• Palmar rash
• Rash on soles
• Lymphadenopathy

In Kawasaki disease the coronary artery is usually involved, and increases the risk of myocardial infarction with thrombosis and aneurysm rupture.

Kawasaki disease is treated by aspirin and IVIg.

Most children with Kawasaki disease completely recover.

Buerger’s Disease

Buerger’s disease is a type of vasculitis that causes necrosis of the fingers and toes.

Symptoms of Buerger’s disease include:

• Auto-amputation of the digits (fingers and toes)
• Gangrene of the digits
• Ulceration of the digits

Raynaud phenomenon is usually present in Buerger’s disease.

Buerger’s disease is highly related to heavy smoking.

There is no cure for Buerger’s disease.

Buerger’s disease progression is halted by smoking cessation.

Small Vessel Vasculitis

Wegener Granulomatosis

Wegener granulomatosis is a necrotizing granulomatous vasculitis that affects the kidneys, lungs, and nasopharynx.

Wegener granulomatosis is a middle-aged man with:

• Sinusitis
• Nasopharyngeal ulcer
• Hemoptysis
• Bilateral nodular lung infiltrates
• Hematuria
• Glomerulonephritis

Wegener granulomatosis disease activity is correlated with serum c-ANCA concentrations.

The histology of vessels affected by Wegener granulomatosis show large necrotizing granulomas and nearby necrotizing vasculitis.

Wegener granulomatosis is treated with cyclophosphamide and steroids.

Relapses are common for Wegener granulomatosis.

Microscopic Polyangiitis (MPA)

Microscopic polyangiitis (MPA) is a necrotizing vasculitis that affects many organs, including the kidney and lung.

Although there is no nasopharyngeal involvement and no granulomas in microscopic polyangiitis (MPA), the presentation is similar to Wegener granulomatosis.

Microscopic polyangiitis (MPA) disease activity is correlated with serum levels of p-ANCA.

Microscopic polyangiitis (MPA) is treated by corticosteroids and cyclophosphamide.

Microscopic polyangiitis (MPA) is prone to relapses.

Churg-Strauss Syndrome

In Churg-Strauss syndrome multiple organs are affected by a necrotizing, granulomatous inflammation with eosinophils, particularly the heart and lungs.

Churg-Strauss syndrome is also associated with peripheral eosinophilia and asthma.

Churg-Strauss syndrome disease activity is correlated with serum p-ANCA concentrations.

Henoch-Schönlein Purpura

Henoch-Schönlein purpura (HSP) is a vasculitis caused by the deposition of the lgA immune complex.

Henoch-Schönlein purpura (HSP) is the most common vasculitis in the pediatric population.

Symptoms of Henoch-Schönlein purpura (HSP) include:

• Hematuria
• GI pain
• Bleeding
• A characteristic rash described as palpable purpura on the buttocks and legs

Henoch-Schönlein purpura (HSP) happens frequently after an upper respiratory tract infection.

Henoch-Schönlein purpura (HSP) illness is self-limited but may return.

If severe Henoch-Schönlein purpura (HSP) is treated with corticosteroids.