Vasculitis resident survival guide

Vasculitis

▸  Causes

▸  Diagnosis

▸  Treatment

▸  Do's

▸  Don'ts

▸  Back to Top

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Dina Elantably, MD[2]

Synonyms and keywords: Arteritis, Angiitis, Vasculitides (plural)

Overview

Vasculitis is the presence of inflammatory leukocytes in the walls of the blood vessels with reactive damage to mural structures leading to compromise of the lumen with downstream ischemia, necrosis, and bleeding. The exact pathogenesis is unknown, and vasculitis can be primary or secondary to underlying disease.

Causes

Vasculitides are categorized primarily by vessel size, together with etiology, pathogenesis, pathology, demographics, and clinical features The extent and severity of vasculitides may vary from self-limited cutaneous vasculitis to severe fatal vasculitides depending on the degree of systemic involvement.

The 2012 Chapel Hill Consensus Conference (CHCC) classified the vasculitides as follows:

Large Vessel Vasculitis Vasculitis affecting large arteries that include aorta and its major branches.
Takayasu arteritis:Arteritis, often granulomatous, predominantly affecting the aorta and/or its major branches. Onset usually in patients younger than 50 years.
Giant cell arteritis: Arteritis, often granulomatous, usually affecting the aorta and/or its major branches, with a predilection for the branches of the carotid and vertebral arteries. Often involves the temporal artery. Onset usually in patients older than 50 years and often associated with polymyalgia rheumatica.
Medium vessel vasculitis: Vasculitis predominantly affects medium arteries defined as the main visceral arteries and branches. Any size artery may be affected. Inflammatory aneurysms and stenoses are common.
Polyarteritis nodosa:Necrotizing arteritis of medium or small arteries without glomerulonephritis or vasculitis in arterioles, capillaries, or venules, and not associated with antineutrophil cytoplasmic antibodies (ANCAs).
Kawasaki disease:Arteritis associated with mucocutaneous lymph node syndrome and predominantly affecting medium and small arteries. Coronary arteries are often involved. Aorta and large arteries may be involved. Usually occurs in infants and young children.
Small vessel vasculitis Vasculitis predominantly affecting small vessels. Medium arteries and veins may be affected.
ANCA-associated vasculitis: Necrotizing vasculitis, with few or no immune deposits, predominantly affecting small vessels associated with myeloperoxidase (MPO) ANCA or proteinase 3 (PR3) ANCA. Not all patients have ANCA.	1- Microscopic polyangiitis: Necrotizing vasculitis, with few or no immune deposits, predominantly affecting small vessels. Necrotizing arteritis involving small and medium arteries may be present. Necrotizing glomerulonephritis is very common. Pulmonary capillaritis often occurs. Granulomatous inflammation is absent.	2- Granulomatosis with polyangiitis Wegener's Granulomatosis: Necrotizing granulomatous inflammation usually involving the upper and lower respiratory tract, and necrotizing vasculitis affecting predominantly small to medium vessels. Necrotizing glomerulonephritis is common.	3- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome): Eosinophil-rich and necrotizing granulomatous inflammation often involving the respiratory tract, and necrotizing vasculitis predominantly affecting small to medium vessels and associated with asthma and eosinophilia. ANCA is more frequent when glomerulonephritis is present.
Immune complex small-vessel vasculitis: Vasculitis with moderate to marked vessel-wall deposits of immunoglobulin and/or complement components predominantly affecting small vessels. Glomerulonephritis is frequent.	1-Anti-glomerular basement membrane disease: Vasculitis affecting glomerular capillaries, pulmonary capillaries, or both, with GBM deposition of anti-GBM autoantibodies. Lung involvement causes pulmonary hemorrhage, and renal involvement causes glomerulonephritis with necrosis and crescents.	2- Essential cryoglobulinemic vasculitis: Vasculitis with cryoglobulin immune deposits affecting small vessels and associated with serum cryoglobulins. Skin, glomeruli, and peripheral nerves are often involved.	3- Henoch-Schonlein Purpura: Vasculitis, with IgA1-dominant immune deposits, affecting small vessels. Often involves the skin and gastrointestinal tract, and frequently causes arthritis. Glomerulonephritis indistinguishable from IgA nephropathy may occur.	4- Hypocomplementemic urticarial vasculitis (anti-C1q vasculitis): Vasculitis accompanied by urticaria and hypocomplementemia affecting small vessels, and associated with anti-C1q antibodies. Glomerulonephritis, arthritis, obstructive pulmonary disease, and ocular inflammation are common.
Variable-vessel vasculitis Vasculitis with no predominant type of vessel involved can affect vessels of any size (small, medium, and large) and type (arteries, veins, and capillaries).
Behçet's syndrome: Behçet's syndrome is characterized by recurrent oral and/or genital aphthous ulcers accompanied by cutaneous, ocular, articular, gastrointestinal, and/or central nervous system inflammatory lesions. Small-vessel vasculitis, thromboangiitis, thrombosis, arteritis, and arterial aneurysms may occur.
Cogan syndrome: Cogan's syndrome is characterized by ocular inflammatory lesions, including interstitial keratitis, uveitis, and episcleritis, and inner ear disease, including sensorineural hearing loss and vestibular dysfunction. Vasculitic manifestations may include arteritis (affecting small, medium, or large arteries), aortitis, aortic aneurysms, and aortic and mitral valvulitis.
Single-organ vasculitis: Vasculitis in arteries or veins of any size in a single organ that has no features that indicate that it is a limited expression of systemic vasculitis.
Cutaneous leukocytoclastic vasculitis
Cutaneous arteritis
Primary central nervous system vasculitis
Isolated aortitis
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 ANCA-associated vasculitis
Cancer-associated vasculitis
other

Diagnosis

History and clinical findings are non-specific to any type of vasculitis. A high index of clinical suspicion is warranted in considering the diagnosis of vasculitis. Shown below is an step-wise approach summarizing the diagnosis of vasculitis according to the Royal College of Physicians guidelines. The diagnosis of the individual vasculitides is generally based on patterns of organ injury, the size of the vessels affected, histopathological features, and characteristic findings on diagnostic imaging.

History: Previous drug usage, Family history of autoimmune rheumatic disease , Fever, sweats, Weight loss, anorexia, Malaise, fatigue, persistent skin rashes, Cutaneous ulcer, Myalgia, Arthralgia, epistaxis, Sinusitis, Painful red eye, Sight loss, Wrist drop, foot drop, Stroke, Seizure, Headache, Scalp tenderness, Jaw claudication, Asthma, Limb claudication, Abdominal pain, Haematemesis, melaena, haematochezia, Frothy urine, haematuria, Scrotal pain

Clinical Findings: cachexia, pallor, purpuric/petechial rash, digital ulcers or gangrene, arthritis, muscle weakness, nasal crusting, nasal bridge collapse, optic neuritis, uveitis, episcleritis, temporal artery tenderness and nodularity, neurological deficit, peripheral neuropathy, absent peripheral pulses

1-Exclude vasculitis “mimics” and secondary causes: Blood cultures, Echocardiogram, Hepatitis screen (B and C), HIV test, Antiglomerular basement membrane antibody, Antiphospholipid antibodies, Antinuclear antibody

2-Assess extent of vasculitis: Urine dipstick and microscopy (all patients), Chest radiography (all patients), Nerve conduction studies/electromyography/CK

3- Confirm the Diagnosis: Skin Biopsy/temporal artery biopsy and/or angiogram

4- Identify specific cause of primary vasculitis ANCA, Cryoglobulin, Complement levels, Eosinophil counts/IgE levels

Treatment

Treatment regimens are based upon the specific diagnosis and the severity or extent of the disease. The treatment used for specific types of Primary vasculitides is summarized as follows:

Large vessel vasculitis:

Takayasu arteritis:

See treamtment of Takayasu arteritis

Pharmacologic medical therapies include

• Corticosteroids: prednisone 1mg/kg qd
• IL-6 receptor inhibitors: Tocilizumab
• B-cell depletion agents: Rituximab
• Cytotoxic drugs: Azathioprine, Methotrexate, Cyclophosphamide

Giant Cell Arteritis:

See Treatment of Giant Cell Arteritis

Medium vessel vasculitis:

Polyarteritis Nodosa:

See treatment of Polyarteritis Nodosa.

Kawasaki disease:

See treatment of Kawasaki disease.

Small vessel vasculitis:

ANCA-associated vasculitis:

Shown below is an algorithm summarizing the treatment of ANCA associated vasculitis (AAV) according to The British Society for Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) , European League Against Rheumatism (EULAR/ERA-EDTA) recommendations and Canadian Vasculitis Research Network (CanVasc)

The treatment generally goes through 3 phases:

1- Remission induction: Involves the use of medium to high doses of glucocorticoids with the use of other immunosuppressive agents.

2- Remission maintenance: Once remission has been attained, the dose of glucocorticoids is usually steadily lowered, as tolerated.

3- Monitoring: Patients require monitoring for both disease activity and drug toxicity during the active treatment phase and recurrence of vasculitis.

Induction of Remission

Cyclophosphamide + Glucocorticoid

OR Rituximab + Glucocorticoid

Vital organ/life threatening/creatinine>500 mmol/L : Add Plasma exchange

No organ threatening involved: Mycophenolate Mofetyl or Methotrexate with Glucocorticoids

Remission

Remission

Switch to Azathioprine or Methotrexate & Taper Glucocorticoids

Continue on Rituximab and Taper Glucocorticoids

After 2 years : Taper Azathioprine or Methotrexate

After 2 years: Stop Rituximab

Behcet disease

See treatment of Behcet disease

Drug induced vasculitis:

• Discontinuation of the culprit Drug along with avoiding subsequent rechallenge.
• In patients with persistent and/or necrotizing skin lesions or other organ damage, treatment with glucocorticoids may be required.
• Patients may require additional immunosuppressive medications for prolonged disease.

Do's

• Do have a high index of suspicion in considering a diagnosis of vasculitis as clinical symptoms and signs are non-specific.
• Exclude Secondary causes of vasculitis first by proper laboratory and radiological tests.
• Assess other system involvement even if the primary presentation is a single organ affection.
• Determine the specific cause of primary systemic vasculitis as well as the severity to define the proper treatment plan.

Don'ts

• Don't miss to exclude secondary causes of vasculitis as the mainstay treatment is to treat the cause.
• Don't miss to investigate other organ affection such as Renal, pulmonary, and CNS.

References

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