Vasculitis resident survival guide

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. The extent and severity of vasculitides may vary from self-limited cutaneous vasculitis to severe fatal systemic vasculitides.

Causes

Vasculitides are categorized primarily by vessel size, together with etiology, pathogenesis, pathology, demographics, and clinical features

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.

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

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.
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.
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 the 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, defined as small intraparenchymal arteries, arterioles, capillaries, and venules. Medium arteries and veins may be affected.
ANCA-associated vasculitis:Necrotizing vasculitis, with few or no immune deposits, predominantly affecting small vessels (ie, capillaries, venules, arterioles, and small arteries), associated with myeloperoxidase (MPO) ANCA or proteinase 3 (PR3) ANCA. Not all patients have ANCA. Add a prefix indicating ANCA reactivity, eg, MPO-ANCA, PR3-ANCA, ANCA-negative. Microscopic polyangiitis: Necrotizing vasculitis, with few or no immune deposits, predominantly affecting small vessels (ie, capillaries, venules, or arterioles). Necrotizing arteritis involving small and medium arteries may be present. Necrotizing glomerulonephritis is very common. Pulmonary capillaritis often occurs. Granulomatous inflammation is absent. Granulomatosis with polyangiitis (Wegener's): Necrotizing granulomatous inflammation usually involving the upper and lower respiratory tract, and necrotizing vasculitis affecting predominantly small to medium vessels (eg, capillaries, venules, arterioles, arteries and veins). Necrotizing glomerulonephritis is common. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): 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 (ie, capillaries, venules, arterioles, and small arteries). Glomerulonephritis is frequent. 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. Cryoglobulinemic vasculitis: Vasculitis with cryoglobulin immune deposits affecting small vessels (predominantly capillaries, venules, or arterioles) and associated with serum cryoglobulins. Skin, glomeruli, and peripheral nerves are often involved. IgA vasculitis (Henoch-Schönlein): Vasculitis, with IgA1-dominant immune deposits, affecting small vessels (predominantly capillaries, venules, or arterioles). Often involves the skin and gastrointestinal tract, and frequently causes arthritis. Glomerulonephritis indistinguishable from IgA nephropathy may occur. Hypocomplementemic urticarial vasculitis (anti-C1q vasculitis): Vasculitis accompanied by urticaria and hypocomplementemia affecting small vessels (ie, capillaries, venules, or arterioles), 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 that can affect vessels of any size (small, medium, and large) and type (arteries, veins, and capillaries).
Behçet's syndrome: Vasculitis occurring in patients with Behçet's syndrome that can affect arteries or veins. 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's syndrome: Vasculitis occurring in patients with Cogan's 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 a systemic vasculitis. The involved organ and vessel type should be included in the name (eg, cutaneous small-vessel vasculitis, testicular arteritis, central nervous system vasculitis). Vasculitis distribution may be unifocal or multifocal (diffuse) within an organ. Some patients originally diagnosed as having single-organ vasculitis will develop additional disease manifestations that warrant redefining the case as one of the systemic vasculitides (eg, cutaneous arteritis later becoming systemic polyarteritis nodosa, etc).
Cutaneous leukocytoclastic angiitis
Cutaneous arteritis
Primary central nervous system vasculitis
Isolated aortitis
Vasculitis associated with systemic disease: Vasculitis that is associated with and may be secondary to (caused by) a systemic disease. The name (diagnosis) should have a prefix term specifying the systemic disease (eg, rheumatoid vasculitis, lupus vasculitis, etc).
Lupus vasculitis
Rheumatoid vasculitis
Sarcoid vasculitis
Others
Vasculitis associated with probable etiology: Vasculitis that is associated with a probable specific etiology. The name (diagnosis) should have a prefix term specifying the association (eg, hydralazine-associated microscopic polyangiitis, hepatitis B virus-associated vasculitis, hepatitis C virus-associated cryoglobulinemic vasculitis, etc).
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

Shown below is an algorithm 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:

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) , The European League Against Rheumatism (EULAR) and European Renal Association – European Dialysis and Transplant Association (ERA-EDTA) and Canadian Vasculitis Research Network (CanVasc) guidelines.

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

Disease control on Drug "Remission"

Disease control on Drug "Remission"

Switch to Azathioprine or MTX & Taper Glucocorticoids

Continue on RTX and Taper Glucocorticoids

Taper Aza or MTX

Stop RTX

Do's

• The content in this section is in bullet points.

Don'ts

• The content in this section is in bullet points.

References

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