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 categorised primarily by vessel size, together with aetiology, pathogenesis, pathology, demographics and clinical features

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

Large Vessel Vasculitis
Takayasu arteritis
Giant cell arteritis
Medium vessel vasculitis
Polyarteritis nodosa
Kawasaki disease
Small vessel vasculitis
ANCA-associated vasculitis: Microscopic polyangiitis Granulomatosis with polyangiitis (Wegener's) Eosinophilic granulomatosis with polyangiitis (Churg-Strauss).
Immune complex small-vessel vasculitis: Anti-glomerular basement membrane disease Cryoglobulinemic vasculitis IgA vasculitis (Henoch-Schönlein) Hypocomplementemic urticarial vasculitis (anti-C1q vasculitis)
Variable-vessel vasculitis
Behçet's syndrome
Cogan's syndrome
Single-organ vasculitis
Cutaneous leukocytoclastic angiitis
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

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

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

No organ threatening involved: Mycophenolate Mofetyl or Methotrexate

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

Disease control on Drug "Remission"

Switch to Azathioprine or MTX & Taper Glucocorticoid

Disease control on Drug "Remission"

Taper Aza or MTX

Continue on RTX and Taper Glucocorticoids

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Stop RTX

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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