Vasculitis resident survival guide

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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[1]:
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.[2] 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) [3], The European League Against Rheumatism (EULAR) and European Renal Association – European Dialysis and Transplant Association (ERA-EDTA)[4] and Canadian Vasculitis Research Network (CanVasc) [5]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
 
 
{{{ }}}
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
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

  1. Jennette JC, Falk RJ, Bacon PA et al (2013) 2012 Revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum 65:1–11. https://doi.org/10.1002/art.37715
  2. An update on the general management approach to common vasculitides Mooikhin Hng, Sizheng S Zhao, Robert J Moots Clinical Medicine Nov 2020, 20 (6) 572-579; DOI: 10.7861/clinmed.2020-0747
  3. Ntatsaki E, Carruthers D, Chakravarty K, et al. BSR, and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology 2014;53:2306–9.
  4. Yates M, Watts R, Bajema I, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis 2016;75:1583–94.
  5. McGeoch L, Twilt M, Famorca L, et al. CanVasc recommendations for the management of antineutrophil cytoplasm antibody-associated vasculitides. J Rheumatol 2016;43:97–120.


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