Vasculitis

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Vasculitis
Classification and external resources
Churg-Strauss syndrome - very high mag.jpg
ICD-10 I77.6, I80, L95, M30-M31
ICD-9 446, 447.6
DiseasesDB 13750
Patient UK Vasculitis
MeSH D014657

Vasculitis (plural: vasculitides)[1] is a group of disorders that destroy blood vessels by inflammation.[2] Both arteries and veins are affected. Lymphangitis is sometimes considered a type of vasculitis.[3] Vasculitis is primarily caused by leukocyte migration and resultant damage.

Although both occur in vasculitis, inflammation of veins (phlebitis) or arteries (arteritis) on their own are separate entities.

Signs and symptoms[edit]

Petechia and purpura on the lower limb due to medication-induced vasculitis.

Possible symptoms include:[4]

Cause[edit]

Classification[edit]

Vasculitis can be classified by the cause, the location, the type of vessel or the size of vessel.

  • Underlying cause. For example, the cause of syphilitic aortitis is infectious (aortitis simply refers to inflammation of the aorta, which is an artery.) However, the causes of many forms of vasculitis are poorly understood. There is usually an immune component, but the trigger is often not identified. In these cases, the antibody found is sometimes used in classification, as in ANCA-associated vasculitides.
  • Location of the affected vessels. For example, ICD-10 classifies "vasculitis limited to skin" with skin conditions (under "L"), and "necrotizing vasculopathies" (corresponding to systemic vasculitis) with musculoskeletal system and connective tissue conditions (under "M"). Arteritis/phlebitis on their own are classified with circulatory conditions (under "I").
  • Type or size of the blood vessels that they predominantly affect.[5] Apart from the arteritis/phlebitis distinction mentioned above, vasculitis is often classified by the caliber of the vessel affected. However, there can be some variation in the size of the vessels affected.

According to the size of the vessel affected, vasculitis can be classified into:[6]

Conditions[edit]

Some disorders have vasculitis as their main feature. The major types are given in the table below:

Comparison of major types of vasculitis
Vasculitis Affected organs Histopathology
Cutaneous small-vessel vasculitis Skin, kidneys Neutrophils, fibrinoid necrosis
Wegener's granulomatosis Nose, lungs, kidneys Neutrophils, giant cells
Churg–Strauss syndrome Lungs, kidneys, heart, skin Histiocytes, eosinophils
Behçet's disease Commonly sinuses, brain, eyes and skin; can affect other organs such as lungs, kidneys, joints;
Kawasaki disease Skin, heart, mouth, eyes Lymphocytes, endothelial necrosis
Buerger's disease Leg arteries and veins (gangrene) Neutrophils, granulomas
"Limited" Wegener's vasculitis Commonly sinuses, brain, and skin; can affect other organs such as lungs, kidneys, joints;

Takayasu's arteritis, polyarteritis nodosa and giant cell arteritis mainly involve arteries and are thus sometimes classed specifically under arteritis.

Furthermore, there are many conditions that have vasculitis as an accompanying or atypical symptom, including:

Several of these vasculitides are associated with antineutrophil cytoplasmic antibodies.[7] These are

  • granulomatosis with polyangiitis (Wegener's granulomatosis)
  • eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).
  • microscopic polyangiitis

Diagnosis[edit]

Severe vasculitis of the major vessels, displayed on FDG-PET/CT
  • Laboratory tests of blood or body fluids are performed for patients with active vasculitis. Their results will generally show signs of inflammation in the body, such as increased erythrocyte sedimentation rate (ESR), elevated C-reactive protein (CRP), anemia, increased white blood cell count and eosinophilia. Other possible findings are elevated antineutrophil cytoplasmic antibody (ANCA) levels and hematuria.
  • Other organ functional tests may be abnormal. Specific abnormalities depend on the degree of various organs involvement. A Brainspect can show decreased blood flow to the brain and brain damage.
  • The definite diagnosis of vasculitis is established after a biopsy of involved organ or tissue, such as skin, sinuses, lung, nerve, brain and kidney. The biopsy elucidates the pattern of blood vessel inflammation.
  • An alternative to biopsy can be an angiogram (x-ray test of the blood vessels). It can demonstrate characteristic patterns of inflammation in affected blood vessels.
  • Acute onset of vasculitis-like symptoms in small children or babies may instead be the life-threatening purpura fulminans, usually associated with severe infection.
Laboratory Investigation of Vasculitic Syndromes[8]
Disease Serologic test Antigen Associated laboratory features
Systemic lupus erythematosus ANA including antibodies to dsDNA and ENA [including SM, Ro (SSA), La (SSB), and RNP] Nuclear antigens Leukopenia, thrombocytopenia, Coombs' test, complement activation: low serum concentrations of C3 and C4, positive immunofluorescence using Crithidia luciliae as substrate, antiphospholipid antibodies (i.e., anticardiolipin, lupus anticoagulant, false-positive VDRL)
Goodpasture's disease Anti-glomerular basement membrane antibody Epitope on noncollagen domain of type IV collagen
Small vessel vasculitis
Microscopic polyangiitis Perinuclear antineutrophil cytoplasmic antibody Myeloperoxidase Elevated CRP
Wegener's granulomatosis Cytoplasmic antineutrophil cytoplasmic antibody Proteinase 3 (PR3) Elevated CRP
Churg-Strauss syndrome perinuclear antineutrophil cytoplasmic antibody in some cases Myeloperoxidase Elevated CRP and eosinophilia
Henoch-Schönlein purpura None
Cryoglobulinemia Cryoglobulins, rheumatoid factor, complement components, hepatitis C
Medium vessel vasculitis
Classical polyarteritis nodosa None Elevated CRP and eosinophilia

In this table: ANA = Antinuclear antibodies, CRP = C-reactive protein, dsDNA = double-stranded DNA, ENA = extractable nuclear antigens, RNP = ribonucleoproteins; VDRL = Venereal Disease Research Laboratory

Treatment[edit]

Treatments are generally directed toward stopping the inflammation and suppressing the immune system. Typically, corticosteroids such as prednisone are used. Additionally, other immune suppression drugs, such as cyclophosphamide and others, are considered. In case of an infection, antimicrobial agents including cephalexin may be prescribed. Affected organs (such as the heart or lungs) may require specific medical treatment intended to improve their function during the active phase of the disease.

References[edit]

  1. ^ "Vasculitis - Definition from the Merriam-Webster Online Dictionary". Retrieved 2009-01-08. 
  2. ^ "Glossary of dermatopathological terms. DermNet NZ". Retrieved 2009-01-08. 
  3. ^ "Vasculitis" at Dorland's Medical Dictionary
  4. ^ "The Johns Hopkins Vasculitis Center - Symptoms of Vasculitis". Retrieved 2009-05-07. 
  5. ^ Jennette JC, Falk RJ, Andrassy K, et al. (1994). "Nomenclature of systemic vasculitides. Proposal of an international consensus conference". Arthritis Rheum. 37 (2): 187–92. doi:10.1002/art.1780370206. PMID 8129773. 
  6. ^ http://www.merckmanuals.com/professional/musculoskeletal_and_connective_tissue_disorders/vasculitis/overview_of_vasculitis.html
  7. ^ Millet A, Pederzoli-Ribeil M, Guillevin L, Witko-Sarsat V, Mouthon L (2013) Antineutrophil cytoplasmic antibody-associated vasculitides: is it time to split up the group? Ann Rheum Dis
  8. ^ Burtis CA, Ashwood ER, Bruns DE. Tietz Textbook of Clinical Chemistry and Molecular Diagnostics, 5th edition. Elsevier Saunders. p. 1568. ISBN 978-1-4160-6164-9.