Vasculitis

Vasculitis

Micrograph showing a vasculitis (Churg-Strauss

syndrome). H&E stain.
Classification and external resources
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. Lymphangitisis sometimes
considered a type of vasculitis.[3] Vasculitis is primarily caused byleukocyte migration and
resultant damage.
Although both occur in vasculitis, inflammation of veins (phlebitis) or arteries (arteritis) on their
own are separate entities.
Contents
[hide]

1 Signs and symptoms

2 Cause
o 2.1 Classification
o 2.2 Conditions

3 Diagnosis

4 Treatment

5 References

Signs and symptoms[edit]

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

Possible symptoms include:[4]

General symptoms: Fever, weight loss

Skin: Palpable purpura, livedo reticularis

Muscles and joints: Myalgia or myositis, arthralgia or arthritis

Nervous system: Mononeuritis multiplex, headache, stroke, tinnitus, reducedvisual

acuity, acute visual loss

Heart and arteries: Myocardial infarction, hypertension, gangrene

Respiratory tract: Nose bleeds, bloody cough, lung infiltrates

GI tract: Abdominal pain, bloody stool, perforations

Kidneys: Glomerulonephritis

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 theaorta, 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]

Large vessel: Polymyalgia rheumatica, Takayasu's arteritis, Temporal arteritis

Medium vessel: Buerger's disease, Cutaneous vasculitis, Kawasaki disease, Polyarteritis

nodosa

Small vessel: Behet's syndrome, ChurgStrauss syndrome, cutaneous

vasculitis, HenochSchnlein purpura,Microscopic polyangiitis, granulomatosis with
polyangiitis, Golfer's vasculitis, cryoglobulinemia.

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

Granulomatosis with
polyangiitis

Nose, lungs, kidneys

Neutrophils, giant cells

ChurgStrauss syndrome Lungs, kidneys, heart, skin

Histiocytes,eosinophils

Behet'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 necrosi
s

Buerger's disease

Leg arteries and veins

(gangrene)

Neutrophils,granulomas

"Limited"
granulomatosis with
polyangiitis 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:

Rheumatic diseases, such as rheumatoid arthritis, systemic lupus erythematosus,

and dermatomyositis

Cancer, such as lymphomas

Infections, such as hepatitis C

Exposure to chemicals and drugs, such as amphetamines, cocaine, and anthrax

vaccines which contain the Anthrax Protective Antigen as the primary ingredient.

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

granulomatosis with polyangiitis (formerly known as 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

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, falsepositive VDRL)

Systemic lupus
erythematosus

ANA including
antibodies to
dsDNA and ENA
[including SM,
Ro (SSA), La
(SSB), and RNP]

Nuclear antigens

Goodpasture's
disease

Anti-glomerular
basement
membrane
antibody

Epitope on
noncollagen
domain of type IV
collagen

Microscopic
polyangiitis

Perinuclear
antineutrophil
cytoplasmic
antibody

Myeloperoxidase

Elevated CRP

Granulomatosis
with polyangiiitis

Cytoplasmic
antineutrophil
cytoplasmic
antibody

Proteinase 3
(PR3)

Elevated CRP

Churg-Strauss
syndrome

perinuclear
antineutrophil
cytoplasmic
antibody in some
cases

Myeloperoxidase

Elevated CRP and eosinophilia

Small vessel
vasculitis

Henoch-Schnlein
None
purpura

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 = doublestranded 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 cyclophosphamideand 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. Jump up^ "Vasculitis - Definition from the Merriam-Webster Online Dictionary".
Retrieved 2009-01-08.
2. Jump up^ "Glossary of dermatopathological terms. DermNet NZ".
Retrieved 2009-01-08.
3. Jump up^ "Vasculitis" at Dorland's Medical Dictionary
4. Jump up^ "The Johns Hopkins Vasculitis Center - Symptoms of Vasculitis".
Retrieved 2009-05-07.
5. Jump up^ 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. Jump
up^http://www.merckmanuals.com/professional/musculoskeletal_and_connective
_tissue_disorders/vasculitis/overview_of_vasculitis.html

7. Jump up^ 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. Jump up^ 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.