| Vasculitis |
|
Micrograph showing a vasculitis (Churg-Strauss syndrome). H&E stain.
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| Classification and external resources |
| Specialty |
Rheumatology |
| ICD-10 |
I77.6, I80, L95, M30-M31 |
| ICD-9-CM |
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.
Contents
- 1 Signs and symptoms
- 2 Cause
- 2.1 Classification
- 2.2 Conditions
- 3 Diagnosis
- 4 Treatment
- 5 References
Signs and symptoms
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, reduced visual 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
Classification
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]
- Large vessel: Polymyalgia rheumatica, Takayasu's arteritis, Temporal arteritis
- Medium vessel: Buerger's disease, Cutaneous vasculitis, Kawasaki disease, Polyarteritis nodosa
- Small vessel: Behçet's syndrome, Churg–Strauss syndrome, cutaneous vasculitis, Henoch–Schönlein purpura, Microscopic polyangiitis, granulomatosis with polyangiitis, Golfer's vasculitis, cryoglobulinemia.
Conditions
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 |
| 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" 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.
In pediatric patients varicella infection may be followed by vasculitis of intracranial vessles. This condition is called post varicella angiopathy amd this may be responsible for arterial ischaemic strokes in children. [7]
Several of these vasculitides are associated with antineutrophil cytoplasmic antibodies.[8] These are
- granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis)
- eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).
- microscopic polyangiitis
Diagnosis
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.
- 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT)has become a widely used imaging tool in patients with suspected Large Vessel Vasculitis, due to the enhanced glucose metabolism of inflamed vessel walls.[9] The combined evaluation of the intensity and the extension of FDG vessel uptake at diagnosis can predict the clinical course of the disease, separating patients with favourable or complicated progress.[10]
- 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[11]
| 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 |
| 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 |
| 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
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
- ^ "Vasculitis - Definition from the Merriam-Webster Online Dictionary". Retrieved 2009-01-08.
- ^ "Glossary of dermatopathological terms. DermNet NZ". Retrieved 2009-01-08.
- ^ "Vasculitis" at Dorland's Medical Dictionary
- ^ "The Johns Hopkins Vasculitis Center - Symptoms of Vasculitis". Retrieved 2009-05-07.
- ^ 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.
- ^ http://www.merckmanuals.com/professional/musculoskeletal_and_connective_tissue_disorders/vasculitis/overview_of_vasculitis.html
- ^ Nita R Sutay, Md Ashfaque Tinmaswala, Shilpa Hegde . http://ijmrhs.com/post-varicella-angiopathy-a-case-report
- ^ 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
- ^ Maffioli L, Mazzone A. NEJM, 2014. http://www.nejm.org/doi/full/10.1056/NEJMc1409206#article
- ^ L. Dellavedova, M. Carletto, P. Faggioli, A. Sciascera, A. Del Sole, A. Mazzone, L. S. Maffioli. EJNMMI, 2015 http://link.springer.com/article/10.1007%2Fs00259-015-3148-9
- ^ 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.
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Cutaneous vasculitis and other vascular-related cutaneous conditions (L95, 709.1)
|
|
| Cutaneous vasculitis |
- Erythema elevatum diutinum
- Capillaritis
- Urticarial vasculitis
- Nodular vasculitis
|
|
| Microvascular occlusion |
- Calciphylaxis
- Cryoglobulinemic purpura/Cryoglobulinemic vasculitis
- vascular coagulopathy: Livedoid vasculitis
- Livedoid dermatitis
- Perinatal gangrene of the buttock
- Malignant atrophic papulosis
- Sneddon's syndrome
|
|
| Purpura |
- Nonthrombocytopenic purpura: Cryofibrinogenemic purpura
- Drug-induced purpura
- Food-induced purpura
- Henoch–Schönlein purpura
- Obstructive purpura
- Orthostatic purpura
- Purpura fulminans
- Purpura secondary to clotting disorders
- Purpuric agave dermatitis
- Pigmentary purpuric eruptions
- Solar purpura
- Traumatic purpura
- Waldenström hyperglobulinemic purpura
- Painful bruising syndrome
- ungrouped: Paroxysmal hand hematoma
- Postcardiotomy syndrome
- Deep vein thrombosis
- Superficial thrombophlebitis
- Mondor's disease
- Blueberry muffin baby
- Fibrinolysis syndrome
|
|
| Systemic vasculitis |
- see Template:Systemic vasculitis
|
|
| Vascular malformations |
- Arteriovenous malformation
- Bonnet–Dechaume–Blanc syndrome
- Cobb syndrome
- Parkes Weber syndrome
- Sinusoidal hemangioma
- lymphatic malformation
- Hennekam syndrome
- Aagenaes syndrome
- telangiectasia: Generalized essential telangiectasia
- Hereditary hemorrhagic telangiectasia
- Unilateral nevoid telangiectasia
|
|
| Ulcer |
- Venous ulcer
- Arterial insufficiency ulcer
- Hematopoietic ulcer
- Neuropathic ulcer
- Acroangiodermatitis
|
|
| Lymphedema |
- see Template:Lymphatic vessel disease
|
|
Ungrouped
vascular-related
cutaneous conditions |
- Raynaud's phenomenon
- Thromboangiitis obliterans
- Erythromelalgia
- Septic thrombophlebitis
- Arteriosclerosis obliterans
- Bier spots/Marshall–White syndrome
- Cholesterol embolus
- Reactive angioendotheliomatosis
- Trousseau's syndrome
|
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Index of skin
|
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| Description |
- Anatomy
- Physiology
- Development
|
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| Disease |
- Infections
- Vesiculobullous
- Dermatitis and eczema
- Papulosquamous
- Urticaria and erythema
- Radiation-related
- Pigmentation
- Mucinoses
- Keratosis, ulcer, atrophy, and necrobiosis
- Vasculitis
- Fat
- Neutrophilic and eosinophilic
- Congenital
- Neoplasms and cancer
- nevi and melanomas
- epidermis
- dermis
- Symptoms and signs
- Terminology
|
|
| Treatment |
- Procedures
- Drugs
- antibiotics
- disinfectants
- emollients and protectives
- itch
- psoriasis
- other
- Wound and ulcer
|
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Systemic connective tissue disorders (M32–M36, 710)
|
|
| General |
| Systemic lupus erythematosus |
- Drug-induced SLE
- Libman-Sacks endocarditis
|
|
| Inflammatory myopathy |
- Myositis
- Dermatopolymyositis
- Dermatomyositis/Juvenile dermatomyositis
- Polymyositis* Inclusion body myositis
|
|
| Scleroderma |
- Systemic scleroderma
- Progressive systemic sclerosis
- CREST syndrome
|
|
- Overlap syndrome / Mixed connective tissue disease
|
|
|
| Other hypersensitivity/autoimmune |
|
|
| Other |
- Behçet's disease
- Polymyalgia rheumatica
- Eosinophilic fasciitis
- Eosinophilia–myalgia syndrome
- fibrillin
- Marfan syndrome
- Congenital contractural arachnodactyly
|
|
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Index of muscle
|
|
| Description |
- Anatomy
- head
- neck
- arms
- chest and back
- diaphragm
- abdomen
- genital area
- legs
- Muscle tissue
- Physiology
|
|
| Disease |
- Myopathy
- Soft tissue
- Connective tissue
- Congenital
- abdomen
- muscular dystrophy
- Neoplasms and cancer
- Injury
- Symptoms and signs
|
|
| Treatment |
- Procedures
- Drugs
- anti-inflammatory
- muscle relaxants
|
|
|
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Inflammation
|
|
| Acute |
| Plasma derived mediators |
- Bradykinin
- complement
- coagulation
- Factor XII
- Plasmin
- Thrombin
|
|
| Cell derived mediators |
| preformed: |
- Lysosome granules
- biogenic amines
|
|
| synthesized on demand: |
- cytokines
- eicosanoids
- Leukotriene B4
- Prostaglandins
- Nitric oxide
- Kinins
|
|
|
|
| Chronic |
- Macrophage
- Epithelioid cell
- Giant cell
- Granuloma
|
|
| Processes |
| Traditional: |
- Rubor
- Calor
- Tumor
- Dolor (pain)
- Functio laesa
|
|
| Modern: |
- Acute-phase reaction/Fever
- Vasodilation
- Increased vascular permeability
- Exudate
- Leukocyte extravasation
- Chemotaxis
|
|
|
| Specific locations |
|
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