Vasculitis Classification and external resources
Micrograph showing a vasculitis (Churg-Strauss syndrome). H&E stain.
ICD-10 I77.6, I80, L95, M30-M31 ICD-9 446, 447.6 DiseasesDB 13750 MeSH D014657
Vasculitis (plural: vasculitides) refers to a heterogeneous group of disorders that are characterized by inflammatory destruction of blood vessels. Both arteries and veins are affected. Lymphangitis is sometimes considered a type of vasculitis. Vasculitis is primarily due to leukocyte migration and resultant damage.
There are many ways to classify vasculitis.
- It can be classified by the underlying cause. For example, the cause of syphilitic aortitis is infectious (aortitis simply refers to arteritis of the aorta, which is an artery.) However, the cause 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.
- It can be classified by the location of the affected vessels. For example, ICD-10 classifies "vasculitis limited to skin" with skin conditions (under "L"), and "necrotizing vasculopathies" with musculoskeletal system and connective tissue conditions (under "M"). Arteritis/phlebitis on their own are classified with circulatory conditions (under "I").
- Vasculitides can be classified by the type or size of the blood vessels that they predominantly affect. 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.
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 Kawasaki disease Skin, heart, mouth, eyes Lymphocytes, endothelial necrosis Buerger's disease Leg arteries and veins (gangrene) Neutrophils, granulomas
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.
Possible symptoms include:
- 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
- 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.
- The definite diagnosis of vasculitis is established after a biopsy of involved organ or tissue, such as skin, sinuses, lung, nerve, 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.
Treatments are generally directed toward stopping the inflammation and suppressing the immune system. Typically, cortisone-related medications, 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.
- ^ "Vasculitis - Definition from the Merriam-Webster Online Dictionary". http://www.merriam-webster.com/dictionary/Vasculitis. Retrieved 2009-01-08.
- ^ "Glossary of dermatopathological terms. DermNet NZ". http://dermnetnz.org/pathology/pathology-glossary.html. Retrieved 2009-01-08.
- ^ "Vasculitis" at Dorland's Medical Dictionary
- ^ 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.
- ^ Unless else specified in boxes, then reference is:
- Stevens & Lowe: Pathology. At Fleshandbones.com
- ^ "The Johns Hopkins Vasculitis Center - Symptoms of Vasculitis". http://vasculitis.med.jhu.edu/whatis/symptoms.html. Retrieved 2009-05-07.
Diseases of the skin and appendages by morphology GrowthsPigmentedDermal and
PurpuraMacularthrombocytopenic purpura · actinic purpuraPapulardisseminated intravascular coagulation · vasculitisIndurated
Cardiovascular disease: vascular disease · Circulatory system pathology (I70–I99, 440–456) Arteries, arterioles
Veinsprimarily lower limb (Deep vein thrombosis)abdomen (Hepatic veno-occlusive disease, Budd–Chiari syndrome, May-Thurner syndrome, Portal vein thrombosis, Renal vein thrombosis)Other Arteries or veins Blood pressure Cutaneous vasculitis and other vascular-related cutaneous conditions (L95, 709.1) Cutaneous
Microvascular occlusion 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 syndromeungrouped: Paroxysmal hand hematoma · Postcardiotomy syndrome · Deep venous thrombosis · Superficial thrombophlebitis · Mondor's disease · Blueberry muffin baby · Fibrinolysis syndrome
Systemic vasculitis Vascular malformationstelangiectasia: Generalized essential telangiectasia · Hereditary hemorrhagic telangiectasia · Unilateral nevoid telangiectasia Ulcer Lymphedema Ungrouped
Systemic CT disorders (M32–M36, 710) GeneralInflammatory myopathy/Myositis: Dermatopolymyositis (Dermatomyositis/Juvenile dermatomyositis, Polymyositis) · Inclusion body myositis Other hypersensitivity/autoimmune Other Inflammation Acute Chronic Processes Specific locationsCardiovascularDigestivemouth (Stomatitis, Gingivitis, Gingivostomatitis, Glossitis, Tonsillitis, Sialadenitis/Parotitis, Cheilitis, Pulpitis, Gnathitis) · tract (Esophagitis, Gastritis, Gastroenteritis, Enteritis, Colitis, Enterocolitis, Duodenitis, Ileitis, Caecitis, Appendicitis, Proctitis) · accessory (Hepatitis, Cholangitis, Cholecystitis, Pancreatitis) · Peritonitis
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