Vasculitis
Encyclopedia
Vasculitis 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.
Although both occur in vasculitis, inflammation of veins (phlebitis
) or arteries (arteritis
) on their own are separate entities.
Takayasu's arteritis
, polyarteritis nodosa
and giant cell arteritis are sometimes classified as vasculitis as well, but rather belong to arteritis
since they mainly involve arteries.
Furthermore, there are many conditions that have vasculitis as an accompanying or atypical symptom, including:
-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.
Inflammation
Inflammation is part of the complex biological response of vascular tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. Inflammation is a protective attempt by the organism to remove the injurious stimuli and to initiate the healing process...
destruction of blood vessels. Both arteries and veins are affected. Lymphangitis
Lymphangitis
Lymphangitis is an inflammation of the lymphatic channels that occurs as a result of infection at a site distal to the channel. The most common cause of lymphangitis in humans is Streptococcus pyogenes...
is sometimes considered a type of vasculitis. Vasculitis is primarily due to leukocyte migration and resultant damage.
Although both occur in vasculitis, inflammation of veins (phlebitis
Phlebitis
Phlebitis is an inflammation of a vein, usually in the legs.When phlebitis is associated with the formation of blood clots , usually in the deep veins of the legs, the condition is called thrombophlebitis...
) or arteries (arteritis
Arteritis
Arteritis is inflammation of the walls of arteries, usually as a result of infection or auto-immune response.-Types:Some disorders have arteritis as their main feature...
) on their own are separate entities.
Classification
There are many ways to classify vasculitis.- It can be classified by the underlying cause. For example, the cause of syphilitic aortitisSyphilitic aortitisSyphilitic aortitis is a disease of the aorta associated with the tertiary stage of syphilis infection. SA begins as inflammation of the adventia, including the vessels that supply the aorta itself with blood, the vasa vasorum...
is infectious (aortitisAortitis-Causes:This inflammation has a number of possible causes, to include: trauma, viral infection, certain immune disorders, and bacterial infection .-Treatment:...
simply refers to arteritis of the aortaAortaThe aorta is the largest artery in the body, originating from the left ventricle of the heart and extending down to the abdomen, where it branches off into two smaller arteries...
, 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 vasculitidesANCA-associated vasculitidesANCA-associated vasculitides are diseases caused by vasculitis in which antineutrophil cytoplasmic antibodies can be detected in the blood.-Types:Three main AAVs are Churg-Strauss syndrome, microscopic polyangiitis and Wegener's granulomatosis...
. - It can be classified by the location of the affected vessels. For example, ICD-10ICD-10The International Statistical Classification of Diseases and Related Health Problems, 10th Revision is a medical classification list for the coding of diseases, signs and symptoms, abnormal findings, complaints, social circumstances, and external causes of injury or diseases, as maintained by the...
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 arteritisArteritisArteritis is inflammation of the walls of arteries, usually as a result of infection or auto-immune response.-Types:Some disorders have arteritis as their main feature...
/phlebitisPhlebitisPhlebitis is an inflammation of a vein, usually in the legs.When phlebitis is associated with the formation of blood clots , usually in the deep veins of the legs, the condition is called thrombophlebitis...
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.
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 Histopathology Histopathology refers to the microscopic examination of tissue in order to study the manifestations of disease... |
| Cutaneous small-vessel vasculitis Cutaneous small-vessel vasculitis Cutaneous small-vessel vasculitis is inflammation of small blood vessels , characterized by palpable purpura. It is the most common vasculitis seen in clinical practice... |
Skin, kidneys | Neutrophils, fibrinoid necrosis Fibrinoid necrosis Fibrinoid necrosis is a form of necrosis, or tissue death, in which there is accumulation of amorphous, basic, proteinaceous material in the tissue matrix with a staining pattern reminiscent of fibrin. It is associated with conditions such as immune vasculitis Fibrinoid necrosis is a form of... |
| Wegener's granulomatosis Wegener's granulomatosis Wegener's granulomatosis , more recently granulomatosis with polyangiitis , is an incurable form of vasculitis that affects the nose, lungs, kidneys and other organs. Due to its end-organ damage, it is life-threatening and requires long-term immunosuppression... |
Nose, lungs, kidneys | Neutrophils, giant cell Giant cell A giant cell is a mass formed by the union of several distinct cells . It can arise in response to an infection or foreign body.Types include:* foreign-body giant cell* Langhans giant cell* Touton giant cells... s |
| Churg–Strauss syndrome | Lungs, kidneys, heart, skin | Histiocyte Histiocyte A histiocyte is a cell that is part of the mononuclear phagocyte system . The mononuclear phagocytic system is part of the organism's immune system... s, eosinophils |
| Kawasaki disease Kawasaki disease Kawasaki disease , also known as Kawasaki syndrome, lymph node syndrome and mucocutaneous lymph node syndrome, is an autoimmune disease in which the medium-sized blood vessels throughout the body become inflamed. It is largely seen in children under five years of age... |
Skin, heart, mouth, eyes | Lymphocyte Lymphocyte A lymphocyte is a type of white blood cell in the vertebrate immune system.Under the microscope, lymphocytes can be divided into large lymphocytes and small lymphocytes. Large granular lymphocytes include natural killer cells... s, endothelial necrosis |
| Buerger's disease Buerger's disease Thromboangiitis obliterans is a recurring progressive inflammation and thrombosis of small and medium arteries and veins of the hands and feet... |
Leg arteries and veins (gangrene Gangrene Gangrene is a serious and potentially life-threatening condition that arises when a considerable mass of body tissue dies . This may occur after an injury or infection, or in people suffering from any chronic health problem affecting blood circulation. The primary cause of gangrene is reduced blood... ) |
Neutrophils, granuloma Granuloma Granuloma is a medical term for a tiny collection of immune cells known as macrophages. Granulomas form when the immune system attempts to wall off substances that it perceives as foreign but is unable to eliminate. Such substances include infectious organisms such as bacteria and fungi as well as... s |
Takayasu's arteritis
Takayasu's arteritis
Takayasu's arteritis is a form of large vessel granulomatous vasculitis with massive intimal fibrosis and vascular narrowing affecting often young or middle-aged women of Asian descent...
, polyarteritis nodosa
Polyarteritis nodosa
Polyarteritis nodosa is a vasculitis of medium & small-sized arteries, which become swollen and damaged from attack by rogue immune cells. Polyarteritis nodosa is also called Kussmaul disease or Kussmaul-Maier disease...
and giant cell arteritis are sometimes classified as vasculitis as well, but rather belong to arteritis
Arteritis
Arteritis is inflammation of the walls of arteries, usually as a result of infection or auto-immune response.-Types:Some disorders have arteritis as their main feature...
since they mainly involve arteries.
Furthermore, there are many conditions that have vasculitis as an accompanying or atypical symptom, including:
- Rheumatic diseases, such as rheumatoid arthritisRheumatoid arthritisRheumatoid arthritis is a chronic, systemic inflammatory disorder that may affect many tissues and organs, but principally attacks synovial joints. The process produces an inflammatory response of the synovium secondary to hyperplasia of synovial cells, excess synovial fluid, and the development...
, systemic lupus erythematosusSystemic lupus erythematosusSystemic lupus erythematosus , often abbreviated to SLE or lupus, is a systemic autoimmune disease that can affect any part of the body. As occurs in other autoimmune diseases, the immune system attacks the body's cells and tissue, resulting in inflammation and tissue damage...
, and dermatomyositisDermatomyositisDermatomyositis is a connective-tissue disease related to polymyositis and Bramaticosis that is characterized by inflammation of the muscles and the skin.- Causes :... - CancerCancerCancer , known medically as a malignant neoplasm, is a large group of different diseases, all involving unregulated cell growth. In cancer, cells divide and grow uncontrollably, forming malignant tumors, and invade nearby parts of the body. The cancer may also spread to more distant parts of the...
, such as lymphomas - Infections, such as hepatitis CHepatitis CHepatitis C is an infectious disease primarily affecting the liver, caused by the hepatitis C virus . The infection is often asymptomatic, but chronic infection can lead to scarring of the liver and ultimately to cirrhosis, which is generally apparent after many years...
- Exposure to chemicals and drugs, such as amphetamines, cocaineCocaineCocaine is a crystalline tropane alkaloid that is obtained from the leaves of the coca plant. The name comes from "coca" in addition to the alkaloid suffix -ine, forming cocaine. It is a stimulant of the central nervous system, an appetite suppressant, and a topical anesthetic...
, and anthrax vaccines which contain the Anthrax Protective Antigen as the primary ingredient.
Symptoms
Possible symptoms include:- General symptoms: FeverFeverFever is a common medical sign characterized by an elevation of temperature above the normal range of due to an increase in the body temperature regulatory set-point. This increase in set-point triggers increased muscle tone and shivering.As a person's temperature increases, there is, in...
, weight loss - Skin: Palpable purpuraPurpuraPurpura is the appearance of red or purple discolorations on the skin that do not blanch on applying pressure. They are caused by bleeding underneath the skin...
, livedo reticularis - Muscles and joints: MyalgiaMyalgiaMyalgia means "muscle pain" and is a symptom of many diseases and disorders. The most common causes are the overuse or over-stretching of a muscle or group of muscles. Myalgia without a traumatic history is often due to viral infections...
or myositisMyositisMyositis is a general term for inflammation of the muscles. Many such conditions are considered likely to be caused by autoimmune conditions, rather than directly due to infection It is also a documented side effect of the lipid-lowering drugs statins and fibrates.Elevation of creatine kinase in...
, arthralgiaArthralgiaArthralgia literally means joint pain; it is a symptom of injury, infection, illnesses or an allergic reaction to medication....
or arthritisArthritisArthritis is a form of joint disorder that involves inflammation of one or more joints.... - Nervous system: Mononeuritis multiplex, headacheHeadacheA headache or cephalalgia is pain anywhere in the region of the head or neck. It can be a symptom of a number of different conditions of the head and neck. The brain tissue itself is not sensitive to pain because it lacks pain receptors. Rather, the pain is caused by disturbance of the...
, strokeStrokeA stroke, previously known medically as a cerebrovascular accident , is the rapidly developing loss of brain function due to disturbance in the blood supply to the brain. This can be due to ischemia caused by blockage , or a hemorrhage...
, tinnitusTinnitusTinnitus |ringing]]") is the perception of sound within the human ear in the absence of corresponding external sound.Tinnitus is not a disease, but a symptom that can result from a wide range of underlying causes: abnormally loud sounds in the ear canal for even the briefest period , ear...
, reduced visual acuityVisual acuityVisual acuity is acuteness or clearness of vision, which is dependent on the sharpness of the retinal focus within the eye and the sensitivity of the interpretative faculty of the brain....
, acute visual loss - Heart and arteries: Myocardial infarctionMyocardial infarctionMyocardial infarction or acute myocardial infarction , commonly known as a heart attack, results from the interruption of blood supply to a part of the heart, causing heart cells to die...
, hypertensionHypertensionHypertension or high blood pressure is a cardiac chronic medical condition in which the systemic arterial blood pressure is elevated. What that means is that the heart is having to work harder than it should to pump the blood around the body. Blood pressure involves two measurements, systolic and...
, gangreneGangreneGangrene is a serious and potentially life-threatening condition that arises when a considerable mass of body tissue dies . This may occur after an injury or infection, or in people suffering from any chronic health problem affecting blood circulation. The primary cause of gangrene is reduced blood... - Respiratory tract: Nose bleeds, bloody coughHemoptysisHemoptysis or haemoptysis is the expectoration of blood or of blood-stained sputum from the bronchi, larynx, trachea, or lungs Hemoptysis or haemoptysis is the expectoration (coughing up) of blood or of blood-stained sputum from the bronchi, larynx, trachea, or lungs Hemoptysis or haemoptysis ...
, lung infiltrates - GI tract: Abdominal painAbdominal painAbdominal pain can be one of the symptoms associated with transient disorders or serious disease. Making a definitive diagnosis of the cause of abdominal pain can be difficult, because many diseases can result in this symptom. Abdominal pain is a common problem...
, bloody stoolMelenaIn medicine, melena or melaena refers to the black, "tarry" feces that are associated with gastrointestinal hemorrhage. The black color is caused by oxidation of the iron in hemoglobin during its passage through the ileum and colon.-Melena vs...
, perforations - Kidneys: GlomerulonephritisGlomerulonephritisGlomerulonephritis, also known as glomerular nephritis, abbreviated GN, is a renal disease characterized by inflammation of the glomeruli, or small blood vessels in the kidneys...
Diagnosis
- 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 rateErythrocyte sedimentation rateThe erythrocyte sedimentation rate , also called a sedimentation rate or Biernacki Reaction, is the rate at which red blood cells sediment in a period of 1 hour...
(ESR), elevated C-reactive proteinC-reactive proteinC-reactive protein is a protein found in the blood, the levels of which rise in response to inflammation...
(CRP), anemiaAnemiaAnemia is a decrease in number of red blood cells or less than the normal quantity of hemoglobin in the blood. However, it can include decreased oxygen-binding ability of each hemoglobin molecule due to deformity or lack in numerical development as in some other types of hemoglobin...
, increased white blood cell countLeukocytosisLeukocytosis is a raised white blood cell count above the normal range in the blood. It is frequently a sign of an inflammatory response, most commonly the result of infection, and is observed in certain parasitic infections...
and eosinophiliaEosinophiliaEosinophilia is a condition in which the eosinophil count in the peripheral blood exceeds 0.45×109/L . A marked increase in non-blood tissue eosinophil count noticed upon histopathologic examination is diagnostic for tissue eosinophilia. Several causes are known, with the most common being...
. Other possible findings are elevated antineutrophil cytoplasmic antibody (ANCA) levels and hematuriaHematuriaIn medicine, hematuria, or haematuria, is the presence of red blood cells in the urine. It may be idiopathic and/or benign, or it can be a sign that there is a kidney stone or a tumor in the urinary tract , ranging from trivial to lethal...
. - 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 biopsyBiopsyA biopsy is a medical test involving sampling of cells or tissues for examination. It is the medical removal of tissue from a living subject to determine the presence or extent of a disease. The tissue is generally examined under a microscope by a pathologist, and can also be analyzed chemically...
of involved organ or tissue, such as skinSkin-Dermis:The dermis is the layer of skin beneath the epidermis that consists of connective tissue and cushions the body from stress and strain. The dermis is tightly connected to the epidermis by a basement membrane. It also harbors many Mechanoreceptors that provide the sense of touch and heat...
, sinuses, lungLungThe lung is the essential respiration organ in many air-breathing animals, including most tetrapods, a few fish and a few snails. In mammals and the more complex life forms, the two lungs are located near the backbone on either side of the heart...
, nerveNerveA peripheral nerve, or simply nerve, is an enclosed, cable-like bundle of peripheral axons . A nerve provides a common pathway for the electrochemical nerve impulses that are transmitted along each of the axons. Nerves are found only in the peripheral nervous system...
, and kidneyKidneyThe kidneys, organs with several functions, serve essential regulatory roles in most animals, including vertebrates and some invertebrates. They are essential in the urinary system and also serve homeostatic functions such as the regulation of electrolytes, maintenance of acid–base balance, and...
. The biopsy elucidates the pattern of blood vessel inflammation. - An alternative to biopsy can be an angiogramAngiogramAngiography or arteriography is a medical imaging technique used to visualize the inside, or lumen, of blood vessels and organs of the body, with particular interest in the arteries, veins and the heart chambers...
(x-rayX-rayX-radiation is a form of electromagnetic radiation. X-rays have a wavelength in the range of 0.01 to 10 nanometers, corresponding to frequencies in the range 30 petahertz to 30 exahertz and energies in the range 120 eV to 120 keV. They are shorter in wavelength than UV rays and longer than gamma...
test of the blood vessels). It can demonstrate characteristic patterns of inflammation in affected blood vessels.
Treatment
Treatments are generally directed toward stopping the inflammation and suppressing the immune system. Typically, cortisoneCortisone
Cortisone is a steroid hormone. It is one of the main hormones released by the adrenal gland in response to stress. In chemical structure, it is a corticosteroid closely related to corticosterone. It is used to treat a variety of ailments and can be administered intravenously, orally,...
-related medications, such as prednisone
Prednisone
Prednisone is a synthetic corticosteroid drug that is particularly effective as an immunosuppressant drug. It is used to treat certain inflammatory diseases and some types of cancer, but has significant adverse effects...
, are used. Additionally, other immune suppression drugs, such as cyclophosphamide
Cyclophosphamide
Cyclophosphamide , also known as cytophosphane, is a nitrogen mustard alkylating agent, from the oxazophorines group....
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.