Vasculitis | |
---|---|
Other names | Vasculitides [1] |
Petechia and purpura on the lower limb due to medication-induced vasculitis | |
Pronunciation | |
Specialty | Rheumatology, Immunology |
Symptoms | Weight loss, fever, myalgia, purpura |
Complications | Gangrene, Myocardial infarction |
Vasculitis is a group of disorders that destroy blood vessels by inflammation. [2] Both arteries and veins are affected. Lymphangitis (inflammation of lymphatic vessels) 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.
Possible signs and symptoms include: [4] [5]
There are several different etiologies for vasculitides. Although infections usually involve vessels as a component of more extensive tissue damage, they can also directly or indirectly cause vasculitic syndromes through immune-mediated secondary events. Simple vascular thrombosis usually only affects the luminal process, but through the process of thrombus organization, it can also occasionally cause a more chronic vasculitic syndrome. The autoimmune etiologies, a particular family of diseases characterized by dysregulated immune responses that produce particular pathophysiologic signs and symptoms, are more prevalent. [6]
Primary systemic, secondary, and single-organ vasculitis are distinguished using the highest classification level in the 2012 Chapel Hill Consensus Conference nomenclature. [7]
Primary systemic vasculitis is catogized by the size of the vessels mainly involved. Primary systemic vasculitis includes large-vessel vasculitis, medium-vessel vasculitis, small-vessel vasculitis, and variable-vessel vasculitis. [7]
The 2012 Chapel Hill Consensus Conference defines large vessel vasculitis (LVV) as a type of vasculitis that can affect any size artery, but it usually affects the aorta and its major branches more frequently than other vasculitides. [7] Takayasu arteritis (TA) and giant cell arteritis (GCA) are the two main forms of LVV. [8]
Medium vessel vasculitis (MVV) is a type of vasculitis that mostly affects the medium arteries, which are the major arteries that supply the viscera and their branches. Any size artery could be impacted, though. [7] The two primary types are polyarteritis nodosa (PAN) and Kawasaki disease (KD). [8]
Small vessel vasculitis (SVV) is separated into immune complex SVV and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). [7]
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a necrotizing vasculitis linked to MPO-ANCA or PR3-ANCA that primarily affects small vessels and has few or no immune deposits. AAV is further classified as eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA), granulomatosis with polyangiitis (Wegener's) (GPA), and microscopic polyangiitis (MPA). [7]
Immune complex small vessel vasculitis (SVV) is vasculitis that primarily affects small vessels and has moderate to significant immunoglobulin and complement component deposits on the vessel wall. [7] Normocomplementemic urticarial vasculitis (HUV) (anti-C1q vasculitis), cryoglobulinemic vasculitis (CV), IgA vasculitis (Henoch-Schönlein) (IgAV), and anti-glomerular basement membrane (anti-GBM) disease are the categories of immune complex SVV. [8]
Variable vessel vasculitis (VVV) is a kind of vasculitis that may impact vessels of all sizes (small, medium, and large) and any type (arteries, veins, and capillaries), with no particular type of vessel being predominantly affected. [7] This category includes Behcet's disease (BD) and Cogan's syndrome (CS). [8]
The subset of illnesses known as secondary vasculitis are those believed to be brought on by an underlying ailment or exposure. Systemic illnesses (such as rheumatoid arthritis), cancer, drug exposure, and infection are the primary causes of vasculitis; however, there are still few factors that have a conclusively shown pathogenic relationship to the condition. [9] Vasculitis frequently coexists with infections, and several infections, including hepatitis B and C, HIV, infective endocarditis, and tuberculosis, are significant secondary causes of vasculitis. [10] Except for rheumatoid vasculitis, the majority of secondary vasculitis forms are exceedingly rare. [11]
Single-organ vasculitis, formerly known as "localized," "limited," "isolated," or "nonsystemic" vasculitis, refers to vasculitis that is limited to one organ or organ system. Examples of this type of vasculitis include gastrointestinal, cutaneous, and peripheral nerve vasculitis. [9]
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 polyangiitis | Cytoplasmic antineutrophil cytoplasmic antibody | Proteinase 3 (PR3) | Elevated CRP |
Eosinophilic granulomatosis with polyangiitis | perinuclear antineutrophil cytoplasmic antibody in some cases | Myeloperoxidase | Elevated CRP and eosinophilia |
IgA vasculitis (Henoch-Schönlein purpura) | None | ||
Cryoglobulinemia | Cryoglobulins, rheumatoid factor, complement components, hepatitis C | ||
Medium vessel vasculitis | |||
Classical polyarteritis nodosa | None | Elevated CRP and eosinophilia | |
Kawasaki's Disease | None | Elevated CRP and ESR |
In this table: ANA = antinuclear antibodies, CRP = C-reactive protein, ESR = erythrocyte sedimentation rate, dsDNA = double-stranded DNA, ENA = extractable nuclear antigens, RNP = ribonucleoproteins; VDRL = Venereal Disease Research Laboratory
Treatments are generally directed toward stopping the inflammation and suppressing the immune system. Typically, corticosteroids such as prednisone are used. Additionally, other immune suppression medications, 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.[ citation needed ]
Giant cell arteritis (GCA), also called temporal arteritis, is an inflammatory autoimmune disease of large blood vessels. Symptoms may include headache, pain over the temples, flu-like symptoms, double vision, and difficulty opening the mouth. Complications can include blockage of the artery to the eye with resulting blindness, as well as aortic dissection, and aortic aneurysm. GCA is frequently associated with polymyalgia rheumatica. It can be confirmed by biopsy of the temporal artery in about 90% of people.
Granulomatosis with polyangiitis (GPA), also known as Wegener's granulomatosis (WG), after the German physician Friedrich Wegener, is a rare long-term systemic disorder that involves the formation of granulomas and inflammation of blood vessels (vasculitis). It is an autoimmune disease and a form of vasculitis that affects small- and medium-size vessels in many organs but most commonly affects the upper respiratory tract, lungs and kidneys. The signs and symptoms of GPA are highly varied and reflect which organs are supplied by the affected blood vessels. Typical signs and symptoms include nosebleeds, stuffy nose and crustiness of nasal secretions, and inflammation of the uveal layer of the eye. Damage to the heart, lungs and kidneys can be fatal.
Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as allergic granulomatosis, is an extremely rare autoimmune condition that causes inflammation of small and medium-sized blood vessels (vasculitis) in persons with a history of airway allergic hypersensitivity (atopy).
Polyarteritis nodosa (PAN) is a systemic necrotizing inflammation of blood vessels (vasculitis) affecting medium-sized muscular arteries, typically involving the arteries of the kidneys and other internal organs but generally sparing the lungs' circulation. Small aneurysms are strung like the beads of a rosary, therefore making this "rosary sign" an important diagnostic feature of the vasculitis. PAN is sometimes associated with infection by the hepatitis B or hepatitis C virus. The condition may be present in infants.
Takayasu's arteritis (TA), also known as aortic arch syndrome, nonspecific aortoarteritis, and pulseless disease, is a form of large vessel granulomatous vasculitis with massive intimal fibrosis and vascular narrowing, most commonly affecting young or middle-aged women of Asian descent, though anyone can be affected. It mainly affects the aorta and its branches, as well as the pulmonary arteries. Females are about 8–9 times more likely to be affected than males.
Arteritis is a vascular disorder characterized by inflammation of the walls of arteries, usually as a result of infection or autoimmune responses. Arteritis, a complex disorder, is still not entirely understood. Arteritis may be distinguished by its different types, based on the organ systems affected by the disease. A complication of arteritis is thrombosis, which can be fatal. Arteritis and phlebitis are forms of vasculitis.
Anti-neutrophil cytoplasmic antibodies (ANCAs) are a group of autoantibodies, mainly of the IgG type, against antigens in the cytoplasm of neutrophils and monocytes. They are detected as a blood test in a number of autoimmune disorders, but are particularly associated with systemic vasculitis, so called ANCA-associated vasculitides (AAV).
Cryoglobulinemia is a medical condition in which the blood contains large amounts of pathological cold sensitive antibodies called cryoglobulins – proteins that become insoluble at reduced temperatures. This should be contrasted with cold agglutinins, which cause agglutination of red blood cells.
Polymyalgia rheumatica (PMR) is a syndrome experienced as pain or stiffness, usually in the neck, shoulders, upper arms, and hips, but which may occur all over the body. The pain can be sudden or can occur gradually over a period. Most people with PMR wake up in the morning with pain in their muscles; however, cases have occurred in which the person has developed the pain during the evenings or has pain and stiffness all day long.
Microscopic polyangiitis is an autoimmune disease characterized by a systemic, pauci-immune, necrotizing, small-vessel vasculitis without clinical or pathological evidence of granulomatous inflammation.
Aortitis is the inflammation of the aortic wall. The disorder is potentially life-threatening and rare. It is reported that there are only 1–3 new cases of aortitis per year per million people in the United States and Europe. Aortitis is most common in people 10 to 40 years of age.
Cerebral vasculitis is vasculitis involving the brain and occasionally the spinal cord. It affects all of the vessels: very small blood vessels (capillaries), medium-size blood vessels, or large blood vessels. If blood flow in a vessel with vasculitis is reduced or stopped, the parts of the body that receive blood from that vessel begins to die. It may produce a wide range of neurological symptoms, such as headache, skin rashes, feeling very tired, joint pains, difficulty moving or coordinating part of the body, changes in sensation, and alterations in perception, thought or behavior, as well as the phenomena of a mass lesion in the brain leading to coma and herniation. Some of its signs and symptoms may resemble multiple sclerosis. 10% have associated bleeding in the brain.
Necrotizing vasculitis, also called systemic necrotizing vasculitis, is a general term for the inflammation of veins and arteries that develops into necrosis and narrows the vessels.
Cryofibrinogenemia refers to a condition classified as a fibrinogen disorder in which a person's blood plasma is allowed to cool substantially, causing the (reversible) precipitation of a complex containing fibrinogen, fibrin, fibronectin, and, occasionally, small amounts of fibrin split products, albumin, immunoglobulins and other plasma proteins.
Cutaneous small-vessel vasculitis (CSVV), is inflammation of small blood vessels, usually accompanied by small lumps beneath the skin. The condition is also known as hypersensitivity vasculitis, cutaneous leukocytoclastic vasculitis, hypersensitivity angiitis, cutaneous leukocytoclastic angiitis, cutaneous necrotizing vasculitis and cutaneous necrotizing venulitis,
Cryoglobulinemic vasculitis is a form of inflammation affecting the blood vessels caused by the deposition of abnormal proteins called cryoglobulins. These immunoglobulin proteins are soluble at normal body temperatures, but become insoluble below 37 °C (98.6 °F) and subsequently may aggregate within smaller blood vessels. Inflammation within these obstructed blood vessels is due to the deposition of complement proteins which activate inflammatory pathways.
Hughes–Stovin syndrome (HSS) is a rare autoimmune disorder often described as inflammation in relation to blood vessels, a form of vasculitis. It is not associated with any known cause and is typically characterized by multiple aneurysms in pulmonary arteries and deep vein thromboses. It is named after the two British physicians, John Patterson Hughes and Peter George Ingle Stovin, who first described it in 1959. HSS is presumed to be a rare variant of Behçet's disease, which entails more general problems with the circulatory system. Due to its clinical similarity with Behçet's disease, it has also been referred to as 'Incomplete Behçet's disease.' Most patients are young adult males between the age of 20–40. Common clinical presentations include fever, cough, dyspnea and hemoptysis. Radiological features are similar to those of Behçet's disease.
Lupus vasculitis is one of the secondary vasculitides that occurs in approximately 50% of patients with systemic lupus erythematosus (SLE).
Vasculitic neuropathy is a peripheral neuropathic disease. In a vasculitic neuropathy there is damage to the vessels that supply blood to the nerves. It can be as part of a systemic problem or can exist as a single-organ issue only affecting the peripheral nervous system (PNS). It is diagnosed with the use of electrophysiological testing, blood tests, nerve biopsy and clinical examination. It is a serious medical condition that can cause prolonged morbidity and disability and generally requires treatment. Treatment depends on the type but it is mostly with corticosteroids or immunomodulating therapies.
Avacopan, sold under the brand name Tavneos, is a medication used to treat anti-neutrophil cytoplasmic autoantibody-associated vasculitis. Avacopan is a complement 5a receptor antagonist and a cytochrome P450 3A4 inhibitor.