Systemic vasculitis

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Systemic vasculitis
Other namesNecrotizing vasculitis
Vasculitis 2.jpg
A case of vasculitis on legs
Specialty Immunology, rheumatology

Necrotizing vasculitis, also called systemic necrotizing vasculitis, [1] is a general term for the inflammation of veins and arteries that develops into necrosis and narrows the vessels. [2]

Contents

Tumors, medications, allergic reactions, and infectious organisms are some of the recognized triggers for these conditions, even though the precise cause of many of them is unknown. Immune complex disease, anti-neutrophil cytoplasmic antibodies, anti-endothelial cell antibodies, and cell-mediated immunity are examples of pathogenetic factors. [2]

Numerous secondary symptoms of vasculitis can occur, such as thrombosis, aneurysm formation, bleeding, occlusion of an artery, loss of weight, exhaustion, depression, fever, and widespread pain that worsens in the morning. [2]

Systemic vasculitides are categorized as small, medium, large, or variable based on the diameter of the vessel they primarily affect. [3]

Classification

Large-vessel vasculitis

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. [4] Takayasu arteritis (TA) and giant cell arteritis (GCA) are the two main forms of LVV. [5]

Takayasu arteritis (TA) is a large-vessel, idiopathic, granulomatous arteritis that primarily affects the aorta, significant branches of it, and (less frequently) the pulmonary arteries. [6] The disease's symptoms can range from catastrophic neurological impairment to an asymptomatic condition brought on by impalpable pulses or bruits. [7] Non-specific features include mild anemia, myalgia, arthralgia, weight loss, malaise, night sweats, and fever. [8]

Giant cell arteritis (GCA) is the most common type of systemic vasculitis in adults. Polymyalgia rheumatica (PMR), headache, jaw claudication, and visual symptoms are the classic manifestations; however, 40% of patients present with a variety of occult manifestations. [9]

Medium vessel vasculitis

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. [4] The two primary types are polyarteritis nodosa (PAN) and Kawasaki disease (KD). [5]

Polyarteritis nodosa (PAN) is a type of systemic necrotizing vasculitis that primarily affects arteries of medium size. While small vessels like arterioles, capillaries, and venules are not affected, small arteries can be. The disease spectrum varies from failure of multiple organs to involvement of a single organ. Almost any organ could be impacted; however, polyarteritis nodosa rarely affects the lungs for unknown reasons. [10]

Kawasaki disease (KD) is a type of systemic vasculitis of medium-sized vessels with an acute onset that primarily affects young children. Fever, conjunctivitis, infection of the skin and mucous membranes, and cervical lymphadenopathy are the main symptoms. [11]

Small vessel vasculitis

Small vessel vasculitis (SVV) is separated into immune complex SVV and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). [4]

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 (EGPA), granulomatosis with polyangiitis (GPA), and microscopic polyangiitis (MPA). [4]

Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic small-vessel vasculitis linked to eosinophilia and asthma. Polyneuropathy, cardiac involvement, skin lesions, involvement of the upper respiratory tract, and lung are typical presentations of eosinophilic granulomatosis with polyangiitis. [12]

Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis (WG), is a rare immune-mediated systemic disease with an unclear etiology. It manifests pathologically as an inflammatory response pattern in the kidneys, upper and lower respiratory tracts, and granulomatous inflammation, which includes necrosis. [13]

Microscopic polyangiitis (MPA) belongs to the group of vasculitides associated with ANCA. Its distinct histology reveals a pauci-immune vasculitis, or necrotizing small vessel vasculitis, with minimal or no immune deposits. [14] The most typical features of microscopic polyangiitis are renal manifestations and general symptoms; lung involvement is also frequently observed. [15]

Immune complex small vessel vasculitis (SVV) is a vasculitis that primarily affects small vessels and has moderate to significant immunoglobulin and complement component deposits on the vessel wall. [4] Hypocomplementemic 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. [5]

Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare immune complex-mediated condition that has persistent acquired hypocomplementemia and urticaria. Many systemic manifestations are linked to hypocomplementemic urticarial vasculitis syndrome, such as leukocytoclastic vasculitis, glomerulonephritis, laryngeal edema, severe angioedema, pulmonary involvement, arthritis, arthralgia, and uveitis. [16]

Cryoglobulinemic vasculitis (CryoVas) is a type of small-vessel vasculitis that primarily affects the kidneys, skin, joints, and peripheral nervous system. Monoclonal immunoglobulins associated with an underlying B-cell lymphoproliferative disorder are known as type I cryovalent vasculitis (CryoVas). Cryoglobulins type II and III, also known as mixed cryoglobulinemia, are composed of polyclonal immunoglobulin (Ig)G and either monoclonal IgM or both with rheumatoid factor activity. The disease can present with a wide range of symptoms, from minor ones like fatigue, purpura, or arthralgia to more serious ones like glomerulonephritis and widespread vasculitis that can be fatal. [17]

Immunoglobulin A (IgA) vasculitis, formerly referred to as Henoch–Schönlein purpura, is a type of immune complex vasculitis that primarily affects IgA deposits in small vessels. Acute enteritis, glomerulonephritis, arthralgias and/or arthritis, and cutaneous purpura are the most common clinical manifestations. Children are more likely than adults to develop IgA vasculitis, and adults tend to have a more severe case. [18]

Anti–glomerular basement membrane (anti-GBM) disease is an uncommon kind of small vessel vasculitis that affects the kidney and lung capillary beds. This illness is also known by its eponym, "Goodpasture sydrome." [19]

Variable vessel vasculitis

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. [4] This category includes Behcet's disease (BD) and Cogan's syndrome (CS). [5]

Behçet’s disease (BD) is a systemic illness marked by frequent episodes of severe inflammation. Genital ulcerations, uveitis, oral aphthous ulcers, and skin lesions are the main symptoms. [20]

Cogan's syndrome (CS) is an uncommon type of autoimmune systemic vasculitis that causes inflammation inside the eyes and malfunctions the vestibulo-auditory system, usually resulting in neurosensory deafness but also tinnitus and vertigo. [21] An upper respiratory tract infection, or less frequently, diarrhea, a dental infection, or an immunization, precedes the onset of the disease. [22]

Signs and symptoms

Prodromal symptoms, constitutional abnormalities, and organ-specific manifestations are common in vasculitis patients. Patients may show up at the emergency room with life-threatening symptoms (such as massive hemoptysis or renal failure) or with nonspecific signs and symptoms (such as a rash, fever, myalgia, arthralgia, malaise, or weight loss) at their family physician's office. The size, location, and extent of the vessels involved all affect the manifestations. [23]

Takayasu arteritis (TA) is typically documented in three distinct phases. There are generalized constitutional inflammatory symptoms during the first stage. Patients may report fever of unknown cause during this phase. Patients may refer to dorsal and thoracic pain in the following phase, and infrequently, neck pain as well. Arterial bruits, intermittent extremity claudication, decreased or absent pulses, and/or variations in arterial blood pressure among upper extremities are the hallmarks of the final phase. [24]

Giant cell arteritis (GCA) often exhibits a wide range of symptoms in its early stages, all of which are related to the localized consequences of systemic and vascular inflammation. The symptoms of GCA include jaw claudication, headaches, and tenderness in the scalp. The most common symptom is headache, which is restricted to the temporal region. [3]

Polyarteritis nodosa (PAN) can affect one organ or cause systemic failure as its clinical manifestation. [25] Although any tissue may be impacted, PAN rarely affects the lungs for unclear reasons. [26] A variety of clinical indicators, including common symptoms like fever, chills, weight loss, myalgia, and arthralgia, are typically present when PAN first manifests. Peripheral nerves and skin are typically involved in PAN. Skin manifestations include purpura, necrotic ulcers, subcutaneous nodules, and livedoid. Mononeuritis multiplex is the main neurological symptom, typically presenting as a drop in the foot or wrist. [3]

Patients with Kawasaki disease often have a fever between 38 and 40 degrees Celsius and often show no prodromal symptoms. Within two to four days of the illness starting, bilateral conjunctival injections without exudate become visible. The term "modifications of the oral cavity" usually refers to conditions such as diffused erythema of the oropharyngeal mucosa, strawberry-like tongue without vesicles or pseudo-membrane formation, bleeding of the lips, redness, fissuring, and dryness. From the first to the fifth day following the onset of fever, polymorphous erythema appears on the body and/or extremities. [3]

Malaise, arthralgia, sinusitis, and rhinitis are typically present at the beginning of Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Prodromes often occur weeks or months before pulmonary-renal syndrome. [3]

Anti-glomerular basement membrane (anti-GBM) vasculitis patients usually report sudden onset of anuria or oliguria. Typically, hematuria or tea-colored urine are noticed. [3]

Many cases of cryoglobulinemia vasculitis are asymptomatic. [27] Hyperviscosity and/or thrombosis are the principal signs and symptoms of type I cryoglobulinemia. As a result, the conditions most frequently manifested as Raynaud's phenomenon, distal gangrene, ischemic ulcers, purpura, livedo reticularis, headache, retinal hemorrhages, and encephalopathy. Nonspecific systemic and musculoskeletal symptoms, such as cutaneous vasculitis and neuropathy, can also be seen in patients with mixed cryoglobulinemia. [28]

Ninety-five percent of cases of immunoglobulin A vasculitis (IgAV) start with a skin rash. [29] Additionally, the illness manifests as the standard tripartite of symptoms pertaining to the gastrointestinal, renal, and musculoskeletal systems. [30]

Recurrent urticaria, with skin eruptions primarily affecting the trunk, face, and upper extremities, is the primary clinical manifestation of hypocomplementemic urticarial vasculitis (HUV). [31]

Oral aphthae are the defining feature of Behçet's disease and manifest in 98% of patients. Compared to oral lesions, genital aphthae are less common. [3]

Often, the upper respiratory tract infection is the initial sign of Cogan's syndrome. [3] Ocular and audio-vestibular symptoms are typical indicators. Non-syphilitic interstitial keratitis (IK), uveitis, retinal vasculitis, conjunctivitis, scleritis, tinnitus, hearing loss, and vertigo are among the range of ocular manifestations. [32]

Diagnosis

To confirm the diagnosis, the initial evaluation consists of a thorough clinical assessment, serological tests, histology when possible, and radiography when necessary. [33]

Individuals experiencing active vasculitis frequently exhibit anemia, thrombocytopenia, and leukocytosis. One of the main characteristics of eosinophilic granulomatosis with polyangiitis is eosinophilia. [23]

Patients with vasculitis frequently have increased erythrocyte sedimentation rate and elevated C-reactive protein levels; however, these symptoms are nonspecific and can arise in a variety of circumstances, most notably infection. When vasculitis is not active, normal erythrocyte sedimentation rate or C-reactive protein level can occur and should not rule out the diagnosis. When paired with congruent clinical features, an elevated erythrocyte sedimentation rate in giant cell arteritis patients can both support the diagnosis and aid in disease monitoring. [23]

In any patient suspected of having vasculitis, measurements of blood urea nitrogen, serum creatinine, and urine should be taken. Hematuria and proteinuria raise the risk of glomerulonephritis. Serum bilirubin and liver enzyme levels (ó-glutamyltransferase, alkaline phosphatase, and aspartate and alanine transaminase) can give indications for liver-related vasculitis, like polyarteritis nodosa. [23]

Antineutrophil cytoplasmic antibodies (ANCAs) are a diverse collection of autoantibodies that target neutrophil enzymes and have been detected in the serum of many vasculitis patients. The conditions known as ANCA-associated vasculitides, which are characterized by circulating ANCAs, comprise granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis. [23]

Chest radiography may reveal nonspecific abnormalities such as cardiomegaly, patchy consolidation, nodules, and infiltrates. These results can happen in a variety of situations, but if they go undiagnosed, they could point to vasculitis. [23]

Aneurysms and vascular occlusion can be seen with angiography. Polyarteritis nodosa can be verified by looking for aneurysms in the renal and mesenteric arteries. While conventional angiography remains the accepted standard diagnostic modality, there is potential for superiority with computed tomography angiography and magnetic resonance angiography, as they can offer important insights into intraluminal pathology and vessel wall thickening. These methods have been applied to Kawasaki disease and Takayasu arteritis diagnosis and follow-up. [23]

When Kawasaki disease is present, transthoracic echocardiography can identify coronary artery abnormalities. [23] Echocardiography reveals coronary artery lesions (ectasia or aneurysm) in about 40% of children with Kawasaki disease. [34] In patients with Takayasu arteritis, echocardiography is used to measure the degree of coronary stenosis and coronary artery blood flow. [35]

For the diagnosis and ongoing observation of large vessel vasculitis, ultrasonography may be helpful. Individuals diagnosed with giant cell arteritis may present with superficial temporal artery stenosis, occlusion, or halo sign (a dark patch surrounding the artery due to vessel wall edema). [23]

When diagnosing patients with granulomatosis with polyangiitis, computed tomography is useful. Results include destruction of punctate bone, primarily in the midline, and thickening of the nasal mucosa. [23] In about 90% of patients with granulomatosis with polyangiitis, a chest computed tomography scan will show nodules or masses. [36]

Systemic vasculitides, such as polyarteritis nodosa, granulomatosis with polyangiitis, and eosinophilic granulomatosis with polyangiitis, can result in motor and sensory neuropathy. Neurologic manifestations should be evaluated by nerve conduction testing. [23]

A biopsy of the affected tissue (such as the skin, the sinuses, lung, artery, nerve, or kidney) is used to make a definitive diagnosis of vasculitis by identifying the pattern of vessel inflammation. Determining the precise type of vasculitis may be made easier by looking for immunoglobulins and complement on the tissue section as detected by immunofluorescence. Although a negative biopsy cannot rule out vasculitis, biopsies are especially useful in ruling out other causes. [23]

Treatment

Treatment is targeted to the underlying cause. However, most vasculitis, in general, are treated with steroids (e.g., methylprednisolone) because the underlying cause of the vasculitis is due to hyperactive immunological damage. Immunosuppressants such as cyclophosphamide and azathioprine may also be given.

A systematic review of antineutrophil cytoplasmic antibody-positive vasculitis identified the best treatments depending on whether the goal is to induce remission or maintenance and depending on the severity of the vasculitis. [37]

Related Research Articles

Rheumatology is a branch of medicine devoted to the diagnosis and management of disorders whose common feature is inflammation in the bones, muscles, joints, and internal organs. Rheumatology covers more than 100 different complex diseases, collectively known as rheumatic diseases, which includes many forms of arthritis as well as lupus and Sjögren's syndrome. Doctors who have undergone formal training in rheumatology are called rheumatologists.

<span class="mw-page-title-main">Vasculitis</span> Medical disorders that destroy blood vessels by inflammation

Vasculitis is a group of disorders that destroy blood vessels by inflammation. Both arteries and veins are affected. Lymphangitis is sometimes considered a type of vasculitis. 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.

<span class="mw-page-title-main">Granulomatosis with polyangiitis</span> Autoimmune disease with chronic blood vessel inflammation

Granulomatosis with polyangiitis (GPA), previously 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.

<span class="mw-page-title-main">Eosinophilic granulomatosis with polyangiitis</span> Medical condition

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).

<span class="mw-page-title-main">Kawasaki disease</span> Disease found in young children

Kawasaki disease is a syndrome of unknown cause that results in a fever and mainly affects children under 5 years of age. It is a form of vasculitis, where medium-sized blood vessels become inflamed throughout the body. The fever typically lasts for more than five days and is not affected by usual medications. Other common symptoms include large lymph nodes in the neck, a rash in the genital area, lips, palms, or soles of the feet, and red eyes. Within three weeks of the onset, the skin from the hands and feet may peel, after which recovery typically occurs. The disease is the leading cause of acquired heart disease in children in developed countries, which include the formation of coronary artery aneurysms and myocarditis.

<span class="mw-page-title-main">Polyarteritis nodosa</span> Systemic necrotizing inflammation of medium-sized muscular arteries

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.

<span class="mw-page-title-main">Takayasu's arteritis</span> Medical condition

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.

<span class="mw-page-title-main">Arteritis</span> Medical condition

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.

<span class="mw-page-title-main">Anti-neutrophil cytoplasmic antibody</span> Group of autoantibodies

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).

<span class="mw-page-title-main">Cryoglobulinemia</span> Presence of cold-sensitive antibodies in the blood

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.

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.

An overlap syndrome is a medical condition which shares features of at least two more widely recognised disorders. Examples of overlap syndromes can be found in many medical specialties such as overlapping connective tissue disorders in rheumatology, and overlapping genetic disorders in cardiology.

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.

Pulmonary-renal syndrome (PRS) is a rare medical syndrome in which respiratory failure involving bleeding in the lungs and kidney failure (glomerulonephritis) occur. PRS is associated with a high rate of morbidity and death. The term was first used by Goodpasture in 1919 to describe the association of respiratory and kidney failure.

Pauci-immune vasculitis is a form of vasculitis that is associated with minimal evidence of hypersensitivity upon immunofluorescent staining for IgG. Often, this is discovered in the setting of the kidney.

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.

<span class="mw-page-title-main">Retinal vasculitis</span> Medical condition

Retinal vasculitis is inflammation of the vascular branches of the retinal artery, caused either by primary ocular disease processes, or as a specific presentation of any systemic form of vasculitis such as Behçet's disease, sarcoidosis, multiple sclerosis, or any form of systemic necrotizing vasculitis such as temporal arteritis, polyarteritis nodosa, and granulomatosis with polyangiitis, or due to lupus erythematosus, or rheumatoid arthritis. Eales disease, pars planitis, birdshot retinochoroidopathy, and Fuchs heterochromic iridocyclitis (FHI) can also cause retinal vasculitis. Infectious pathogens such as Mycobacterium tuberculosis, visceral larva migrans can also cause retinal vasculitis. Drug-induced vasculitis may involve retina as well, as seen in methamphetamine induced vasculitis.

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.

References

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  24. ↑ KESER, Gökhan; AKSU, Kenan; DİRESKENELİ, Haner (August 16, 2018). "Takayasu arteritis: an update". Turkish Journal of Medical Sciences. 48 (4). The Scientific and Technological Research Council of Turkey (TUBITAK-ULAKBIM) - DIGITAL COMMONS JOURNALS: 681–697. doi:10.3906/sag-1804-136. hdl: 11424/219927 . ISSN   1300-0144. PMID   30114347. S2CID   52014268.
  25. ↑ Howard, Travis; Ahmad, Kinza; Swanson, Jerome (Allen) A.; Misra, Sanjay (2014). "Polyarteritis Nodosa". Techniques in Vascular and Interventional Radiology. 17 (4). Elsevier BV: 247–251. doi:10.1053/j.tvir.2014.11.005. ISSN   1089-2516. PMC   4363102 . PMID   25770638.
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  27. ↑ TREJO, OLGA; RAMOS-CASALS, MANUEL; GARCÍA-CARRASCO, MARIO; YAGÜE, JORDI; JIMÉNEZ, SÒNIA; DE LA RED, GLORIA; CERVERA, RICARD; FONT, JOSEP; INGELMO, MIGUEL (2001). "Cryoglobulinemia". Medicine. 80 (4). Ovid Technologies (Wolters Kluwer Health): 252–262. doi:10.1097/00005792-200107000-00004. ISSN   0025-7974. PMID   11470986.
  28. ↑ Ramos-Casals, Manuel; Trejo, Olga; García-Carrasco, Mario; Cervera, Ricard; Font, Josep (2000). "Mixed cryoglobulinemia: new concepts". Lupus. 9 (2). SAGE Publications: 83–91. doi:10.1191/096120300678828127. ISSN   0961-2033. PMID   10787003. S2CID   19390158.
  29. ↑ Nong, Bao-Ren; Huang, Yung-Feng; Chuang, Chih-Ming; Liu, Chia-Chia; Hsieh, Kai-Sheng (August 2007). "Fifteen-year experience of children with Henoch-Schönlein purpura in southern Taiwan, 1991-2005". Journal of Microbiology, Immunology, and Infection. 40 (4): 371–376. PMID   17712473.
  30. ↑ Eleftheriou, Despina; Brogan, Paul A. (2009). "Vasculitis in children". Best Practice & Research Clinical Rheumatology. 23 (3). Elsevier BV: 309–323. doi:10.1016/j.berh.2009.02.001. ISSN   1521-6942. PMC   7106032 . PMID   19508940.
  31. ↑ Buck, Andrew; Christensen, Jim; McCarty, Morgan (2012). "Hypocomplementemic Urticarial Vasculitis Syndrome: A Case Report and Literature Review". The Journal of Clinical and Aesthetic Dermatology. 5 (1). Matrix Medical Communications: 36–46. PMC   3277093 . PMID   22328958.
  32. ↑ Kessel, Aharon; Vadasz, Zahava; Toubi, Elias (2014). "Cogan syndrome — Pathogenesis, clinical variants and treatment approaches". Autoimmunity Reviews. 13 (4–5). Elsevier BV: 351–354. doi:10.1016/j.autrev.2014.01.002. ISSN   1568-9972. PMID   24418297.
  33. ↑ Miller, Anne; Basu, Neil; Luqmani, Raashid (2008). "Assessment of systemic vasculitis". Autoimmunity Reviews. 8 (2). Elsevier BV: 170–175. doi:10.1016/j.autrev.2008.07.001. ISSN   1568-9972. PMID   18672099.
  34. ↑ Baer, Aryeh Z.; Rubin, Lorry G.; Shapiro, Craig A.; Sood, Sunil K.; Rajan, Sujatha; Shapir, Yehuda; Romano, Angela; Bierman, Fredrick Z. (July 1, 2006). "Prevalence of Coronary Artery Lesions on the Initial Echocardiogram in Kawasaki Syndrome". Archives of Pediatrics & Adolescent Medicine. 160 (7). American Medical Association (AMA): 686–690. doi:10.1001/archpedi.160.7.686. ISSN   1072-4710. PMID   16818833. S2CID   32194867.
  35. ↑ Espinola-Zavaleta, Nilda; Soto, María Elena; Bauk, Lucas; Miguel Casanova, José; Keirns, Candace; Vanzzini, Nydia Avila; Reyes, Pedro (2005). "Coronary Reserve in Takayasu's Arteritis: Transesophageal Echocardiographic Analysis". Echocardiography. 22 (7). Wiley: 593–598. doi:10.1111/j.1540-8175.2005.40105.x. ISSN   0742-2822. PMID   16060896.
  36. ↑ Nataraja, Anjali; Mukhtyar, Chetan; Hellmich, Bernhard; Langford, Carol; Luqmani, Raashid (2007). "Outpatient assessment of systemic vasculitis". Best Practice & Research Clinical Rheumatology. 21 (4). Elsevier BV: 713–732. doi:10.1016/j.berh.2007.01.004. ISSN   1521-6942. PMID   17678832.
  37. ↑ Bosch X, Guilabert A, Espinosa G, Mirapeix E (2007). "Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systematic review". JAMA. 298 (6): 655–69. doi: 10.1001/jama.298.6.655 . PMID   17684188.
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