Systemic vasculitis
Systemic vasculitis | |
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Other names | Necrotizing vasculitis |
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]
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]
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
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 does not affect 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 (Churg-Strauss) (EGPA), granulomatosis with polyangiitis (Wegener's) (GPA), and microscopic polyangiitis (MPA).[4]
Eosinophilic granulomatosis with polyangiitis (Churg–Strauss, EGPA) is a systemic small-vessel vasculitis linked to eosinophilia and asthma. Peripheral neuropathy, 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 vasculitis that primarily affects small vessels and has moderate to significant
Hypocomplementemic
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]
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 (
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]
Signs and symptoms
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]
Patients with
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]
Many cases of
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
Oral
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
In order 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 Churg-Strauss syndrome 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
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
Systemic vasculitides, such as
A
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
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