Systemic vasculitis

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

Classification

Large-vessel vasculitis

The 2012 Chapel Hill Consensus Conference defines large vessel vasculitis 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. Takayasu arteritis and giant cell arteritis are the two main forms of LVV.
Takayasu arteritis is a large-vessel, idiopathic, granulomatous arteritis that primarily affects the aorta, significant branches of it, and the pulmonary arteries. The disease's symptoms can range from catastrophic neurological impairment to an asymptomatic condition brought on by impalpable pulses or bruits. Non-specific features include mild anemia, myalgia, arthralgia, weight loss, malaise, night sweats, and fever.
Giant cell arteritis is the most common type of systemic vasculitis in adults. Polymyalgia rheumatica, headache, jaw claudication, and visual symptoms are the classic manifestations; however, 40% of patients present with a variety of occult manifestations.

Medium vessel vasculitis

Medium vessel vasculitis 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. The two primary types are polyarteritis nodosa and Kawasaki disease.
Polyarteritis nodosa 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.
Kawasaki disease 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.

Small vessel vasculitis

Small vessel vasculitis is separated into immune complex SVV and antineutrophil cytoplasmic antibody -associated vasculitis.
Antineutrophil cytoplasmic antibody -associated vasculitis 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, granulomatosis with polyangiitis, and microscopic polyangiitis.
Eosinophilic granulomatosis with polyangiitis 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.
Granulomatosis with polyangiitis, formerly known as Wegener's granulomatosis, 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.
Microscopic polyangiitis 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. The most typical features of microscopic polyangiitis are renal manifestations and general symptoms; lung involvement is also frequently observed.
Immune complex small vessel vasculitis is a vasculitis that primarily affects small vessels and has moderate to significant immunoglobulin and complement component deposits on the vessel wall. Hypocomplementemic urticarial vasculitis, cryoglobulinemic vasculitis, IgA vasculitis, and anti-glomerular basement membrane disease are the categories of immune complex SVV.
Hypocomplementemic urticarial vasculitis syndrome 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.
Cryoglobulinemic vasculitis 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. Cryoglobulins type II and III, also known as mixed cryoglobulinemia, are composed of polyclonal immunoglobulin 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.
Immunoglobulin A 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.
Anti-glomerular basement membrane 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 syndrome".

Variable vessel vasculitis

Variable vessel vasculitis is a kind of vasculitis that may impact vessels of all sizes and any type, with no particular type of vessel being predominantly affected. This category includes Behcet's disease and Cogan's syndrome.
Behçet's disease is a systemic illness marked by frequent episodes of severe inflammation. Genital ulcerations, uveitis, oral aphthous ulcers, and skin lesions are the main symptoms.
Cogan's syndrome 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. An upper respiratory tract infection, or less frequently, diarrhea, a dental infection, or an immunization, precedes the onset of the disease.

History

Kussmaul and Maier gave the first detailed description of systemic necrotizing arteritis in 1866.
In 1919 Karl Theodor Fahr described acute arterial lesions which were always present in malignant nephrosclerosis. These injuries were the most severe and widespread in the kidneys and occurred less frequently and severely in other organs, particularly in the pancreas, adrenals and intestines. Fahr believed that necrotizing arteriolitis was the primary cause of the lesions, malignant necrosis and hypertension in these cases by narrowing the renal vascular bed.

Signs and symptoms

, constitutional abnormalities, and organ-specific manifestations are common in vasculitis patients. Patients may show up at the emergency room with life-threatening symptoms or with nonspecific signs and symptoms at their family physician's office. The size, location, and extent of the vessels involved all affect the manifestations.
Takayasu arteritis 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.
Giant cell arteritis 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.
Polyarteritis nodosa can affect one organ or cause systemic failure as its clinical manifestation. Although any tissue may be impacted, PAN rarely affects the lungs for unclear reasons. 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.
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 diffuse 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.
Malaise, arthralgia, sinusitis, and rhinitis are typically present at the beginning of Anti-neutrophil cytoplasmic antibody -associated vasculitis. Prodromes often occur weeks or months before pulmonary-renal syndrome.
Anti-glomerular basement membrane vasculitis patients usually report sudden onset of anuria or oliguria. Typically, hematuria or tea-colored urine are noticed.
Many cases of cryoglobulinemia vasculitis are asymptomatic. 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.
Ninety-five percent of cases of immunoglobulin A vasculitis start with a skin rash. Additionally, the illness manifests as the standard tripartite of symptoms pertaining to the gastrointestinal, renal, and musculoskeletal systems.
Recurrent urticaria, with skin eruptions primarily affecting the trunk, face, and upper extremities, is the primary clinical manifestation of hypocomplementemic urticarial vasculitis.
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.
Often, the upper respiratory tract infection is the initial sign of Cogan's syndrome. Ocular and audio-vestibular symptoms are typical indicators. Non-syphilitic interstitial keratitis, uveitis, retinal vasculitis, conjunctivitis, scleritis, tinnitus, hearing loss, and vertigo are among the range of ocular manifestations.