Vasculitis

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Vasculitis
Other namesVasculitides [1]
HSP Vasculitis.jpg
Petechia and purpura on the lower limb due to infection-associated vasculitis.
Pronunciation
Specialty Rheumatology, Immunology
Symptoms Weight loss, fever, myalgia, purpura, abdominal pain
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.

Contents

Signs and symptoms

The clinical presentation of the various vasculitides on the skin and internal organs is mostly determined by the diameter or size of the vessels mainly affected. [4] Non-specific symptoms are common and include fever, headache, fatigue, myalgia, weight loss, and arthralgia. [5] [6]

All forms of vasculitis, even large vessel vasculitides, may cause skin manifestations. The most common skin manifestations include purpura, nodules, livedo reticularis, skin ulcers, and purpuric urticaria. [7]

TypeNameMain symptoms
Primary large vessel vasculitis [8] Takayasu arteritis Diminished or absent pulses, vascular bruits, hypertension, Takayasu retinopathy, and aortic regurgitation. [9]
Giant cell arteritis Headache, scalp tenderness, jaw claudication, and blindness. [10]
Primary medium vessel vasculitis [8] Polyarteritis nodosa Mononeuritis multiplex, nodules, purpura, livedo, and hypertension. [11]
Kawasaki disease Fever, conjunctivitis, exanthema, palmoplantar erythema, cervical lymphadenopathy, and mucosal enanthema. [12] [13]
Primary small vessel antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis [8] Microscopic polyangiitis Focal segmental rapidly progressive glomerulonephritis, proteinuria, hemoptysis, palpable purpura, abdominal pain, and peripheral neuropathy. [14]
Granulomatosis with polyangiitis Crusting rhinorrhea, sinusitis, chronic otitis media, nasal obstruction, shortness of breath, and chronic cough. [15] [16]
Eosinophilic granulomatosis with polyangiitis Asthma, allergic rhinitis, sinusitis, nasal polyps, peripheral neuropathy, pulmonary infiltrates, and abdominal pain. [17] [18]
Primary immune complex small vessel vasculitis [8] Anti-glomerular basement membrane disease Glomerulonephritis, lung hemorrhage, hematuria, hemoptysis, cough, and dyspnea. [19]
Cryoglobulinemic vasculitis Palpable purpura, Raynaud's phenomenon, joint pain, and peripheral neuropathy. [20]
IgA vasculitis Palpable purpura, arthralgia, abdominal pain, nephritis, and haematuria. [21]
Hypocomplementemic urticarial vasculitis Hives, arthralgia, membranoproliferative glomerulonephritis, and chronic obstructive pulmonary disease. [22]
Primary variable vessel vasculitis [8] Behcet’s disease Oral ulcers, genital ulcers, papulopustular lesions, uveitis, superficial venous thrombosis and deep vein thrombosis. [23]
Cogan’s syndrome Interstitial keratitis, ocular redness, vertigo, and tinnitus. [24]
Single-organ vasculitis [25] [8] Cutaneous small-vessel vasculitis Palpable purpura, necrosis, ulceration, bullae, and nodules. [26]
Cutaneous arteritisNodules, livedo reticularis, ulcers, and gangrene. [27]
Primary central nervous system vasculitisHeadache, cognitive impairment, stroke, encephalopathy, and seizures. [28]
Retinal vasculitis Visual impairments, floaters, and macular edema. [29]
Secondary vasculitis [8] Lupus vasculitis Palpable purpura, petechiae, papulonodular lesions, urticaria lesions, and mononeuritis multiplex. [30]
Rheumatoid vasculitis Purpura, focal digital lesions, ulcers, digital necrosis, pyoderma, distal sensory or motor neuropathy, and mononeuritis multiplex. [31]

Causes

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. [32]

Classification

Primary systemic, secondary, and single-organ vasculitis are distinguished using the highest classification level in the 2012 Chapel Hill Consensus Conference nomenclature. [33]

Primary systemic vasculitis

Primary systemic vasculitis is categorized 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. [33]

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. [33] Takayasu arteritis (TA) and giant cell arteritis (GCA) are the two main forms of LVV. [8]

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. [33] The two primary types are polyarteritis nodosa (PAN) and Kawasaki disease (KD). [8]

Small vessel vasculitis

Small vessel vasculitis (SVV) is separated into immune complex SVV and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). [33]

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). [33]

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. [33] 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

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. [33] This category includes Behcet's disease (BD) and Cogan's syndrome (CS). [8]

Secondary vasculitis

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. [34] Vasculitis frequently coexists with infections, and several infections, including hepatitis B and C, HIV, infective endocarditis, and tuberculosis, are significant secondary causes of vasculitis. [35] Except for rheumatoid vasculitis, the majority of secondary vasculitis forms are exceedingly rare. [36]

Single-organ vasculitis

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. [34]

Diagnosis

Micrograph showing a vasculitis (eosinophilic granulomatosis with polyangiitis). H&E stain. Churg-Strauss syndrome - very high mag.jpg
Micrograph showing a vasculitis (eosinophilic granulomatosis with polyangiitis). H&E stain.
Severe vasculitis of the major vessels, displayed on FDG-PET/CT Vasculitis FDG PET-CT.png
Severe vasculitis of the major vessels, displayed on FDG-PET/CT
  • Some types of vasculitis display leukocytoclasis, which is vascular damage caused by nuclear debris from infiltrating neutrophils. [37] It typically presents as palpable purpura. [37] Conditions with leucocytoclasis mainly include hypersensitivity vasculitis (also called leukocytoclastic vasculitis) and cutaneous small-vessel vasculitis (also called cutaneous leukocytoclastic angiitis).
Laboratory Investigation of Vasculitic Syndromes [40]
DiseaseSerologic testAntigenAssociated laboratory features
Systemic lupus erythematosusANA including antibodies to dsDNA and ENA [including SM, Ro (SSA), La (SSB), and RNP]Nuclear antigensLeukopenia, 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 diseaseAnti-glomerular basement membrane antibodyEpitope on noncollagen domain of type IV collagen
Small vessel vasculitis
Microscopic polyangiitisPerinuclear antineutrophil cytoplasmic antibodyMyeloperoxidaseElevated CRP
Granulomatosis with polyangiitisCytoplasmic antineutrophil cytoplasmic antibodyProteinase 3 (PR3)Elevated CRP
Eosinophilic granulomatosis with polyangiitisperinuclear antineutrophil cytoplasmic antibody in some casesMyeloperoxidaseElevated CRP and eosinophilia
IgA vasculitis (Henoch-Schönlein purpura)None
CryoglobulinemiaCryoglobulins, rheumatoid factor, complement components, hepatitis C
Medium vessel vasculitis
Classical polyarteritis nodosaNoneElevated CRP and eosinophilia
Kawasaki's DiseaseNoneElevated 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

Treatment

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 ]

See also

Related Research Articles

<span class="mw-page-title-main">Giant cell arteritis</span> Inflammatory disease of large blood vessels

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.

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">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">Myeloperoxidase</span> Mammalian protein found in Homo sapiens

Myeloperoxidase (MPO) is a peroxidase enzyme that in humans is encoded by the MPO gene on chromosome 17. MPO is most abundantly expressed in neutrophils, and produces hypohalous acids to carry out their antimicrobial activity, including hypochlorous acid, the sodium salt of which is the chemical in bleach. It is a lysosomal protein stored in azurophilic granules of the neutrophil and released into the extracellular space during degranulation. Neutrophil myeloperoxidase has a heme pigment, which causes its green color in secretions rich in neutrophils, such as mucus and sputum. The green color contributed to its outdated name verdoperoxidase.

<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).

Microscopic polyangiitis is an autoimmune disease characterized by a systemic, pauci-immune, necrotizing, small-vessel vasculitis without clinical or pathological evidence of granulomatous inflammation.

<span class="mw-page-title-main">Friedrich Wegener</span> German pathologist (1907–1990)

Friedrich Wegener was a German pathologist who is notable for his description of a rare disease originally referred to Wegener disease and now referred to as granulomatosis with polyangiitis. Although this disease was known before Wegener's description, from the 1950s onwards it was generally referred to as Wegener's granulomatosis.

<span class="mw-page-title-main">Rapidly progressive glomerulonephritis</span> Medical condition

Rapidly progressive glomerulonephritis (RPGN) is a syndrome of the kidney that is characterized by a rapid loss of kidney function, with glomerular crescent formation seen in at least 50% or 75% of glomeruli seen on kidney biopsies. If left untreated, it rapidly progresses into acute kidney failure and death within months. In 50% of cases, RPGN is associated with an underlying disease such as Goodpasture syndrome, systemic lupus erythematosus or granulomatosis with polyangiitis; the remaining cases are idiopathic. Regardless of the underlying cause, RPGN involves severe injury to the kidneys' glomeruli, with many of the glomeruli containing characteristic glomerular crescents.

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.

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.

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

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.

<span class="mw-page-title-main">Cutaneous small-vessel vasculitis</span> Inflammation of small blood vessels, accompanied by skin bumps

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,

<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.

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

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.

References

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  2. ↑ "Glossary of dermatopathological terms. DermNet NZ". Archived from the original on 20 December 2008. Retrieved 8 January 2009.
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