Vasculitis

Template:Short description Template:Use dmy dates Template:Use American English Template:Infobox medical condition (new) Vasculitis is a group of disorders that destroy blood vessels by inflammation.<ref name="urlGlossary of dermatopathological terms. DermNet NZ">{{#invoke:citation/CS1|citation |CitationClass=web }}</ref> Both arteries and veins are affected. Lymphangitis (inflammation of lymphatic vessels) is sometimes considered a type of vasculitis.<ref>Template:DorlandsDict</ref> 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.

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.<ref name="Braun-Falco´s">Template:Cite book</ref> Non-specific symptoms are common and include fever, headache, fatigue, myalgia, weight loss, and arthralgia.<ref name="Internal Medicine">Template:Cite book</ref><ref name="Jayne diagnosis 2009">Template:Cite journal</ref>

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.<ref name="What Do We Have to Know?">Template:Cite journal</ref>

Type	Name	Main symptoms
Primary large vessel vasculitis<ref name="Overview of the 2012 revised International Chapel Hill Consensus"/>	Takayasu arteritis	Diminished or absent pulses, vascular bruits, hypertension, Takayasu retinopathy, and aortic regurgitation.<ref name="Johnston 2024">Template:Cite journal</ref>
	Giant cell arteritis	Headache, scalp tenderness, jaw claudication, and blindness.<ref name="Hoffman 2016">Template:Cite journal</ref>
Primary medium vessel vasculitis<ref name="Overview of the 2012 revised International Chapel Hill Consensus"/>	Polyarteritis nodosa	Mononeuritis multiplex, nodules, purpura, livedo, and hypertension.<ref name="Forbess 2015">Template:Cite journal</ref>
	Kawasaki disease	Fever, conjunctivitis, exanthema, palmoplantar erythema, cervical lymphadenopathy, and mucosal enanthema.<ref name="Son 2013">Template:Cite journal</ref><ref name="Hedrich 2018">Template:Cite journal</ref>
Primary small vessel antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis<ref name="Overview of the 2012 revised International Chapel Hill Consensus"/>	Microscopic polyangiitis	Focal segmental rapidly progressive glomerulonephritis, proteinuria, hemoptysis, palpable purpura, abdominal pain, and peripheral neuropathy.<ref name="Chung 2010">Template:Cite journal</ref>
	Granulomatosis with polyangiitis	Crusting rhinorrhea, sinusitis, chronic otitis media, nasal obstruction, shortness of breath, and chronic cough. <ref name="Comarmond 2014">Template:Cite journal</ref><ref name="Grygiel-Górniak 2018">Template:Cite journal</ref>
	Eosinophilic granulomatosis with polyangiitis	Asthma, allergic rhinitis, sinusitis, nasal polyps, peripheral neuropathy, pulmonary infiltrates, and abdominal pain.<ref name="Vaglio 2013">Template:Cite journal</ref><ref name="White 2023">Template:Cite journal</ref>
Primary immune complex small vessel vasculitis<ref name="Overview of the 2012 revised International Chapel Hill Consensus"/>	Anti-glomerular basement membrane disease	Glomerulonephritis, lung hemorrhage, hematuria, hemoptysis, cough, and dyspnea.<ref name="Bharati 2024">Template:Cite journal</ref>
	Cryoglobulinemic vasculitis	Palpable purpura, Raynaud's phenomenon, joint pain, and peripheral neuropathy.<ref name="Silva 2019">Template:Cite journal</ref>
	IgA vasculitis	Palpable purpura, arthralgia, abdominal pain, nephritis, and haematuria.<ref name="Pillebout 2021">Template:Cite journal</ref>
	Hypocomplementemic urticarial vasculitis	Hives, arthralgia, membranoproliferative glomerulonephritis, and chronic obstructive pulmonary disease.<ref name="Gu 2021">Template:Cite journal</ref>
Primary variable vessel vasculitis<ref name="Overview of the 2012 revised International Chapel Hill Consensus"/>	Behçet's disease	Oral ulcers, genital ulcers, papulopustular lesions, uveitis, superficial venous thrombosis and deep vein thrombosis.<ref name="Bettiol 2020">Template:Cite journal</ref>
	Cogan syndrome	Interstitial keratitis, ocular redness, vertigo, and tinnitus.<ref name="Iliescu 2024">Template:Cite journal</ref>
Single-organ vasculitis<ref name="Localized Forms">Template:Cite journal</ref><ref name="Overview of the 2012 revised International Chapel Hill Consensus"/>	Cutaneous small-vessel vasculitis	Palpable purpura, necrosis, ulceration, bullae, and nodules.<ref name="Russell 2006">Template:Cite journal</ref>
	Cutaneous arteritis	Nodules, livedo reticularis, ulcers, and gangrene.<ref name="Furukawa 2012">Template:Cite journal</ref>
	Primary central nervous system vasculitis	Headache, cognitive impairment, stroke, encephalopathy, and seizures.<ref name="Junek 2023">Template:Cite journal</ref>
	Retinal vasculitis	Visual impairments, floaters, and macular edema.<ref name="Abu El-Asrar 2005">Template:Cite journal</ref>
Secondary vasculitis<ref name="Overview of the 2012 revised International Chapel Hill Consensus"/>	Lupus vasculitis	Palpable purpura, petechiae, papulonodular lesions, urticaria lesions, and mononeuritis multiplex.<ref name="Leone 2021">Template:Cite journal</ref>
	Rheumatoid vasculitis	Purpura, focal digital lesions, ulcers, digital necrosis, pyoderma, distal sensory or motor neuropathy, and mononeuritis multiplex.<ref name="Bartels 2010">Template:Cite journal</ref>

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.<ref name="Pathobiology of Human Disease">Template:Cite book</ref>

Classification

Primary systemic, secondary, and single-organ vasculitis are distinguished using the highest classification level in the 2012 Chapel Hill Consensus Conference nomenclature.<ref name="Chapel Hill Consensus">Template:Cite journal</ref>

Primary systemic vasculitis

{{#invoke:Labelled list hatnote|labelledList|Main article|Main articles|Main page|Main pages}} 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.<ref name="Chapel Hill Consensus"/>

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.<ref name="Chapel Hill Consensus"/> Takayasu arteritis (TA) and giant cell arteritis (GCA) are the two main forms of LVV.<ref name="Overview of the 2012 revised International Chapel Hill Consensus">Template:Cite journal</ref>

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.<ref name="Chapel Hill Consensus"/> The two primary types are polyarteritis nodosa (PAN) and Kawasaki disease (KD).<ref name="Overview of the 2012 revised International Chapel Hill Consensus"/>

Small vessel vasculitis

Small vessel vasculitis (SVV) is separated into immune complex SVV and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).<ref name="Chapel Hill Consensus"/>

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).<ref name="Chapel Hill Consensus"/>

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.<ref name="Chapel Hill Consensus"/> 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.<ref name="Overview of the 2012 revised International Chapel Hill Consensus"/>

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.<ref name="Chapel Hill Consensus"/> This category includes Behcet's disease (BD) and Cogan's syndrome (CS).<ref name="Overview of the 2012 revised International Chapel Hill Consensus"/>

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.<ref name="Classification and classification criteria">Template:Cite journal</ref> Vasculitis frequently coexists with infections, and several infections, including hepatitis B and C, HIV, infective endocarditis, and tuberculosis, are significant secondary causes of vasculitis.<ref name="Diagnostic approach">Template:Cite journal</ref> Except for rheumatoid vasculitis, the majority of secondary vasculitis forms are exceedingly rare.<ref name="How to diagnose and treat secondary forms of vasculitis">Template:Cite journal</ref>

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.<ref name="Classification and classification criteria"/>

Diagnosis

File:Churg-Strauss syndrome - very high mag.jpg

Micrograph showing a vasculitis (eosinophilic granulomatosis with polyangiitis). H&E stain.

File:Vasculitis FDG PET-CT.png

Severe vasculitis of the major vessels, displayed on FDG-PET/CT

• Laboratory tests of blood or body fluids are performed for patients with active vasculitis. Their results will generally show signs of inflammation in the body, such as increased erythrocyte sedimentation rate (ESR), elevated C-reactive protein (CRP), anemia, increased white blood cell count and eosinophilia. Other possible findings are elevated antineutrophil cytoplasmic antibody (ANCA) levels and hematuria.
• Other organ functional tests may be abnormal. Specific abnormalities depend on the degree of various organs involvement. A brain SPECT can show decreased blood flow to the brain and brain damage.
• The definite diagnosis of vasculitis is established after a biopsy of involved organ or tissue, such as skin, sinuses, lung, nerve, brain, and kidney. The biopsy elucidates the pattern of blood vessel inflammation.

• Some types of vasculitis display leukocytoclasis, which is vascular damage caused by nuclear debris from infiltrating neutrophils.<ref name=medscape-leukocytoclasis>{{#invoke:citation/CS1|citation

|CitationClass=web }} Updated: 25 October 2018</ref> It typically presents as palpable purpura.<ref name=medscape-leukocytoclasis/> Conditions with leucocytoclasis mainly include hypersensitivity vasculitis (also called leukocytoclastic vasculitis) and cutaneous small-vessel vasculitis (also called cutaneous leukocytoclastic angiitis).

• An alternative to biopsy can be an angiogram (x-ray test of the blood vessels). It can demonstrate characteristic patterns of inflammation in affected blood vessels.
• 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT)has become a widely used imaging tool in patients with suspected Large Vessel Vasculitis, due to the enhanced glucose metabolism of inflamed vessel walls.<ref>Template:Cite journal</ref> The combined evaluation of the intensity and the extension of FDG vessel uptake at diagnosis can predict the clinical course of the disease, separating patients with favourable or complicated progress.<ref>Template:Cite journal</ref>
• Acute onset of vasculitis-like symptoms in small children or babies may instead be the life-threatening purpura fulminans, usually associated with severe infection.

Laboratory Investigation of Vasculitic Syndromes<ref>Template:Cite book</ref>

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

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, 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.Template:Cn

See also

• Golfer's vasculitis