Vasculitis

Vasculitis is a group of disorders that destroy blood vessels by inflammation. Both arteries and veins are affected. Lymphangitis (inflammation of lymphatic vessels) is sometimes considered a type of vasculitis. Vasculitis is primarily caused by leukocyte migration and resultant damage. Although both occur in vasculitides, 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. Non-specific symptoms are common and include fever, headache, fatigue, myalgia, weight loss, and arthralgia.

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

{| class="wikitable plainrowheaders"

!scope="col"|Type

!scope="col"|Name

!scope="col"|Main symptoms

!scope="row" rowspan="2" |Primary large vessel vasculitis

|Giant cell arteritis

|Headache, scalp tenderness, jaw claudication, and blindness.

!scope="row" rowspan="2" |Primary medium vessel vasculitis

|Kawasaki disease

|Fever, conjunctivitis, exanthema, palmoplantar erythema, cervical lymphadenopathy, and mucosal enanthema.

!scope="row" rowspan="3" |Primary small vessel antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis

|Granulomatosis with polyangiitis

|Crusting rhinorrhea, sinusitis, chronic otitis media, nasal obstruction, shortness of breath, and chronic cough.

|Eosinophilic granulomatosis with polyangiitis

|Asthma, allergic rhinitis, sinusitis, nasal polyps, peripheral neuropathy, pulmonary infiltrates, and abdominal pain.

!scope="row" rowspan="4" |Primary immune complex small vessel vasculitis

|Cryoglobulinemic vasculitis

|Palpable purpura, Raynaud's phenomenon, joint pain, and peripheral neuropathy.

|IgA vasculitis

|Palpable purpura, arthralgia, abdominal pain, nephritis, and haematuria.

|Hypocomplementemic urticarial vasculitis

|Hives, arthralgia, membranoproliferative glomerulonephritis, and chronic obstructive pulmonary disease.

!scope="row" rowspan="2" |Primary variable vessel vasculitis

|Cogan syndrome

|Interstitial keratitis, ocular redness, vertigo, and tinnitus.

!scope="row" rowspan="4" |Single-organ vasculitis

|Cutaneous arteritis

|Nodules, livedo reticularis, ulcers, and gangrene.

|Primary central nervous system vasculitis

|Headache, cognitive impairment, stroke, encephalopathy, and seizures.

|Retinal vasculitis

|Visual impairments, floaters, and macular edema.

!scope="row" rowspan="2" |Secondary vasculitis

|Rheumatoid vasculitis

|Purpura, focal digital lesions, ulcers, digital necrosis, pyoderma, distal sensory or motor neuropathy, and mononeuritis multiplex.

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.

Classification

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

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 vasculitides, small-vessel vasculitides, and variable-vessel vasculitides.

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

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 vasculitides. It typically presents as palpable purpura. 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.

Acute onset of vasculitis-like symptoms in small children or babies may instead be the life-threatening purpura fulminans, usually associated with severe infection.

{| class="wikitable"

|+ Laboratory Investigation of Vasculitic Syndromes

! 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.