brand logo

Am Fam Physician. 2011;83(5):556-565

Author disclosure: Nothing to disclose.

The systemic vasculitides are characterized by inflammation of blood vessel walls. Vessels of any type, in any organ can be affected, resulting in a broad spectrum of signs and symptoms. The heterogenous nature of vasculitides presents a diagnostic challenge. The American College of Rheumatology classification criteria and the Chapel Hill Consensus Conference nomenclature are the most widely used to distinguish different forms of vasculitis. The Chapel Hill Consensus Conference nomenclature defines 10 primary vasculitides based on vessel size (large, medium, and small). The diagnosis relies on the recognition of a compatible clinical presentation supported by specific laboratory or imaging tests and confirmatory histology. Antineutrophilic cytoplasmic antibody testing has been of particular benefit in defining a subgroup of small vessel vasculitides. Treatment is based on clinical presentation and the pattern of organ involvement. Glucocorticoids are the primary treatment for many forms of vasculitis. Additional immunosuppressive agents, including methotrexate and cyclophosphamide, are sometimes required. Newer approaches, such as the use of anti-tumor necrosis factor or B cell therapies, are being tried in resistant cases. Patients can experience considerable treatment-related toxicity, especially infection from immunosuppressive therapy and adverse effects from steroids (e.g., osteoporosis, diabetes mellitus, cataract). Vitamin D and calcium prophylaxis are recommended in patients on long-term steroid therapy.

Vasculitis refers to a heterogenous group of disorders in which there is inflammation and damage in blood vessel walls, leading to tissue necrosis. These are relatively uncommon disorders, with a reported annual incidence of 40 to 54 cases per 1 million persons.1 The incidence appears to be affected by geography, age, and seasonal challenges. Vasculitis may be limited to skin or other organs, or may be a multisystem disorder with multiple manifestations.

Clinical recommendationEvidence ratingReferences
The American College of Rheumatology classification criteria and the Chapel Hill Consensus Conference nomenclature may be used to distinguish different forms of vasculitis.C26
The Birmingham Vasculitis Activity Score can be used to monitor the activity of the specific condition during treatment.C9
A definitive diagnosis of systemic vasculitis should be made by the presence of characteristic symptoms and signs of vasculitis and at least one of the following: histologic evidence of vasculitis, positive serology for antineutrophilic cytoplasmic antibody, or specific indirect evidence of vasculitis.C1016
For patients with localized and early vasculitis, treatment with steroids and methotrexate or cyclophosphamide is recommended for induction of remission.C1820, 23
Steroids and cyclophosphamide are recommended for initial treatment of generalized organ-threatening vasculitis.C1820
Patients presenting with severe life-threatening vasculitis (severe renal failure or pulmonary hemorrhage) should be treated with cyclophosphamide (pulsed intravenous or continuous oral) and steroids, with adjuvant plasma exchange.C1823

Classification

Numerous classifications of vasculitis have been proposed. The American College of Rheumatology has classification criteria for seven primary vasculitides: polyarteritis nodosa, Churg-Strauss syndrome, Wegener granulomatosis, hypersensitivity vasculitis, Henoch-Schönlein purpura, giant cell arteritis, and Takayasu arteritis.2 Microscopic polyangiitis is not included in the classification criteria. The American College of Rheumatology criteria were designed for research studies, but are often used for diagnosis, and do not include antineutrophilic cytoplasmic antibody (ANCA) testing for the diagnosis of small vessel vasculitis or require biopsy or angiography for vasculitis classification. The criteria have poor reliability when applied as diagnostic criteria.3

In 1994, the Chapel Hill Consensus Conference proposed a nomenclature defining 10 primary vasculitides based on vessel size (large, medium, and small; Table 1).4 The nomenclature includes microscopic polyangiitis and defines it as vasculitis involving microscopic vessels (arterioles, venules, and small capillaries). Microscopic polyangiitis is distinguished from polyarteritis nodosa by the presence of microscopic vessel involvement. Polyarteritis nodosa must have no involvement of microscopic vessels, including no glomerulonephritis. The nomenclature also stresses the importance of ANCA testing in the diagnosis of vasculitis, particularly in differentiating Wegener granulomatosis and microscopic polyangiitis in persons with pulmonary and renal involvement. The advantage of the Chapel Hill Consensus Conference nomenclature is its clarity and simplicity. However, the usefulness of this nomenclature as diagnostic criteria has been questioned.5,6

There is no recommended diagnostic standard for vasculitis. In the absence of validated diagnostic criteria, the American College of Rheumatology classification criteria and the Chapel Hill Consensus Conference nomenclature are most widely used in clinical practice to distinguish different forms of vasculitis.

Pathogenesis

The pathogenesis of vasculitides is poorly understood. Three possible mechanisms of vascular damage are immune complex deposition, ANCAs (humoral response), and T-lymphocyte response with granuloma formation (cell-mediated).7,8 The end result of these various pathways is endothelial cell activation, with subsequent vessel obstruction and ischemia of dependent tissue. This may cause hemorrhage in the surrounding tissues and, in some cases, weakening of the vessel wall, which leads to the formation of aneurysms. For almost all forms of vasculitis, the triggering event initiating and driving this inflammatory response is unknown. Many small vessel vasculitides have a paucity of vascular immune deposits and, therefore, other mechanisms have been sought for these so-called pauci-immune vasculitides.

Clinical Features

Patients with vasculitis typically have prodromal symptoms, constitutional disturbances, and organ-specific manifestations. Patients can present to the family physician with nonspecific signs or symptoms (e.g., fever, rash, myalgia, arthralgia, malaise, weight loss) or to the emergency department with life-threatening features (e.g., massive hemoptysis, renal failure). Manifestations vary, depending on the size, site, and extent of vessels involved. Clinical manifestations of various forms of vasculitis are discussed in Table 2.

VasculitisOrgan involvementAge (years)Clinical features
Small vessel
Churg-Strauss syndromeRespiratory tract, heart50 to 60Allergic rhinitis, asthma, peripheral eosinophilia
Cryoglobulinemic vasculitisSkin, kidney40 to 50Recurrent palpable purpura, polyarthralgia, glomerulonephritis
Cutaneous leukocytoclastic angiitisSkinAny agePalpable purpura, cutaneous infarcts, necrotic papules, urticaria
Henoch-Schönlein purpuraSkin, gastrointestinal tract, kidney, joint3 to 8Purpura, arthritis, abdominal pain, gastrointestinal bleeding, glomerulonephritis
Microscopic polyangiitisSkin, lung, heart, kidney, liver, gastrointestinal tract50 to 60Palpable purpura, pulmonary hemorrhage, glomerulonephritis
Wegener granulomatosisUpper and lower respiratory tracts, kidney40 to 50Pneumonitis with bilateral nodular and cavitary infiltrates, mucosal ulceration of nasopharynx, chronic sinusitis, glomerulonephritis
Medium vessel
Kawasaki diseaseCoronary arteries, aorta and its branches2 to 4Fever, conjunctivitis, desquamating skin rash, enlarged cervical lymph nodes
Polyarteritis nodosaRenal and visceral organs, spares lung30 to 40Fever, weight loss, hypertension, abdominal pain, melena, peripheral neuritis, renal ischemia
Large vessel
Giant cell arteritisExtracranial branches of carotid artery, often involves temporal artery50 to 60Fever, visual disturbances, facial pain and headache (often along the course of superficial temporal artery)
Takayasu arteritisAorta and its major branches30 to 40More common in young Asian women
Markedly lower blood pressure and weaker pulse in upper extremities, with coldness and numbness of fingers, visual disturbances, hypertension, neurologic deficit

The Birmingham Vasculitis Activity Score is a checklist of signs and symptoms that are consistent with the diagnosis of systemic vasculitis and can be used to monitor the activity of the specific condition during treatment (Table 3).9

FeatureNoneActive disease
General
Arthralgia or arthritis
Fever ≥ 100°F (38°C)
Myalgia
Weight loss ≥ 4 lb (2 kg)
Cutaneous
Gangrene
Infarct
Other skin vasculitis
Purpura
Ulcer
Mucous membranes/eyes
Adnexal inflammation
Blepharitis/keratitis
Blurred vision
Genital ulcers
Mouth ulcers/granulomata
Red eye conjunctivitis/retinal hemorrhages
Red eye (epi)scleritis
Retinal vasculitis
Significant proptosis
Sudden vision loss
Thrombosis/retinal exudates
Uveitis
Ears, nose, throat
Bloody nasal discharge/nasal crusts/conductive hearing loss
Paranasal sinus involvement
Sensorineural hearing loss
Subglottic stenosis
Ulcers or granulomata
Chest
Endobronchial involvement
Hemorrhage
Infiltrate
Massive hemoptysis/alveolar nodules or cavities
Pleural effusion/pleurisy
Respiratory failure
Wheeze
Cardiovascular
Cardiomyopathy
Congestive cardiac failure
Ischemic cardiac pain
Loss of pulses
Pericarditis
Valvular heart disease
Abdominal
Bloody diarrhea
Ischemic abdominal pain
Peritonitis
Renal
Creatinine 1.41 to 2.82 mg per dL (125 to 249 μmol per L)
Creatinine 2.83 to 5.64 mg per dL (250 to 499 μmol per L)
Creatinine ≥ 5.65 mg per dL (500 μmol per L)
Creatinine clearance decrease of > 25 percent
Hematuria (≥ 10 red blood cells per high-power field)
Hypertension
Proteinuria > 1+
Nervous system
Cord lesion
Cranial nerve palsy
Headache
Meningitis
Motor mononeuritis multiplex
Seizures (not hypertensive)
Sensory peripheral neuropathy
Stroke
Other
___________________
___________________
___________________
___________________
Persistent disease only
All the above abnormalities are caused by low-grade disease and not new or worse disease

General Diagnostic Approach

The primary systemic vasculitides are difficult to diagnose because the clinical manifestations mimic several infectious, neoplastic, and autoimmune conditions (Table 4). Attempts should first be made to exclude the malignant and infectious processes. The patient's age, sex, and demographic or ethnic origin are also important. Finally, type and extent of organ involvement and the size of the vessels involved should be determined. Certain organ-specific symptoms, not otherwise explained, may be clues leading to a more specific diagnosis (Table 2). A definitive diagnosis of systemic vasculitis should be made by the presence of characteristic symptoms and signs of vasculitis and at least one of the following: histologic evidence of vasculitis; positive serology for ANCA; or specific indirect evidence of vasculitis.1016 It is important to ascertain that no other diagnosis accounts for the presenting symptoms and signs.

Antiphospholipid syndrome
Atheroembolic disease
Atheromatous vascular disease
Cocaine and amphetamine abuse
Hypersensitivity reactions
Infective endocarditis
Multiple myeloma
Paraneoplastic syndromes
Secondary causes of vasculitis (rheumatoid arthritis, systemic lupus erythematosus, scleroderma, hepatitis B and C infection, lymphoma, and solid organ malignancy)
Sickle cell disease

Laboratory Testing

Laboratory testing is important to determine the organs involved, to exclude other diseases, and to monitor the disease and treatment toxicity. A typical plan of investigation for a patient with vasculitis is summarized in Table 5.

TestIndicationUse
Chemistry
Blood urea nitrogen, creatinine, electrolyte measurementsProteinuria, hematuria, suspicion of glomerulonephritisDiagnosis, disease and treatment toxicity monitoring
Liver functionFever, fatigue, suspicion of infection, monitoring patients treated with hepatotoxic drugs (e.g., methotrexate, azathioprine [Imuran])Diagnosis, disease and treatment toxicity monitoring
Hematology
Clotting screenBleeding or thrombosisDiagnosis, disease monitoring
Complete blood countFever, fatigue, symptoms of bone marrow suppressionDiagnosis, disease and treatment toxicity monitoring
C-reactive protein, erythrocyte sedimentation rateArthralgia, fever, suspicion of giant cell arteritisDiagnosis, disease monitoring
Imaging
AngiographySuspicion of Takayasu arteritis, polyarteritis nodosa, or Kawasaki diseaseDiagnosis, disease monitoring
Chest or organ-specific computed tomography, magnetic resonance imagingGuided by organ-specific clinical symptomsChest computed tomography is diagnostic and used for monitoring of Takayasu arteritis and Kawasaki disease
Computed tomography can be used for monitoring Wegener granulomatosis
Chest radiographyPulmonary infiltrates, nodules, patchy consolidation, pleural effusion, cardiomegalyDiagnosis and monitoring of Wegener granulomatosis, Takayasu arteritis, Kawasaki disease
EchocardiographySuspicion of Kawasaki diseaseDiagnosis of coronary artery abnormality in Kawasaki disease
Microbiology
Hepatitis B and C serology, human immunodeficiency virus testingHepatitis or immunodeficiencyDiagnosis of hepatitis B virus–related polyarteritis nodosa and hepatitis C virus–related cryoglobulinemic vasculitis
Urine cultureAbnormal urinalysisDisease monitoring
Serology
Anticardiolipin antibodiesBleeding, thrombosis, fetal lossDiagnosis,* disease monitoring
Antineutrophilic cytoplasmic antibodies, and if positive, specific target antigen(proteinase 3 or myeloperoxidase)Suspicion of microscopic polyangiitis, Churg-Strauss syndrome, or Wegener granulomatosisDiagnosis, disease monitoring
Antinuclear antibodyArthralgia, myalgiaDiagnosis,* disease monitoring
Complement (C3 and C4)Arthralgia, myalgiaDisease monitoring
CryoglobulinsPurpura, arthritis, hepatitis CDiagnosis of cryoglobulinemic vasculitis
Immunoglobulins and protein electrophoresisArthralgia, bone lesions, defective immune statusDiagnosis*
Rheumatoid factorArthralgia, myalgiaDiagnosis*
Tissue biopsy
Biopsy of involved organ or tissueGuided by organ-specific clinical symptoms(e.g., skin, sinuses, lung, artery, nerve, kidney)Definitive diagnosis in some vasculitides, such as Wegner granulomatosis and giant cell arteritis
Urinalysis
Analysis and microscopyFever, leukocytosis, suspicion of cystitis or renal involvementDiagnosis, disease and treatment toxicity monitoring
Other
Nerve conduction test, electromyographic testMotor and sensory neuropathyDiagnosis, disease monitoring

COMPLETE BLOOD COUNT

Patients with active vasculitis often have leukocytosis, anemia, and thrombocytopenia. Eosinophilia is a prominent feature in Churg-Strauss syndrome. A complete blood count is important to look for the bone marrow suppression that may result from vasculitis treatment.

ACUTE PHASE REACTANTS

An increased erythrocyte sedimentation rate and an elevated C-reactive protein level are common in patients with vasculitis, but are nonspecific and can occur in many settings, particularly infection. A normal erythrocyte sedimentation rate and C-reactive protein level can occur in vasculitis when the disease is inactive, and should not exclude the diagnosis. In patients with giant cell arteritis, an increased erythrocyte sedimentation rate can suggest the diagnosis and can be useful for disease monitoring when combined with compatible clinical features.

RENAL FUNCTION TESTS AND URINALYSIS

Blood urea nitrogen and serum creatinine measurements should be obtained, and urinalysis should be performed in any patient with suspected vasculitis. Proteinuria and hematuria suggest the possibility of glomerulonephritis. Monitoring creatinine and the urinalysis is useful to detect changes in disease activity. It may be helpful to identify bladder toxicity in patients treated with cyclophosphamide.

LIVER FUNCTION TESTS

Serum bilirubin and liver enzyme levels (aspartate and alanine transaminase, alkaline phosphatase, γ-glutamyltransferase) can provide clues for vasculitis that affects the liver, such as polyarteritis nodosa. Serial liver function tests are also important in monitoring patients treated with hepatotoxic drugs, such as methotrexate and azathioprine (Imuran).

ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES

Serum from many patients with vasculitis shows the presence of ANCAs, which are a heterogeneous group of autoantibodies directed against enzymes found in neutrophils. There are two types of ANCAs based on indirect immunofluorescence patterns: cytoplasmic and perinuclear. The most common corresponding antigens identified by enzyme immunoassay are proteinase 3 for cytoplasmic ANCA and myeloperoxidase for perinuclear ANCA. All positive ANCA results based on immunofluorescence should be confirmed by enzyme immunoassay.10,11

The disorders characterized by circulating ANCAs are called ANCA-associated vasculitides, and include Churg-Strauss syndrome, microscopic polyangiitis, and Wegener granulomatosis. Either type of ANCA may occur in a patient with ANCA-associated small vessel vasculitis, but cytoplasmic ANCA is typically found in Wegener granulomatosis, and perinuclear ANCA is typically found in microscopic polyangiitis and Churg-Strauss syndrome. ANCAs are useful quantitative markers for these conditions, and the level reflects the degree of inflammation.12 As such, ANCAs increase during recurrence and are useful in monitoring response to treatment.13,14 Persistence of ANCAs is a predictor of poor response to treatment. Approximately 10 percent of patients with Wegener granulomatosis or microscopic polyangiitis have negative assays for ANCAs; therefore, a negative result does not completely rule out these diseases.17 In addition, ANCAs have been reported in other conditions, such as infections, inflammatory bowel disease, and drug-induced vasculitis.

Imaging

CHEST RADIOGRAPHY

Nonspecific abnormalities that can be seen on chest radiography include infiltrates, nodules, patchy consolidation, pleural effusion, and cardiomegaly. These findings can occur in many settings, but if unexplained, may raise the suspicion of vasculitis.

ANGIOGRAPHY

Angiography can show vascular occlusion and aneurysm. A diagnosis of polyarteritis nodosa can be confirmed by detection of aneurysms in mesenteric and renal arteries. Although conventional angiography is still accepted as the recommended diagnostic modality, computed tomography angiography and magnetic resonance angiography may be superior because they can provide valuable information regarding intraluminal pathology and thickening of the vessel wall. These techniques have been used for diagnosis and monitoring of Takayasu arteritis and Kawasaki disease.

ECHOCARDIOGRAPHY

Transthoracic echocardiography detects coronary artery abnormality in Kawasaki disease. Approximately 40 percent of children with Kawasaki disease have coronary artery lesions (ectasia or aneurysm) on echocardiography.15 Echocardiography is used for assessing coronary artery blood flow and the degree of coronary stenosis in patients with Takayasu arteritis.16

ULTRASONOGRAPHY

Ultrasonography may be useful for diagnosis and monitoring of large vessel vasculitis. Patients with giant cell arteritis may have stenosis, occlusion, or halo sign (a dark area around the artery from vessel wall edema) of the superficial temporal artery.

COMPUTED TOMOGRAPHY

Computed tomography is of diagnostic value in patients with sinonasal Wegener granulomatosis. Findings include thickening of nasal mucosa and punctate bony destruction, mainly in the midline. Nodules or masses can be seen on chest computed tomography in approximately 90 percent of patients with Wegener granulomatosis.18

Other Testing

NERVE CONDUCTION TESTING

Motor and sensory neuropathy can occur in the context of systemic vasculitis when the vasa nervorum are affected (e.g., polyarteritis nodosa, Wegener granulomatosis, Churg-Strauss syndrome). Nerve conduction testing should be used for the evaluation of neurologic manifestations.

TISSUE BIOPSY

The definitive diagnosis of vasculitis is established by biopsy of the involved tissue (e.g., skin, sinuses, lung, artery, nerve, kidney), which determines the pattern of vessel inflammation. The presence of immunoglobulins and complement found by immunofluorescence on the tissue section may be helpful in elucidating the specific type of vasculitis. Biopsies are particularly valuable in ruling out other causes, but a negative biopsy does not rule out vasculitis.

Treatment

The initial presentation may be to the family physician or any number of subspecialists, including a dermatologist, rheumatologist, nephrologist, otolaryngologist, or pulmonologist. Most patients are treated by a rheumatologist experienced with these diseases. The rheumatologist works with the patient's family physician, who monitors the disease progression and complications of treatment.

The treatment includes three phases: induction of remission, maintenance, and treatment of relapse. The severity and extent of the disease divides patients into three groups: those with localized or early disease, those with generalized disease with threatened organ involvement, and those with severe or life-threatening disease. For patients with localized and early disease, treatment with steroids and methotrexate or cyclophosphamide is recommended for induction of remission.1820 Methotrexate may be associated with a higher relapse rate.21,22 Evidence of relapse or disease progression despite treatment with methotrexate requires the use of cyclophosphamide. Initial treatment of generalized organ-threatening disease should include steroids and cyclophosphamide.1820 Cyclophosphamide can be administered as an intravenous infusion every two weeks (and later every three weeks), or as a daily low-dose oral treatment. There is no difference in remission rates or relapse risk between oral and intravenous regimens. Steroids are given as daily oral prednisone (1 mg per kg, up to 60 mg daily). Pulsed intravenous steroids can be given just before or with the first two intravenous pulses of cyclophosphamide. Patients presenting with severe life-threatening disease (severe renal failure or pulmonary hemorrhage) should be treated with cyclophosphamide (pulsed intravenous or continuous oral) and steroids, with adjuvant plasma exchange.19,23

Maintenance therapy with either azathioprine or methotrexate is initiated if remission has occurred after three to six months of induction therapy. Steroid dosage is tapered during this phase. Patients may need to continue maintenance treatment for up to 24 months.24 Maintenance treatment for up to five years is recommended in patients with Wegener granulomatosis and patients who remain ANCA-positive.19 Some patients may require treatment indefinitely. Disease relapse may occur anytime after the remission. Serial measurements of ANCA are not closely associated with disease activity; therefore, treatment should not be solely guided on the basis of an increase in ANCA.25 Relapsing disease can be managed with an increase in steroid dose, optimization of the current immunosuppressant, or combination of an immunosuppressant with an increased dose of steroid.

Novel biologic therapies targeted against specific components of the immune system are being used for systemic vasculitis, particularly for patients in whom conventional therapy has failed. Agents such as infliximab (Remicade; human chimeric anti-tumor necrosis factor [TNF]-α monoclonal antibody), etanercept (Enbrel; fusion protein of the p75 TNF-α receptor and immunoglobulin G1), adalimumab (Humira; fully humanized IgG1 anti-TNF-α monoclonal antibody), rituximab (Rituxan; anti-CD20 chimeric mouse/human monoclonal antibody), anakinra (Kineret; recombinant interleukin-1 receptor antagonist), and intravenous immune globulins may be used in refractory disease.26,27

Patients with systemic vasculitis are at increased risk of comorbidities resulting from disease-related end organ damage and immunosuppressive therapy. The immunosuppressive medications used for the treatment of systemic vasculitis cause serious adverse effects during the first year of therapy. Steroids and cyclophosphamide predispose patients to life-threatening infections. Cyclophosphamide can cause hemorrhagic cystitis, ovarian and testicular failure, and bladder cancer. Diagnosis and treatment of these complications are coordinated with the family physician. Recommendations regarding detecting and preventing these complications include use of mesna (Mesnex) for protecting against urothelial toxicity of cyclophosphamide, antifungal prophylaxis, prophylaxis against Pneumocystis jiroveci, consideration for Staphylococcus aureus treatment, screening for cervical malignancy, and counseling about infertility with cyclophosphamide. Adverse effects of long-term steroid use (e.g., diabetes mellitus, osteoporosis, cataract) should be assessed. Vitamin D and calcium prophylaxis are recommended in patients on long-term therapy with steroids. Table 6 summarizes the drugs and treatments for systemic vasculitis.23

VasculitisDrug/treatmentIndication
Small vessel
Antineutrophilic cytoplasmic antibody–associated small vessel vasculitis (Churg-Strauss syndrome, microscopic polyangiitis, Wegener granulomatosis)PrednisoloneFirst-line therapy in conjunction with cyclophosphamide in generalized disease; first-line therapy in localized/early disease
MethylprednisoloneSevere vasculitis with rapidly progressive glomerulonephritis
MethotrexateFirst-line therapy in conjunction with steroids in localized/early disease
CyclophosphamideFirst-line therapy in generalized disease, aggressive local disease, and life-threatening disease
PlasmapheresisProgressive severe renal disease
Intravenous immune globulinRefractory disease
Azathioprine (Imuran)Refractory or relapsing disease
Biologic therapy used alone or in combination with standard treatment (infliximab [Remicade], rituximab [Rituxan], antithymocyte globulin)Refractory or relapsing disease
Intravenous immune globulinRefractory or relapsing disease
Interferon alfaRefractory or relapsing disease
Trimethoprim/sulfamethoxazole (Bactrim, Septra)In conjunction with prednisolone and cyclophosphamide for Pneumocystis jiroveci
Prophylaxis
BisphosphonateBone protection with long-term steroid
Cutaneous leukocytoclastic angiitisAntihistaminics plus nonsteroidal anti-inflammatory drugsSymptom control in absence of systemic disease
PrednisoloneSevere cutaneous or systemic disease
Essential cryoglobulinemic vasculitisInterferon alfa plus oral ribavirinHepatitis C–related cryoglobulinemic vasculitis
Therapy same as antineutrophilic cytoplasmic antibody–associated vasculitisNonviral-related cryoglobulinemic vasculitis
Henoch-Schönlein purpuraSteroids plus cyclophosphamideHenoch-Schönlein purpura with nephritis (most cases without renal involvement resolve spontaneously)
Medium vessel
Kawasaki diseaseIntravenous immune globulin with aspirinFirst-line therapy
Intravenous immune globulin plus heparin infusionSecond-line therapy in patients who do not initially respond to intravenous immune globulin and aspirin combination
Methylprednisolone followed by prednisoloneSecond-line therapy
Polyarteritis nodosaPrednisoloneFirst-line therapy
MethylprednisoloneFulminant disease
CyclophosphamideFirst-line therapy (used in combination with steroids in non–hepatitis B-associated polyarteritis nodosa)
Antiviral agents (interferon alfa plus lamivudine [Epivir])Hepatitis B–associated polyarteritis nodosa
PlasmapheresisHepatitis B–associated polyarteritis nodosa
BisphosphonateBone protection with long-term steroid
Large vessel
Giant cell arteritis and Takayasu arteritisPrednisoloneFirst-line in active Takayasu arteritis and in giant cell arteritis without eye symptoms
MethylprednisoloneConsider in giant cell arteritis with significant visual disturbance
MethotrexateAdjunct to steroids for maintenance therapy
Reduces risk of first or second relapse
Decreased cumulative dose of steroids
Allows earlier discontinuation of steroids
AzathioprineAdjunct to prednisolone for maintenance therapy
BisphosphonateBone protection with long-term steroid
AspirinIn conjunction with maintenance therapy for prevention of cerebrovascular and cardiovascular ischemic events

Management of systemic vasculitis is complicated. Educating patients about signs and symptoms, and monitoring typical adverse effects are helpful. Many patients will have a relatively benign, self-limited course, especially if the disease is limited to the skin; however, for patients with aggressive disease, such as ANCA-associated small vessel vasculitis, it is imperative to begin treatment without delay. The multisystem involvement in systemic vasculitis necessitates a multidisciplinary team approach to patient care. Recent advances in therapy have led to considerably better outcomes in patients with vasculitis.

Continue Reading


More in AFP

More in Pubmed

Copyright © 2011 by the American Academy of Family Physicians.

This content is owned by the AAFP. A person viewing it online may make one printout of the material and may use that printout only for his or her personal, non-commercial reference. This material may not otherwise be downloaded, copied, printed, stored, transmitted or reproduced in any medium, whether now known or later invented, except as authorized in writing by the AAFP.  See permissions for copyright questions and/or permission requests.