Version May 2024
INTRODUCTION — Vasculitis is defined as the presence of inflammation in a blood vessel that may occur as a primary process or secondary to an underlying disease. Clinical symptoms vary widely depending upon the type and location of the vessels involved and the extent of inflammation [1]. Because of the multisystem nature of these disorders, many pediatric subspecialties, including rheumatology, dermatology, nephrology, pulmonology, and cardiology, may be involved in the diagnostic work-up and ongoing care of a child with vasculitis. Prompt recognition and treatment of these disorders are important as they can be severe and life-threatening conditions without appropriate management.
The approach to evaluating a child with suspected vasculitis is reviewed here. The classification and overview of the management of childhood vasculitis are discussed separately. (See "Vasculitis in children: Incidence and classification" and "Vasculitis in children: Management overview".)
CLINICAL PRESENTATION — Clinical symptoms vary widely depending upon the types and location of vessels involved, the extent of inflammation, and the degree to which vascular integrity has been compromised. Many patients ultimately diagnosed with a specific vasculitis will have presented initially with a combination of fever or other constitutional symptoms or signs (eg, weight loss, weakness, fatigue) and laboratory evidence of inflammation in addition to skin lesions. These early features are neither specific nor sensitive for vasculitis and may be found in other conditions including common infections. In patients with such nonspecific systemic symptoms, vasculitis should be suspected if symptoms do not resolve or improve within days to weeks, as would be expected in a "self-limited" infectious illness. In addition to systemic symptoms, the presence of multiorgan involvement, in the absence of an infectious cause, should heighten one's suspicion of vasculitis, and certain patterns of organ involvement in the presence of inflammation may be diagnostic. (See "Vasculitis in children: Incidence and classification", section on 'Classification criteria'.)
Kawasaki disease and immunoglobulin A (IgA) vasculitis (Henoch-Schönlein purpura), the most common forms of primary vasculitis in childhood, are described as acute vasculitides [2]. Although their disease courses are usually self-limited, the risk for sustained cardiac damage in Kawasaki disease and potential for persisting nephritis in IgA vasculitis demand early recognition to mitigate the effects of these potential problems. Thus, familiarity with the characteristic features of these diseases is imperative. The combination of lower-extremity purpura, abdominal pain, joint pain, and glomerulonephritis suggests the diagnosis of IgA vasculitis (picture 1), whereas the syndrome of persistent fever, conjunctivitis, rash, mucocutaneous changes, and swelling of the hands and feet suggests the diagnosis of Kawasaki disease (picture 2 and picture 3 and picture 4 and picture 5). Recognition of these combinations of features is often sufficient to make a "syndromic" diagnosis. Caregivers should be alert to evolving combinations of features when only a few features are initially present. (See "IgA vasculitis (Henoch-Schönlein purpura): Clinical manifestations and diagnosis", section on 'Diagnosis' and "Kawasaki disease: Clinical features and diagnosis".)
The other primary vasculitides are chronic (eg, granulomatosis with polyangiitis, polyarteritis nodosa, microscopic polyangiitis, Takayasu arteritis) and relatively rare in childhood. Although characteristic clinical findings typical for individual vasculitides have been described, children may have incomplete or overlapping diagnostic features. In pediatric registry studies from the 1990s [3,4] and a pediatric rheumatologist survey from 2005 [5], respectively two-thirds and one-third of children with vasculitis were considered to have an "unclassified" vasculitis according to the adult-based American College of Rheumatology (ACR) classification criteria.
The differences between adult- and pediatric-onset vasculitis are now better characterized [6], and more pediatric patients with vasculitis are classifiable for a number of reasons: the development of a pediatric–specific classification for vasculitis [7]; improved disease nomenclature and framework for classification [8]; the incorporation of new mechanistic knowledge, particularly for small-vessel vasculitis, such as the presence of specific antineutrophil cytoplasmic antibodies or the prominence of immune complexes in the vessel wall [8]; and the discovery and description of several monogenic autoinflammatory syndromes primarily manifesting as vasculitis [9,10].
REFERRAL — Children who present with features consistent with IgA vasculitis or Kawasaki disease can be managed for the most part by general pediatricians. For those patients with more severe manifestations, or with vasculitis features that are less typical of IgA vasculitis or Kawasaki disease, referral should be made to a tertiary care center where the expertise of pediatric rheumatologists, nephrologists, dermatologists, and other subspecialists can be sought.
DIFFERENTIATING PRIMARY VASCULITIS FROM OTHER DISORDERS — The majority of children with vasculitis are diagnosed by pattern recognition because the most common vasculitides, IgA vasculitis and Kawasaki disease, have typical combinations of features that are diagnostic (see 'Clinical presentation' above). However, the diagnosis is more challenging in children with clinical features seen in chronic systemic vasculitis. These cases require a high index of suspicion since the features may overlap with common disorders, vasculitis may occur as a secondary feature of other systemic disorders, and vasculitic features may be mimicked by other conditions [11]. In addition, the clinical presentation varies widely depending upon the type and location of the vessels involved, the extent of inflammation, and subsequent vessel wall damage with associated hemodynamic changes (table 1).
Differential diagnosis — Because of the rarity of the primary vasculitides (other than the acute vasculitis syndromes such as Kawasaki disease or IgA vasculitis), a patient with clinical features "suggestive" of chronic vasculitis is more likely to have another disease.
In the early stages of presentation, the differential diagnosis is broad (table 2) [11]:
●Drugs – Antimicrobials, biologic agents, diuretics, anticonvulsants, and antithyroid medications
●Infections:
•Bacterial/fungal – Subacute bacterial endocarditis and bacteremia, particularly meningococcemia, and Group A streptococcal infections (see "Clinical manifestations of meningococcal infection")
•Viral – Human immunodeficiency virus (HIV), hepatitis, cytomegalovirus, Epstein-Barr virus, parvovirus B19, and herpes zoster/varicella, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)
•Other organisms – Tuberculosis, syphilis, Rocky Mountain spotted fever, typhus, and rickettsial pox
●Malignancy – Lymphoma, leukemia
●Rheumatologic disorders:
•Systemic lupus erythematosus (SLE) (see "Childhood-onset systemic lupus erythematosus (SLE): Clinical manifestations and diagnosis")
•Systemic juvenile idiopathic arthritis (see "Systemic juvenile idiopathic arthritis: Clinical manifestations and diagnosis")
•Juvenile dermatomyositis (see "Juvenile dermatomyositis and other idiopathic inflammatory myopathies: Epidemiology, pathogenesis, and clinical manifestations")
•Sarcoidosis (see "Clinical manifestations and diagnosis of sarcoidosis")
●Kidney disorders – Anti-glomerular basement membrane (GBM) disease (Goodpasture's)
●Monogenic disorders:
•Adenosine deaminase 2 (ADA2) deficiency (see "Adenosine deaminase deficiency: Pathogenesis, clinical manifestations, and diagnosis")
•STING-associated vasculopathy with onset in infancy (SAVI) (see "Autoinflammatory diseases mediated by interferon production and signaling (interferonopathies)", section on 'STING-associated vasculopathy with onset in infancy (SAVI)')
•Haploinsufficiency of A20 (HA20 disease) presenting in early childhood as a Behçet-like syndrome [12] (see "Autoinflammatory diseases mediated by NFkB and/or aberrant TNF activity", section on 'Haploinsufficiency of A20')
Even in the presence of apparent vasculitic lesions, there are noninflammatory mechanisms involving blood vessels that may cause similar findings [13]. For example, cutaneous lesions (eg, purpura) may be caused by vascular occlusion. The following noninflammatory mechanisms may mimic vasculitis:
●Lesions outside the vessel wall – Neurofibromatosis (see "Neurofibromatosis type 1 (NF1): Pathogenesis, clinical features, and diagnosis")
●Lesions inside the vessel wall – Thrombocytopenia, thrombotic conditions (eg, antiphospholipid syndrome), and embolic conditions (eg, atrial myxoma) (see "Approach to the child with unexplained thrombocytopenia" and "Clinical manifestations of antiphospholipid syndrome")
●Spasm of the vessels – Drug-induced vasospasm (eg, ergot, cocaine, phenylpropanolamine) and Raynaud phenomenon (see "Clinical manifestations and diagnosis of Raynaud phenomenon")
●Abnormal vessels themselves – Coarctation of the aorta and fibromuscular dysplasia (see "Clinical manifestations and diagnosis of coarctation of the aorta")
Differentiating the above listed mimics and secondary causes of vasculitis from primary vasculitis often requires a comprehensive evaluation while considering the differential diagnostic possibilities, including taking a complete history of symptoms and recent exposures (drugs or infectious) and thorough review of systems, alongside a careful physical examination and investigative work-up. A diagnosis of primary vasculitis can only be made once secondary causes or mimics of vasculitis have been eliminated. Monogenic disorders mimicking vasculitis are rare but should be considered in cases that present in infancy or very early childhood [12,14-17].
Further evaluation including imaging or histologic review of tissue samples from involved organs may be necessary to make a more definitive diagnosis, especially in children with severe disease who may require therapy that has potentially serious adverse effects.
The diagnostic approach for each individual primary vasculitis is discussed in detail separately. (See appropriate topic reviews.)
Initial evaluation — The initial evaluation while differentiating other disorders (see 'Differential diagnosis' above) includes a detailed history, comprehensive physical examination, and basic laboratory testing for all children and adolescents suspected of having vasculitis.
History — Constitutional symptoms, such as malaise, fatigue, weight loss, and fever, are frequently present; however, they are nonspecific. A careful and detailed history should identify which organ systems are affected and the extent and severity of their involvement.
The history should include the following:
●A thorough review of each major organ system to determine whether organs other than those implicated by the presenting complaint are involved and also to determine if there is a specific pattern of findings that may be suggestive or diagnostic of a particular disease.
●Recent illness, particularly infections, either in the patient or in close contacts.
●Recent travel to a tuberculosis-endemic country, which would raise suspicion of a mycobacterial infection.
●Exposure to any medications or toxins that may cause secondary vasculitis.
●History of any predisposing condition for secondary vasculitis, such as hepatitis, SLE, juvenile dermatomyositis, or malignancy.
●A timeline of the symptoms. Children with IgA vasculitis and Kawasaki disease tend to have acute presentations with characteristic signs and symptoms, whereas children with other systemic vasculitides may present with a longer duration of symptoms that evolve and progress over time. Children with major organ involvement can present with variable durations of symptoms. These children may be extremely unwell, requiring admission to the hospital or the intensive care unit.
●Presentation with systemic inflammation in early life with skin vasculitis should raise the suspicion of a monogenic disorder such as deficiency of adenosine deaminase 2 (DADA2) or SAVI [14-17], and, if oral or genital ulcerations are present, HA20 should be considered [12]. (See 'Differential diagnosis' above.)
Physical examination — A thorough physical examination may reveal involvement of organs or systems that was not apparent from the medical history. Specific identified abnormalities (below) may support a diagnosis of vasculitis, whereas other findings such as hepatosplenomegaly or widespread lymphadenopathy may be more characteristic of a malignancy or a systemic infection.
The physical examination should include:
●Palpation of all pulses for volume and symmetry (reduced or absent pulses may be seen in Takayasu arteritis).
●Measurement of blood pressure (BP). BPs should be assessed manually and with the appropriate-sized cuff to optimize accuracy. In addition, BPs should be measured in all four limbs because a significant asymmetry (difference of >10 mmHg between limbs) raises the suspicion of Takayasu arteritis. BP values are compared with normative BP percentiles based upon data on sex, age, height, and BP measurements from the National Health and Nutrition Examination Survey (NHANES) and other population-based studies (table 3 and table 4). Elevated BP may be a marker of kidney involvement, which can occur in several vasculitides. (See "Definition and diagnosis of hypertension in children and adolescents".)
●Examination of the ocular fundi and periungual capillary beds for evidence of vascular abnormalities (picture 6). Referral to an ophthalmologist in cases of suspected vasculitis is warranted, especially in the presence of any ocular symptoms.
●Auscultation of the neck, abdomen, and extremities to detect the presence of a bruit, which suggests altered blood flow through an affected vessel.
●Examination of the respiratory system. Observe for signs of respiratory distress and assess oxygen saturation. Auscultate for stridor or other adventitious sounds.
●Examination of the skin for lesions suggestive of vascular insufficiency or inflammation, such as palpable purpura, livedo reticularis (picture 7), nodules, ulcers, and nonblanching rashes.
●Neurologic examination, especially evaluating for peripheral neuropathy.
Laboratory evaluation — There are no specific laboratory tests to diagnose vasculitis. However, diagnosing active vasculitis in the absence of any evidence of inflammation is difficult.
The initial laboratory analysis should include the following:
●Complete blood count and differential – Elevated white blood cell count may be indicative of inflammation due to vasculitis or infection. Anemia can occur during inflammation or as part of a chronic disease.
●Platelet count – To eliminate thrombocytopenia as the cause of the purpuric or petechial skin lesions. Platelets are also nonspecifically raised during inflammation.
●Urinalysis – An abnormal urine sediment (eg, hematuria) may be the only sign of kidney involvement in some cases of systemic vasculitis. Examination of a fresh-spun urine sample provides the highest yield for detecting dysmorphic red blood cells or red blood cell casts that are indicative of glomerulonephritis, the more severe manifestation of several types of vasculitis. (See "Glomerular disease: Evaluation in children".)
●Liver enzymes – To detect hepatic involvement.
●Serum creatinine and blood urea nitrogen – To detect kidney involvement and evaluate kidney function.
●Erythrocyte sedimentation rate and/or C-reactive protein – As indicators of inflammation.
Clinical correlation with vessel size — Once diseases or conditions mimicking vasculitis or causing secondary vasculitis have been excluded and following comprehensive clinical history, physical examination, and basic laboratory evaluation, the next step is to reconcile whether the findings are consistent with one of the known types of primary vasculitis.
The primary types of vasculitis are commonly classified and differentiated according to vessel size [8]. Nonspecific complaints (ie, fever, fatigue, anorexia, weight loss, and arthralgias) are generally present early in the course of the disease, irrespective of the size of the involved vessels. Clinical manifestations identified by repeat evaluations may become evident over time as the disease evolves, and these findings may correlate with the size of vessels involved.
●Large- and medium-size vessels – When predominately large- or medium-sized vessels are involved (such as in patients with Takayasu arteritis or polyarteritis nodosa), symptoms and clinical manifestations of arterial insufficiency to the affected organs become apparent. Involvement of large vessels to the extremities, such as the subclavian or femoral arteries, leads to claudication, while involvement of the visceral vessels may lead to hypertension (kidney arteries); abdominal pain, especially ischemic bowel pain such as postprandial pain or abdominal pain accompanied by gastrointestinal bleeding (mesenteric arteries); chest pain (aorta or coronary arteries); or neurologic symptoms (cerebral vasculature).
●Small vessels – Inflammation of smaller vessels (such as in granulomatosis with polyangiitis, microscopic polyangiitis, IgA vasculitis, and eosinophilic granulomatosis with polyangiitis [Churg-Strauss]) leads to symptoms in richly vascularized organs including the skin, lungs, kidneys, gastrointestinal tract, and brain [1]. Symptoms and findings might include hemoptysis, abdominal complaints, hematuria, and neurologic dysfunction. Characteristic vasculitic skin lesions include palpable purpura (picture 1), livedo reticularis (picture 7), nonblanching lesions, nodules, ulcers, and palmar/plantar erythema.
Further evaluation — If the diagnosis of a specific vasculitis is not clear after the initial assessment, additional investigations are used to confirm the presence of vasculitis and to identify the specific disorder, if possible. These include additional laboratory tests, organ-specific imaging and functional studies, and tissue biopsy.
Additional laboratory testing — Other laboratory tests that may be helpful in confirming the presence of vasculitis or in making a specific diagnosis include:
●Elevation of plasma von Willebrand factor (VWF) – VWF is an acute-phase reactant that is increased 50 to 75 percent in inflammatory conditions [18]. More dramatic elevations (three- to fourfold) occur with vascular endothelium injury due to inflammation of small- and medium-sized vessels. VWF is also elevated in patients with vascular damage due to stroke, trauma, and severe infection. It may be helpful in differentiating vasculitis from malignancy or more self-limited infections and, if abnormal, may be useful as a biomarker to track disease activity.
●Antineutrophilic cytoplasmic antibodies (ANCAs) – ANCAs may be present in patients with small-vessel vasculitis. Perinuclear ANCA (pANCA) that is directed against myeloperoxidase (MPO) on immunofluorescence is associated with microscopic polyangiitis, whereas cytoplasmic ANCA (cANCA) that is directed against proteinase 3 (PR3) is associated with granulomatosis with polyangiitis [19-22]. The absence of ANCAs in these conditions, however, does not necessarily preclude their diagnosis, as ANCAs are not present in all cases. (See "Clinical spectrum of antineutrophil cytoplasmic autoantibodies" and "Vasculitis in children: Incidence and classification".)
●Antinuclear antibodies (ANAs) – If SLE is suspected in the differential diagnosis, then testing for ANAs is useful. A negative test result excludes SLE. A positive test is not valuable, because ANA is present both in the normal population and in other conditions. More specific autoantibody tests are required to support a diagnosis of SLE or other related conditions, such as mixed connective tissue disease. In addition, ANAs may interact with some assays of ANCAs, so knowledge of a patient's ANA status is also important for ruling out a false-positive ANCA. (See "Measurement and clinical significance of antinuclear antibodies".)
●Complement – Low serum complement levels may be present in essential mixed cryoglobulinemia, hypocomplementemic urticarial vasculitis, and SLE, but not in most other vasculitic disorders. Among those with cryoglobulins, infection with hepatitis C virus is in the differential. (See "Overview and clinical assessment of the complement system" and "Extrahepatic manifestations of hepatitis C virus infection".)
●Anti-glomerular basement membrane (GBM) antibodies – GBM antibodies should be ordered in patients with pulmonary-renal syndrome (diffuse alveolar hemorrhage and glomerulonephritis seen in a variety of diseases including granulomatosis with polyangiitis, microscopic polyangiitis, and SLE) to rule out anti-GBM disease.
●Tests for bacterial and viral infections – Evaluation for infectious diseases should include diagnostic studies to detect bacterial (eg, blood and urine cultures) or viral (eg, serology, culture, or rapid diagnostic studies) infections. (See "Fever of unknown origin in children: Evaluation", section on 'Overview of evaluation'.)
Organ-specific imaging and functional studies — Imaging and functional studies are used to evaluate involved organ systems or those that are suspected of being involved based upon the history, physical examination, and laboratory testing. The types of studies chosen will depend upon the particular organ system involved.
●Pulmonary studies – For suspected pulmonary involvement, chest radiographs, pulmonary function tests (PFT), computed tomography (CT) scans, and sometimes bronchoalveolar lavage may be useful. Chest radiographs are simple and easily accessible. Findings such as infiltrates or nodules may be seen in pulmonary vasculitis (as part of granulomatosis with polyangiitis or microscopic polyangiitis) but can also be seen in infection. Measurement of the diffusing capacity of carbon monoxide (DLCO) may provide a noninvasive tool for identifying a pulmonary hemorrhage since the only condition that elevates the DLCO above 100 percent is blood in the airways [23]. If the patient is too sick to perform PFTs, or if consolidation, granulomata, or lymphadenopathy is suspected, further imaging such as chest CT may be indicated. However, some CT findings are nonspecific, and bronchoalveolar lavage may be needed to determine if an infiltrate seen on chest radiograph or CT is due to hemorrhage or infection, for example. PFT may show an obstructive pattern in eosinophilic granulomatosis with polyangiitis in keeping with asthma.
●Cardiac and vascular studies – Patients with clinical cardiac involvement should have an echocardiogram to evaluate for both structural and functional abnormalities. The presence of coronary aneurysms with some other clinical features may be diagnostic of Kawasaki disease, although other vasculitides (such as polyarteritis nodosa), infections (Epstein-Barr virus), and inflammatory disorders (systemic juvenile idiopathic arthritis) also may cause coronary artery dilatation.
Large- and medium-sized vessel abnormalities may be detected by Doppler ultrasound, CT angiogram/magnetic resonance arteriography (MRA), positron emission tomography (PET), and conventional angiography (image 1). Such imaging should be pursued in children suspected of having Takayasu arteritis (large-vessel vasculitis), polyarteritis nodosa (predominantly medium-vessel vasculitis), or Kawasaki disease (when there is suspicion of vessel involvement beyond the coronary arteries).
The imaging modality is best guided by the suspected location and size of involved vessels. As an example, Doppler ultrasound of the kidneys may be employed if kidney artery involvement is suspected, while mesenteric angiography is indicated if gastrointestinal involvement is suspected, such as in polyarteritis nodosa. When larger vessels are involved, MRA of the aorta and great vessels, including some major branches, such as the coronaries and kidney arteries, may be an alternative to contrast arteriography in children. When the two techniques were compared in 10 children with various vascular lesions (age range: 1 month to 16 years), excellent correlation was found between the images (r = 0.99) [24]. For conditions involving smaller vessels, conventional angiography remains the preferred imaging modality and is more likely to demonstrate abnormalities such as small-vessel aneurysms that are characteristic of polyarteritis nodosa.
●Ear, nose, and throat studies – In children with suspected sinus disease, such as is found in granulomatosis with polyangiitis, sinus radiographs should be ordered, and, in some cases, a sinus CT or magnetic resonance imaging (MRI) is required for confirmation. Findings on sinus imaging may include thickening of the sinus mucosa, opacification of the sinuses or paranasal sinuses, or bony thickening and destruction [25]. MRI may be better at demonstrating soft tissue abnormalities, including granulomas, but is less commonly ordered and does not characterize bone lesions well [26]. Bronchoscopy or upper-airway imaging studies may be needed to evaluate upper-airway symptoms suggestive of subglottic stenosis seen in granulomatosis with polyangiitis, particularly in pediatric cases [27]. (See "Granulomatosis with polyangiitis and microscopic polyangiitis: Clinical manifestations and diagnosis", section on 'Ear, nose, and throat involvement' and "Granulomatosis with polyangiitis and microscopic polyangiitis: Clinical manifestations and diagnosis", section on 'Tracheal and pulmonary involvement'.)
Tissue biopsy — The gold standard for definitive diagnosis of a vasculitis remains histopathologic evidence of vascular inflammation. A tissue biopsy may be necessary if the diagnosis cannot be made on the basis of syndrome recognition with or without imaging studies. If the affected organ is easily accessible, such as the skin, the procedure is relatively minor and poses minimal risk to the patient. In the case of kidney involvement, there is a higher biopsy-associated risk but also a high diagnostic yield, and the information obtained about disease activity, extent, severity, and ultimately prognosis is valuable [28].
Some biopsies, such as nasal or transbronchial lung biopsy, have very low diagnostic yields. Other organs are less accessible, such as the brain or the lungs. In this setting, the risk of doing the biopsy plus the potential diagnostic yield is weighed against the risks of administrating a potentially long-term toxic treatment in a patient without diagnostic confirmation of a specific vasculitis. Such cases should be evaluated on their individual merits in consultation with experts.
SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Vasculitis".)
SUMMARY
●Overview – Vasculitis is defined as the presence of inflammation in a blood vessel, which may occur as a primary process or secondary to an underlying disease. Prompt recognition and treatment of these disorders are important as they can rapidly evolve to being severe and life-threatening conditions without appropriate treatment. (See 'Introduction' above.)
●Clinical presentation – The diagnosis of vasculitis should be considered in children and adolescents who present with persisting systemic symptoms of inflammation and evidence of multiorgan dysfunction. Clinical symptoms vary widely depending upon the types and location of vessels involved, the extent of inflammation, and the degree to which vascular integrity has been compromised.
In some cases, the initial presentation is restricted to a rash and nonspecific constitutional signs and symptoms, including fever, weight loss, weakness, or fatigue, which may be difficult to distinguish from findings seen in children with other disorders (table 1). However, in the majority of cases, a particular combination of findings or pattern of organ involvement (pattern or syndrome recognition) are indicative and sometimes sufficient to diagnosis a specific vasculitis, such as immunoglobulin A (IgA) vasculitis (Henoch-Schönlein purpura) or Kawasaki disease, the two most common causes of primary vasculitis in childhood. (See 'Clinical presentation' above.)
●Differential diagnosis – Patients with other disorders, including those associated with secondary vasculitis, can present with symptoms and findings similar to those with a primary vasculitis. A combination of clinical, pathologic, immunologic, and radiologic features will generally distinguish a primary vasculitis from other conditions including noninflammatory conditions (table 2). (See 'Differentiating primary vasculitis from other disorders' above.)
●Initial evaluation – The initial evaluation of any child suspected of having vasculitis includes a detailed history, comprehensive physical examination, and basic laboratory testing. The goal is to both support a diagnosis of vasculitis and to exclude other, more common conditions that may present similarly. In most cases, the diagnosis of a primary vasculitis is made based upon clinical findings and pattern recognition of the characteristic findings associated with the specific disease. (See 'Initial evaluation' above.)
●Clinical correlation with vessel size and location – The primary vasculitides are commonly classified and differentiated according to vessel size. Early in the course of the disease, nonspecific complaints (ie, fever, fatigue, anorexia, weight loss, and arthralgias) are generally present irrespective of the size of the involved vessels, but over time and with further evaluation, some clinical features typically correlate with the size and location of vessel involvement. (See 'Clinical correlation with vessel size' above and "Vasculitis in children: Incidence and classification".)
●Additional evaluation – If the diagnosis of a specific vasculitis is not clear after the initial assessment, additional investigations including imaging and tissue biopsy are used to differentiate vasculitis from other conditions and to determine the specific vasculitic disorder. (See 'Further evaluation' above.)
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