Vasculitis in Juvenile-Onset Systemic Lupus Erythematosus
Identifieur interne : 000990 ( Pmc/Corpus ); précédent : 000989; suivant : 000991Vasculitis in Juvenile-Onset Systemic Lupus Erythematosus
Auteurs : Eve M. D. Smith ; Hanna Lythgoe ; Christian M. HedrichSource :
- Frontiers in Pediatrics [ 2296-2360 ] ; 2019.
Abstract
Juvenile-onset systemic lupus erythematosus (JSLE) is a rare, heterogeneous multisystem autoimmune disease that can affect any organ, and present with diverse clinical and serological manifestations. Vasculitis can be a feature of JSLE. It more commonly presents as cutaneous vasculitis than visceral vasculitis, which can affect the central nervous system, peripheral nervous system, lungs, gut, kidneys, heart, and large vessels. The incidence and prevalence of vasculitis in JSLE has not been well described to date. Symptoms of vasculitis can be non-specific and overlap with other features of JSLE, requiring careful consideration for the diagnosis to be achieved and promptly treated. Biopsies are often required to make a definitive diagnosis and differentiate JSLE related vasculitis from other manifestations of JSLE, vasculopathies, and JSLE related antiphospholipid syndrome. Visceral vasculitis can be life threatening, and its presence at the time of JSLE diagnosis is associated with permanent organ damage, which further highlights the importance of prompt recognition and treatment. This review will focus on the presentation, diagnosis, management and outcomes of vasculitis in JSLE, highlighting gaps in the current evidence base.
Url:
DOI: 10.3389/fped.2019.00149
PubMed: 31143758
PubMed Central: 6521594
Links to Exploration step
PMC:6521594Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Vasculitis in Juvenile-Onset Systemic Lupus Erythematosus</title><author><name sortKey="Smith, Eve M D" sort="Smith, Eve M D" uniqKey="Smith E" first="Eve M. D." last="Smith">Eve M. D. Smith</name><affiliation><nlm:aff id="aff1"><institution>Department of Women and Children's Health, Institute of Translational Medicine, University of Liverpool</institution>,<addr-line>Liverpool</addr-line>,<country>United Kingdom</country></nlm:aff></affiliation><affiliation><nlm:aff id="aff2"><institution>Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust</institution>,<addr-line>Liverpool</addr-line>,<country>United Kingdom</country></nlm:aff></affiliation></author><author><name sortKey="Lythgoe, Hanna" sort="Lythgoe, Hanna" uniqKey="Lythgoe H" first="Hanna" last="Lythgoe">Hanna Lythgoe</name><affiliation><nlm:aff id="aff3"><institution>St Helen's and Knowsley Teaching Hospital NHS Trust</institution>,<addr-line>St Helens</addr-line>,<country>United Kingdom</country></nlm:aff></affiliation></author><author><name sortKey="Hedrich, Christian M" sort="Hedrich, Christian M" uniqKey="Hedrich C" first="Christian M." last="Hedrich">Christian M. Hedrich</name><affiliation><nlm:aff id="aff1"><institution>Department of Women and Children's Health, Institute of Translational Medicine, University of Liverpool</institution>,<addr-line>Liverpool</addr-line>,<country>United Kingdom</country></nlm:aff></affiliation><affiliation><nlm:aff id="aff2"><institution>Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust</institution>,<addr-line>Liverpool</addr-line>,<country>United Kingdom</country></nlm:aff></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">31143758</idno><idno type="pmc">6521594</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6521594</idno><idno type="RBID">PMC:6521594</idno><idno type="doi">10.3389/fped.2019.00149</idno><date when="2019">2019</date><idno type="wicri:Area/Pmc/Corpus">000990</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">000990</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Vasculitis in Juvenile-Onset Systemic Lupus Erythematosus</title><author><name sortKey="Smith, Eve M D" sort="Smith, Eve M D" uniqKey="Smith E" first="Eve M. D." last="Smith">Eve M. D. Smith</name><affiliation><nlm:aff id="aff1"><institution>Department of Women and Children's Health, Institute of Translational Medicine, University of Liverpool</institution>,<addr-line>Liverpool</addr-line>,<country>United Kingdom</country></nlm:aff></affiliation><affiliation><nlm:aff id="aff2"><institution>Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust</institution>,<addr-line>Liverpool</addr-line>,<country>United Kingdom</country></nlm:aff></affiliation></author><author><name sortKey="Lythgoe, Hanna" sort="Lythgoe, Hanna" uniqKey="Lythgoe H" first="Hanna" last="Lythgoe">Hanna Lythgoe</name><affiliation><nlm:aff id="aff3"><institution>St Helen's and Knowsley Teaching Hospital NHS Trust</institution>,<addr-line>St Helens</addr-line>,<country>United Kingdom</country></nlm:aff></affiliation></author><author><name sortKey="Hedrich, Christian M" sort="Hedrich, Christian M" uniqKey="Hedrich C" first="Christian M." last="Hedrich">Christian M. Hedrich</name><affiliation><nlm:aff id="aff1"><institution>Department of Women and Children's Health, Institute of Translational Medicine, University of Liverpool</institution>,<addr-line>Liverpool</addr-line>,<country>United Kingdom</country></nlm:aff></affiliation><affiliation><nlm:aff id="aff2"><institution>Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust</institution>,<addr-line>Liverpool</addr-line>,<country>United Kingdom</country></nlm:aff></affiliation></author></analytic><series><title level="j">Frontiers in Pediatrics</title><idno type="eISSN">2296-2360</idno><imprint><date when="2019">2019</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p>Juvenile-onset systemic lupus erythematosus (JSLE) is a rare, heterogeneous multisystem autoimmune disease that can affect any organ, and present with diverse clinical and serological manifestations. Vasculitis can be a feature of JSLE. It more commonly presents as cutaneous vasculitis than visceral vasculitis, which can affect the central nervous system, peripheral nervous system, lungs, gut, kidneys, heart, and large vessels. The incidence and prevalence of vasculitis in JSLE has not been well described to date. Symptoms of vasculitis can be non-specific and overlap with other features of JSLE, requiring careful consideration for the diagnosis to be achieved and promptly treated. Biopsies are often required to make a definitive diagnosis and differentiate JSLE related vasculitis from other manifestations of JSLE, vasculopathies, and JSLE related antiphospholipid syndrome. Visceral vasculitis can be life threatening, and its presence at the time of JSLE diagnosis is associated with permanent organ damage, which further highlights the importance of prompt recognition and treatment. This review will focus on the presentation, diagnosis, management and outcomes of vasculitis in JSLE, highlighting gaps in the current evidence base.</p></div></front><back><div1 type="bibliography"><listBibl><biblStruct><analytic><author><name sortKey="Toubi, E" uniqKey="Toubi E">E Toubi</name></author><author><name sortKey="Kessel, A" uniqKey="Kessel A">A Kessel</name></author><author><name sortKey="Bamberger, E" uniqKey="Bamberger E">E Bamberger</name></author><author><name sortKey="Golan, Td" uniqKey="Golan T">TD Golan</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Callen, Jp" uniqKey="Callen J">JP Callen</name></author><author><name sortKey="Kingman, J" uniqKey="Kingman J">J Kingman</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Zecevic, Rd" uniqKey="Zecevic R">RD Zecevic</name></author><author><name sortKey="Vojvodic, D" uniqKey="Vojvodic D">D Vojvodic</name></author><author><name sortKey="Ristic, B" 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<front><journal-meta><journal-id journal-id-type="nlm-ta">Front Pediatr</journal-id><journal-id journal-id-type="iso-abbrev">Front Pediatr</journal-id><journal-id journal-id-type="publisher-id">Front. Pediatr.</journal-id><journal-title-group><journal-title>Frontiers in Pediatrics</journal-title></journal-title-group><issn pub-type="epub">2296-2360</issn><publisher><publisher-name>Frontiers Media S.A.</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="pmid">31143758</article-id><article-id pub-id-type="pmc">6521594</article-id><article-id pub-id-type="doi">10.3389/fped.2019.00149</article-id><article-categories><subj-group subj-group-type="heading"><subject>Pediatrics</subject><subj-group><subject>Mini Review</subject></subj-group></subj-group></article-categories><title-group><article-title>Vasculitis in Juvenile-Onset Systemic Lupus Erythematosus</article-title></title-group><contrib-group><contrib contrib-type="author"><name><surname>Smith</surname><given-names>Eve M. D.</given-names></name><xref ref-type="aff" rid="aff1"><sup>1</sup></xref><xref ref-type="aff" rid="aff2"><sup>2</sup></xref><xref ref-type="corresp" rid="c001"><sup>*</sup></xref><xref ref-type="author-notes" rid="fn002"><sup>†</sup></xref><uri xlink:type="simple" xlink:href="http://loop.frontiersin.org/people/507657/overview"></uri></contrib><contrib contrib-type="author"><name><surname>Lythgoe</surname><given-names>Hanna</given-names></name><xref ref-type="aff" rid="aff3"><sup>3</sup></xref><xref ref-type="author-notes" rid="fn002"><sup>†</sup></xref><uri xlink:type="simple" xlink:href="http://loop.frontiersin.org/people/701159/overview"></uri></contrib><contrib contrib-type="author"><name><surname>Hedrich</surname><given-names>Christian M.</given-names></name><xref ref-type="aff" rid="aff1"><sup>1</sup></xref><xref ref-type="aff" rid="aff2"><sup>2</sup></xref></contrib></contrib-group><aff id="aff1"><sup>1</sup><institution>Department of Women and Children's Health, Institute of Translational Medicine, University of Liverpool</institution>,<addr-line>Liverpool</addr-line>,<country>United Kingdom</country></aff><aff id="aff2"><sup>2</sup><institution>Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust</institution>,<addr-line>Liverpool</addr-line>,<country>United Kingdom</country></aff><aff id="aff3"><sup>3</sup><institution>St Helen's and Knowsley Teaching Hospital NHS Trust</institution>,<addr-line>St Helens</addr-line>,<country>United Kingdom</country></aff><author-notes><fn fn-type="edited-by"><p>Edited by: Claudio Pignata, University of Naples Federico II, Italy</p></fn><fn fn-type="edited-by"><p>Reviewed by: Micaela Fredi, Azienda Socio Sanitaria Territoriale of the Spedali Civili of Brescia, Italy; Klaus Tenbrock, RWTH Aachen Universität, Germany</p></fn><corresp id="c001">*Correspondence: Eve M. D. Smith <email>esmith8@liverpool.ac.uk</email></corresp><fn fn-type="other" id="fn001"><p>This article was submitted to Pediatric Immunology, a section of the journal Frontiers in Pediatrics</p></fn><fn fn-type="other" id="fn002"><p>†Joint first authors</p></fn></author-notes><pub-date pub-type="epub"><day>09</day><month>5</month><year>2019</year></pub-date><pub-date pub-type="collection"><year>2019</year></pub-date><volume>7</volume><elocation-id>149</elocation-id><history><date date-type="received"><day>25</day><month>12</month><year>2018</year></date><date date-type="accepted"><day>01</day><month>4</month><year>2019</year></date></history><permissions><copyright-statement>Copyright © 2019 Smith, Lythgoe and Hedrich.</copyright-statement><copyright-year>2019</copyright-year><copyright-holder>Smith, Lythgoe and Hedrich</copyright-holder><license xlink:href="http://creativecommons.org/licenses/by/4.0/"><license-p>This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.</license-p></license></permissions><abstract><p>Juvenile-onset systemic lupus erythematosus (JSLE) is a rare, heterogeneous multisystem autoimmune disease that can affect any organ, and present with diverse clinical and serological manifestations. Vasculitis can be a feature of JSLE. It more commonly presents as cutaneous vasculitis than visceral vasculitis, which can affect the central nervous system, peripheral nervous system, lungs, gut, kidneys, heart, and large vessels. The incidence and prevalence of vasculitis in JSLE has not been well described to date. Symptoms of vasculitis can be non-specific and overlap with other features of JSLE, requiring careful consideration for the diagnosis to be achieved and promptly treated. Biopsies are often required to make a definitive diagnosis and differentiate JSLE related vasculitis from other manifestations of JSLE, vasculopathies, and JSLE related antiphospholipid syndrome. Visceral vasculitis can be life threatening, and its presence at the time of JSLE diagnosis is associated with permanent organ damage, which further highlights the importance of prompt recognition and treatment. This review will focus on the presentation, diagnosis, management and outcomes of vasculitis in JSLE, highlighting gaps in the current evidence base.</p></abstract><kwd-group><kwd>childhood lupus</kwd><kwd>JSLE</kwd><kwd>vasculitis</kwd><kwd>cutaneous vasculitis</kwd><kwd>visceral vasculitis</kwd></kwd-group><counts><fig-count count="3"></fig-count><table-count count="1"></table-count><equation-count count="0"></equation-count><ref-count count="78"></ref-count><page-count count="9"></page-count><word-count count="6433"></word-count></counts></article-meta></front><body><sec sec-type="intro" id="s1"><title>Introduction</title><p>Vasculitis is a well-recognized feature of juvenile-onset systemic lupus erythematosus (JSLE). Despite this, there is a paucity of literature in this area. Vasculitis is defined by inflammatory changes to vessel walls, affecting different types (arteries, veins, capillaries) and/or sizes of vessels (large, medium, small) and a variety of sites (e.g., skin or visceral/internal organs). Symptoms of JSLE-related vasculitis can be non-specific (e.g., fatigue, fever, weight loss) and overlap with other features of the disease. Careful consideration is required to detect vasculitis, in particular visceral vasculitis, which is less common but potentially life-threatening and requires prompt and aggressive treatment. Histopathological assessment of tissue biopsies are the diagnostic gold standard, but patients may be classified with “probable vasculitis” based on clinical features alone (<xref rid="B1" ref-type="bibr">1</xref>). This review will update readers on literature available and highlight gaps in the current evidence-base.</p><p>Vasculitis can be associated with disease flares in SLE (<xref rid="B2" ref-type="bibr">2</xref>–<xref rid="B4" ref-type="bibr">4</xref>), and is therefore integral part of disease activity assessment tools, including the Systemic Lupus Activity Index (SLAM), British Isles Lupus Assessment Group (BILAG), and Systemic Lupus Activity Assessment Index 2000 (SLEDAI) (<xref rid="B5" ref-type="bibr">5</xref>). Patients with skin lesions that are not specific to SLE, such as cutaneous vasculitis, experience significantly more active disease when compared to patients with lupus-specific skin lesions only (e.g., malar rash) (<xref rid="B3" ref-type="bibr">3</xref>). The presence of major organ vasculitis at baseline influences the damage trajectory in JSLE patients, and is associated with greater JSLE related permanent organ damage (<xref rid="B6" ref-type="bibr">6</xref>).</p></sec><sec id="s2"><title>Cutaneous Vasculitis</title><sec><title>Epidemiology</title><p>The exact prevalence of cutaneous vasculitis in JSLE is not known. A study involving 179 patients with JSLE in the UK found that 12% experienced cutaneous vasculitis (<xref rid="B7" ref-type="bibr">7</xref>). A Brazilian study of 414 patients with SLE including 60 patients with JSLE found that 21.6% of JSLE patients developed cutaneous vasculitis compared with 15.4% of those with adult-onset disease (aSLE, difference was not statistically significant) (<xref rid="B8" ref-type="bibr">8</xref>). A study involving 50 aSLE patients found individuals with cutaneous vasculitis to be significantly younger when compared to patients who did not develop vasculitis (26.5 vs. 30.3 years; <italic>p</italic> = 0.018) (<xref rid="B9" ref-type="bibr">9</xref>).</p></sec><sec><title>Associations With Other Organ Manifestations of SLE</title><p>In aSLE cutaneous vasculitis has been shown to be associated with lupus nephritis, hyopocomplementaemia (<xref rid="B9" ref-type="bibr">9</xref>, <xref rid="B10" ref-type="bibr">10</xref>), musculoskeletal, constitutional, cardiovascular manifestations and Sjogren's syndrome (<xref rid="B9" ref-type="bibr">9</xref>). In a further study involving 170 aSLE patients, patients with lupus nephritis were shown to be at increased risk of cutaneous vasculitis (<xref rid="B10" ref-type="bibr">10</xref>). Lastly, cutaneous vasculitis may also be associated with neuropsychiatric lupus in aSLE (<xref rid="B11" ref-type="bibr">11</xref>, <xref rid="B12" ref-type="bibr">12</xref>).</p></sec><sec><title>Clinical Presentation and Pathophysiology</title><p>In JSLE, skin manifestations can be divided into lupus-specific (e.g., malar rash, discoid lupus, panniculitis) and lupus non-specific, including cutaneous vasculitis. SLE-associated cutaneous vasculitis affects small or medium-sized vessels in the skin and subcutaneous tissues. It has a wide variety of presentations that depend on the size of vessels involved and the extent of the vasculature affected. Cutaneous vasculitis most frequently affects the lower and upper limbs (<xref rid="B13" ref-type="bibr">13</xref>).</p><p>Vasculitis affecting the <bold>small vessels</bold> of the skin (arterioles, capillaries, post-capillary venules in the superficial, and mid-dermis) usually presents with petechiae, purpura, and/or punctate vasculitis lesions. Petechiae are pinprick macules which do not blanch and are not palpable, resulting from capillary inflammation and red blood cell extravasation (<xref ref-type="fig" rid="F1">Figure 1A</xref>) (<xref rid="B14" ref-type="bibr">14</xref>). Purpura are caused by inflammation of venules and/or arterioles and consist of larger papules and plaques which do not blanch and become palpable as damage progresses (<xref ref-type="fig" rid="F1">Figure 1B</xref>) (<xref rid="B14" ref-type="bibr">14</xref>). Punctate vasculitic lesions, ulcerations and tissue necrosis are caused by reduced perfusion; shallow ulcers are caused when this affects the small vessels and deeper ulcers are caused when medium-sized vessels are affected (<xref ref-type="fig" rid="F1">Figure 1C</xref>).</p><fig id="F1" position="float"><label>Figure 1</label><caption><p>Skin manifestations in SLE and SLE-like disease. Small vessel vasculitis is a “common” feature in SLE-associated skin vasculitis. <bold>(A)</bold> Petechia and ecchymosis are the result of capillary inflammation and red blood cell extravasation; <bold>(B)</bold> palpable purpura are caused by inflammatory damage to venules and/or arterioles; <bold>(C)</bold> ulcerations and tissue necrosis are the result of reduced perfusion; <bold>(D,E)</bold> chilblain lesions can manifest as chilblains (cold induced sores) that may ulcerate, or painful and/or itchy bluish-reddish discoloration with swelling; <bold>(F)</bold> vasculopathy and finger atrophy in a patient with complement deficiency and secondary type I interferon upregulation.</p></caption><graphic xlink:href="fped-07-00149-g0001"></graphic></fig><p>Vasculitis of <bold>medium sized vessels</bold> in the dermis or subcutaneous layers may cause livedo reticularis, nodules, and/or the aforementioned deep ulcers (<xref rid="B15" ref-type="bibr">15</xref>). Livedo reticularis is a small or widespread area of mottled, reticulated, reddish-purplish discoloration of the skin caused by compromised blood flow in the medium-sized vessels (<xref rid="B15" ref-type="bibr">15</xref>). Cutaneous ulcers, nodules, digital gangrene, livedo racemosa, and pyoderma-gangrenosum-like lesions are indicative of arterial involvement. Individuals affected have higher probability of associated visceral vasculitis (<xref rid="B16" ref-type="bibr">16</xref>). Lesions mimicking vasculitis can be caused by haemorrhagic and vaso-occlusive disease (<xref rid="B17" ref-type="bibr">17</xref>).</p><p>Cutaneous vasculitis in JSLE is most commonly an immune-complex mediated small-vessel vasculitis (<xref rid="B18" ref-type="bibr">18</xref>) (<xref ref-type="fig" rid="F2">Figure 2</xref>). Histological examination of lesions allows determination of the size of vessel affected and immune cells driving inflammation. Typical findings in lupus-related cutaneous vasculitis are small (predominantly) and medium vessel (less commonly) neutrophilic vasculitis with IgG, IgM and/or complement deposition at the basement membrane zone on direct immunofluorescence examination (<xref rid="B14" ref-type="bibr">14</xref>).</p><fig id="F2" position="float"><label>Figure 2</label><caption><p>Pro-inflammatory mechanisms in immune complex vasculitis. Immune complex vasculitis is not disease specific and can be a feature or leading symptom of various disorders, including infections and autoimmune/inflammatory conditions. Immune complex deposition result in complement activation, which in turn mediates local inflammation and oedema. This results in the recruitment of immune cells, including macrophages, neutrophils, and NK cells, which further contribute to inflammation and tissue damage through inflammatory cytokine expression. Mast cell and basophil degranulation further amplifying tissue edema and mediates vasodilation. Reproduced with permission from (<xref rid="B19" ref-type="bibr">19</xref>).</p></caption><graphic xlink:href="fped-07-00149-g0002"></graphic></fig></sec><sec><title>Urticarial Vasculitis</title><p>Urticarial vasculitis is a recognized rare presentation of SLE presenting with hives lasting more than 24 h which may be entirely asymptomatic, pruritic, or painful. It usually resolves with hyperpigmentation or purpura (<xref rid="B18" ref-type="bibr">18</xref>, <xref rid="B20" ref-type="bibr">20</xref>, <xref rid="B21" ref-type="bibr">21</xref>). The incidence of JSLE-associated urticarial vasculitis is unknown but there are several case reports (<xref rid="B22" ref-type="bibr">22</xref>–<xref rid="B25" ref-type="bibr">25</xref>). Urticarial vasculitis is an immune-complex mediated small-vessel process with leukocytoplastic changes on histology (<xref rid="B14" ref-type="bibr">14</xref>). The term “hypocomplementemic urticarial vasculitis” describes the coexistence of hypocomplementemia. Affected individuals frequently exhibit anti-C1q antibodies, which may contribute to altered immune complex processing and removal, and associated systemic involvement (<xref rid="B21" ref-type="bibr">21</xref>, <xref rid="B26" ref-type="bibr">26</xref>, <xref rid="B27" ref-type="bibr">27</xref>).</p></sec><sec><title>Cryoglobulinaemic Vasculitis</title><p>Cutaneous vasculitis can present with cryoglobulinaemic vasculitis, manifesting as purpuric lesions. IgM and C3 containing immune complexes are present on direct immunofluorescence (<xref ref-type="fig" rid="F2">Figure 2</xref>). Reports on cryoglobulinaemia and vasculitis in children are limited (<xref rid="B13" ref-type="bibr">13</xref>, <xref rid="B28" ref-type="bibr">28</xref>).</p></sec><sec><title>Diagnosis</title><p>The diagnosis of SLE-associated cutaneous vasculitis is based on clinical assessment. However, where practical and in cases of uncertainty, biopsies should be taken to confirm the diagnosis (<xref rid="B15" ref-type="bibr">15</xref>). This is of particular importance because cutaneous vasculitis can be a sign of high disease activity, and may trigger escalation of treatment (<xref rid="B3" ref-type="bibr">3</xref>). A study in aSLE patients found that while 36% of patients with digital lesions were clinically diagnosed with vasculitis, following dermatological/histological review only 4% actually had confirmed vasculitis (<xref rid="B29" ref-type="bibr">29</xref>). Timing, location, and appropriate depth of biopsy are important for diagnostic accuracy. Ideally, biopsies should be taken within 48 h of the lesions appearing, to avoid false negative results (<xref rid="B14" ref-type="bibr">14</xref>).</p></sec><sec><title>Treatment</title><p>Topical and low-dose systemic corticosteroids, and/or antimalarial agents (usually Hydroxychloroquine) are usually considered first-line treatment for cutaneous vasculitis. More severe disease may require high-dose steroids, intravenous immunoglobulins (IVIG), plasmapheresis, and/or cytotoxic treatments (<xref rid="B18" ref-type="bibr">18</xref>). Rituximab can be effective in some SLE patients with otherwise treatment refractory cutaneous vasculitis (<xref rid="B15" ref-type="bibr">15</xref>).</p></sec></sec><sec id="s3"><title>Visceral Vasculitis</title><p>Visceral vasculitis is present in approximately 6% of aSLE patients (<xref rid="B30" ref-type="bibr">30</xref>). Reliable data on the prevalence in JSLE does not exist. It can affect a number of organs including the central nervous system (CNS), peripheral nervous system (PNS), lungs, gut, and more rarely the kidneys, heart, and large abdominal and/or thoracic vessels (<xref rid="B1" ref-type="bibr">1</xref>). Visceral vasculitis usually manifests in the context of disease flares, and can coexist with cutaneous vasculitis (<xref rid="B1" ref-type="bibr">1</xref>).</p><sec><title>CNS Vasculitis</title><p>In patients developing neuropsychiatric manifestations of SLE, CNS vasculitis should be considered. However, the contribution of CNS vasculitis in CNS lupus may be limited (<xref rid="B1" ref-type="bibr">1</xref>). Indeed, other mechanisms including T-cell and autoantibody-mediated damage to neuronal tissue underlie the majority of neuropsychiatric manifestations (<xref rid="B31" ref-type="bibr">31</xref>, <xref rid="B32" ref-type="bibr">32</xref>). Historic postmortem studies estimated the incidence of CNS vasculitis in SLE to be around 7–10% (<xref rid="B33" ref-type="bibr">33</xref>). In the context of neuropsychiatric SLE it is particularly important to consider the contribution of antiphospholipid syndrome (APS) (<xref rid="B34" ref-type="bibr">34</xref>). SLE vasculitis and APS are the result of very different underlying pathologies that may require differential treatment. APS leads to thrombo-occlusive vasculopathy, warranting treatment with anticoagulation (e.g., heparin) and/or anti-aggregation (e.g., apsirin, clopidogrel) and/or intensive immunosuppressive therapy (e.g., corticosteroids, IVIG, rituximab or other immunosuppressive therapy, plasma exchange) in the context of catastrophic APS (<xref rid="B35" ref-type="bibr">35</xref>). Case management is informed by the overall clinical picture and laboratory parameters. While APS is characterized by the presence of persistent moderate-high titers of antiphospholipid antibodies associated with APS, SLE flares with vasculitis are associated with leukopenia, anti-ds-DNA antibodies and hypocomplementaemia (<xref rid="B1" ref-type="bibr">1</xref>).</p><p>Diagnosing CNS vasculitis requires a high index of suspicion, a systematic multi-disciplinary approach to diagnostic evaluation with MRI imaging playing a central role, and thorough exclusion of the differential diagnoses. Investigations should be directed at the exclusion of underlying conditions, including infections (the most common causes of secondary CNS vasculitis), drug-induced vasculitis, malignancy-associated vasculitis, non-vasculitic inflammatory brain diseases, demyelinating disorders, and antibody-mediated inflammatory brain diseases (<xref rid="B36" ref-type="bibr">36</xref>). The recent European evidence-based recommendations for diagnosis and treatment of JSLE (the SHARE initiative) imply that the initial diagnostic work-up of patients with suspected neuropsychiatric JSLE should be performed as in patients without SLE, and lumbar puncture/cerebrospinal fluid analysis, electroencephalogram, neuropsychological assessment of cognitive function, ophthalmologist review, nerve conductional studies, and MRI scanning should be considered. However, SHARE did not provide specific recommendations for suspected SLE CNS vasculitis, but highlighted that a MRI scan cannot exclude neuropsychiatric lupus (<xref rid="B37" ref-type="bibr">37</xref>). Even with angiography, false negative results are possible if the small vessels are predominantly involved (<xref rid="B38" ref-type="bibr">38</xref>), and brain biopsy may be required (<xref rid="B1" ref-type="bibr">1</xref>, <xref rid="B36" ref-type="bibr">36</xref>, <xref rid="B39" ref-type="bibr">39</xref>). Treatment usually involves high-dose glucocorticoids and cyclophosphamide, and may include plasmapheresis and IVIG (<xref rid="B40" ref-type="bibr">40</xref>).</p></sec><sec><title>Peripheral Nervous System (PNS) Involvement</title><p>Peripheral neuropathies including mononeuritis multiplex can be the result of vasculitis and ischemic damage in JSLE. Mononeuritis multiplex is substantially more common in aSLE, when compared to JSLE patients. It is characterized clinically by symmetric, mild-to-moderately severe sensorimotor polyneuropathy (<xref rid="B39" ref-type="bibr">39</xref>). Nerve biopsies usually unveil axonal degeneration and/or depletion, which can be associated with nonspecific vascular changes or chronic perivascular inflammation. Occasionally, biopsies may show necrotising vasculitis (<xref rid="B41" ref-type="bibr">41</xref>). As few as two cases of mononeuritis multiplex have been described in JSLE patients to date (<xref rid="B42" ref-type="bibr">42</xref>, <xref rid="B43" ref-type="bibr">43</xref>). Other causes of mononeuritis multiplex in children include Eosinophilic Granulomatosis with Polyangiitis (EGPA; formerly known as Churg-Strauss syndrome), polyarteritis nodosa, hypersensitivity/toxic vasculitides, diabetes mellitus, and Tangier's disease (an inborn error of metabolism) (<xref rid="B43" ref-type="bibr">43</xref>). Treatment of PNS vasculitis in JSLE may involve high-dose corticosteroids and cyclophosphamide, but is based upon aSLE case series (<xref rid="B43" ref-type="bibr">43</xref>).</p></sec><sec><title>Pulmonary Vasculitis</title><p>Acute lupus pneumonitis and diffuse alveolar hemorrhage (DAH) are the two most common pulmonary presentations of vasculitis in SLE. Fortunately, both are relatively rare [pneumonitis: 0–14% (<xref rid="B44" ref-type="bibr">44</xref>), DAH: 2–5.4% of aSLE patients (<xref rid="B45" ref-type="bibr">45</xref>, <xref rid="B46" ref-type="bibr">46</xref>)]. Reports on the prevalence in JSLE currently do not exist. Both conditions may present with sudden-onset dyspnea, cough, fever, and hypoxia. Main clinical differences are pleuritic chest pain in acute lupus pneumonitis, and blood stained sputum or haemoptysis in DAH (<xref rid="B47" ref-type="bibr">47</xref>). However, none of these necessarily have to be present. Radiological features of acute lupus pneumonitis on computed tomography (CT) scan include an often bilateral diffuse acinar filling pattern in the lower lobes, and frequently coexists with pleural effusion in ~50% of patients (<xref rid="B48" ref-type="bibr">48</xref>). In DAH, diffuse alveolar opacities are characteristically seen on CT (<xref ref-type="fig" rid="F3">Figure 3</xref>). Diffusion capacity for carbon monoxide (DLCO) is usually increased due to the increased availability of hemoglobin within the alveoli (<xref rid="B47" ref-type="bibr">47</xref>). In both conditions, bronchoscopy and bronchial alveolar lavage (BAL) are required (where the patient's condition allows it) to exclude infectious differential diagnoses or confirm the diagnosis. In DAH, aspirated fluid is bloody and microscopically red blood cells and haemosiderin-laden macrophages are present (<xref rid="B45" ref-type="bibr">45</xref>). Occasionally, an open lung biopsy may be needed to reach a diagnosis of acute lupus pneumonitis, with pathological findings including capillaritis, diffuse alveolar damage and necrosis, cellular infiltrates, hyaline membranes, and sometimes alveolar hemorrhage (<xref rid="B47" ref-type="bibr">47</xref>). The pathogenesis of both acute pulmonary pneumonitis and DAH is incompletely understood, but thought to involve deposition of immune complexes in the alveolar septae and blood vessels, and activation of complement leading to capillaritis, with immunohistological studies demonstrating immune complexes which include anti-ds-DNA antibodies and C3 (<xref rid="B49" ref-type="bibr">49</xref>) (<xref ref-type="fig" rid="F2">Figure 2</xref>).</p><fig id="F3" position="float"><label>Figure 3</label><caption><p>Diffuse alveolar hemorrhage in a 16-year-old SLE patient.</p></caption><graphic xlink:href="fped-07-00149-g0003"></graphic></fig><p>Treatment of both acute lupus pneumonitis and DAH is based upon case reports and small case series. Early detection and treatment initiation are crucial. Patients are usually treated with broad-spectrum antibiotics, high-dose corticosteroids and/or cyclophosphamide, and may need mechanical ventilation, IVIG and potentially plasma exchange (<xref rid="B46" ref-type="bibr">46</xref>, <xref rid="B47" ref-type="bibr">47</xref>, <xref rid="B50" ref-type="bibr">50</xref>). Both conditions are characterized by poor prognosis with associated mortality rates of ~50%. In those who survive, persistent interstitial infiltrates and lung function abnormalities are frequently seen, with potential for progression on to chronic interstitial pneumonitis (<xref rid="B48" ref-type="bibr">48</xref>).</p></sec><sec><title>Gastrointestinal Vasculitis</title><p>Gastrointestinal symptoms are not uncommon in JSLE patients and may relate to either treatment-related side effects, infections or JSLE disease activity. Lupus mesenteric vasculitis (LMV), characteristically presents with the clinical picture of an “acute abdomen” with sudden onset, diffuse and severe abdominal pain which can be associated with nausea, rectal bleeding, and vomiting (<xref rid="B51" ref-type="bibr">51</xref>). Of note, corticosteroids and/or other immunosuppressive treatments may mask symptoms and result in diagnostic and therapeutic delay. The estimated prevalence of LMV ranges between 0.2 and 9.7% among aSLE patients, making up for ~29–65% of aSLE patients presenting with an acute abdomen (<xref rid="B51" ref-type="bibr">51</xref>). In a Taiwanese study comparing aSLE and JSLE patients presenting with acute abdominal pain, LMV was the most common cause of admission in the JSLE patients. Furthermore, LMV was significantly more prevalent in JSLE patients when compared to the aSLE group [12/38 (31.6%) vs. 15/1081 (3.9%); <italic>p</italic> = 0.016], occurring in JSLE patients with high disease activity (SLE disease activity index, SLEDAI scores >8). Children were also found to be more likely to experience recurrent episodes of LMV (39.1% vs. 14.8%; <italic>p</italic> = 0.009) (<xref rid="B52" ref-type="bibr">52</xref>). In contrast to this study, other studies have reported LMV in JSLE patients with low SLEDAI scores (<xref rid="B53" ref-type="bibr">53</xref>, <xref rid="B54" ref-type="bibr">54</xref>).</p><p>Bowel ischemia secondary to LMV can result in perforation, hemorrhage and high mortality rates of up to 50%. The importance of early laparotomy was emphasized by a study demonstrating significantly higher survival in patients who underwent early intervention (0/33 deaths when laparotomy was undertaken within 24–48 h vs. 10/11 when laparotomy was performed after 48 h) (<xref rid="B55" ref-type="bibr">55</xref>). LMV most frequently affects the superior mesenteric artery that supplies the ileum and jejunum (80–85%), with the rectum less frequently affected (14%), and gastric involvement being very uncommon (<xref rid="B56" ref-type="bibr">56</xref>). Abdominal CT is a useful investigation for diagnosing LMV. It is characterized by the presence of dilated bowels, target lesions (abnormal bowel wall enhancement), comb signs (engorgement of mesenteric vessels), bowel wall thickening, and ascites (<xref rid="B57" ref-type="bibr">57</xref>). Histology may demonstrate arteritis, venulitis, immune complex, C3, and fibrinogen deposition, inflammatory cell infiltration, necrosis, and thrombosis of vessels affected (<xref rid="B58" ref-type="bibr">58</xref>). No randomized trials are available investigating treatment and associated outcomes in LMV. Currently, treatment involves “bowel rest,” intravenous corticosteroids, and cyclophosphamide in severe cases (<xref rid="B39" ref-type="bibr">39</xref>, <xref rid="B53" ref-type="bibr">53</xref>, <xref rid="B58" ref-type="bibr">58</xref>, <xref rid="B59" ref-type="bibr">59</xref>). Successful use of Rituximab has also been described in case series (<xref rid="B58" ref-type="bibr">58</xref>).</p></sec><sec><title>Renal Vasculitis</title><p>The prevalence of renal vasculitis in JSLE has not been investigated. In aSLE, vasculitic changes affecting the larger arterioles and small kidney arteries may (rarely) accompany proliferative lupus nephritis (<xref rid="B60" ref-type="bibr">60</xref>). Focal segmental necrotizing glomerulonephritis with fibrinoid necrosis can be seen, and may lead to rapidly progressive renal failure (<xref rid="B61" ref-type="bibr">61</xref>). In a Spanish study evaluating the prevalence and clinical characteristics of vasculitis in a cohort of 670 aSLE patients, 76/670 (11%) patients exhibited any vasculitis, but “only” 2/76 (3%) experienced renal vasculitis (<xref rid="B13" ref-type="bibr">13</xref>). Treatment of renal vasculitis in JSLE usually involves high-dose corticosteroid and cyclophosphamide (<xref rid="B39" ref-type="bibr">39</xref>).</p></sec><sec><title>Cardiac Vasculitis</title><p>Cardiac involvement in JSLE typically comprises pericarditis, cardiomegaly, valvulitis, and conduction abnormalities. SLE is associated with an increase in coronary heart disease risk, and a 50-fold increased risk of myocardial infarction (<xref rid="B62" ref-type="bibr">62</xref>). Coronary artery vasculitis occurs in both JSLE and adult SLE, but is rare across all age groups (<xref rid="B63" ref-type="bibr">63</xref>–<xref rid="B66" ref-type="bibr">66</xref>). Clinical differentiation of “classical” coronary artery disease from coronary arteritis is difficult and requires angiography. Coronary vasculitis usually results in segments of tapered narrowing, coronary ectasia, and/or aneurysms. Histopathological examination reveals thrombosis, deposition of immune complexes, infiltration of lymphocytes/neutrophils, and associated fibrinoid necrosis (<xref rid="B64" ref-type="bibr">64</xref>). A retrospective study assessed findings and outcomes in JSLE patients who were referred for echocardiography during their initial presentation for either tachycardia or a new murmur. Four patients demonstrated coronary artery dilatation (suggestive of coronary arteritis) which resolved once JSLE was treated, leading the authors to conclude that coronary arteritis may be more common in JSLE than previously appreciated (<xref rid="B63" ref-type="bibr">63</xref>). In a retrospective Taiwanese study looking at cardiopulmonary involvement in JSLE over a 20-year period, 6/157 patients exhibited coronary artery abnormalities, including vascular dilation, aneurysms, vasculitis, and stenosis (<xref rid="B67" ref-type="bibr">67</xref>). With SLE itself being a risk factor for cardiovascular disease, further studies are required longitudinally evaluating the impact of echocardiogram surveillance and treatment of coronary vasculitis on long-term cardiovascular risk.</p></sec><sec><title>Aortic Vasculitis</title><p>Vasculitis in SLE predominantly affects medium and small vessels. Aortic (large vessel) involvement has been reported in small case series and collated within a meta-analysis of 35 cases, of which 5/35 patients developed SLE in childhood, with aortic involvement manifesting between the ages of 23 and 38 years old. Thoracic aneurysms correlated with dissection and cystic medial degeneration, while abdominal lesions correlated with atherosclerosis. A total of 21/35 (60%) cases required surgery and death was observed in 11/35 (31.4%) patients. Thoracic lesions resulted in higher mortality rates than abdominal lesions (<xref rid="B68" ref-type="bibr">68</xref>). Treatment of aortic vasculitis includes corticosteroids, cyclophosphamide and blood pressure control. Surgery may be required in some cases (<xref rid="B39" ref-type="bibr">39</xref>, <xref rid="B69" ref-type="bibr">69</xref>). See <xref rid="T1" ref-type="table">Table 1</xref> for a summary of the prevalence of different vasculitic manifestations and the main treatments used.</p><table-wrap id="T1" position="float"><label>Table 1</label><caption><p>Prevalence and treatment of different vasculitc manifestations in JSLE.</p></caption><table frame="hsides" rules="groups"><thead><tr><th valign="top" align="left" rowspan="1" colspan="1"><bold>Vasculitic manifestation</bold></th><th valign="top" align="left" rowspan="1" colspan="1"><bold>Prevalence in JSLE</bold></th><th valign="top" align="left" rowspan="1" colspan="1"><bold>Usual treatments</bold></th></tr></thead><tbody><tr><td valign="top" align="left" rowspan="1" colspan="1">Cutaneous vasculitis</td><td valign="top" align="left" rowspan="1" colspan="1">12–21.6% (<xref rid="B7" ref-type="bibr">7</xref>, <xref rid="B8" ref-type="bibr">8</xref>)</td><td valign="top" align="left" rowspan="1" colspan="1">• Mild—moderate disease/first line treatments—topical and low-dose systemic corticosteroids, and/or antimalarial agents.<break></break>• More severe disease/second line treatment—high-dose steroids, IVIG, plasmapheresis and/or cytotoxic treatments (<xref rid="B18" ref-type="bibr">18</xref>). Rituximab is an option with otherwise treatment refractory cutaneous vasculitis (<xref rid="B15" ref-type="bibr">15</xref>).</td></tr><tr><td valign="top" align="left" rowspan="1" colspan="1">CNS vasculitis</td><td valign="top" align="left" rowspan="1" colspan="1">NA</td><td valign="top" align="left" rowspan="1" colspan="1">High-dose glucocorticoids and cyclophosphamide, and may require plasmapheresis and IVIG (<xref rid="B40" ref-type="bibr">40</xref>).</td></tr><tr><td valign="top" align="left" rowspan="1" colspan="1">PNS vasculitis</td><td valign="top" align="left" rowspan="1" colspan="1">NA</td><td valign="top" align="left" rowspan="1" colspan="1">High-dose corticosteroids and cyclophosphamide (<xref rid="B43" ref-type="bibr">43</xref>).</td></tr><tr><td valign="top" align="left" rowspan="1" colspan="1">Pulmonary vasculitis</td><td valign="top" align="left" rowspan="1" colspan="1">NA</td><td valign="top" align="left" rowspan="1" colspan="1">Broad-spectrum antibiotics, high-dose corticosteroids, and/or cyclophosphamide. May need mechanical ventilation, IVIG, and potentially plasma exchange (<xref rid="B46" ref-type="bibr">46</xref>, <xref rid="B47" ref-type="bibr">47</xref>, <xref rid="B50" ref-type="bibr">50</xref>).</td></tr><tr><td valign="top" align="left" rowspan="1" colspan="1">Gastrointestinal vasculitis</td><td valign="top" align="left" rowspan="1" colspan="1">31.6%<xref ref-type="table-fn" rid="TN1"><sup>*</sup></xref> (<xref rid="B52" ref-type="bibr">52</xref>)</td><td valign="top" align="left" rowspan="1" colspan="1">Bowel rest, intravenous corticosteroids, and cyclophosphamide in severe cases (<xref rid="B39" ref-type="bibr">39</xref>, <xref rid="B53" ref-type="bibr">53</xref>, <xref rid="B58" ref-type="bibr">58</xref>, <xref rid="B59" ref-type="bibr">59</xref>). Use of Rituximab has also been described in case series (<xref rid="B58" ref-type="bibr">58</xref>).</td></tr><tr><td valign="top" align="left" rowspan="1" colspan="1">Aortic vasculitis</td><td valign="top" align="left" rowspan="1" colspan="1">NA</td><td valign="top" align="left" rowspan="1" colspan="1">Corticosteroids, cyclophosphamide, and blood pressure control. Surgery may be required in some cases (<xref rid="B39" ref-type="bibr">39</xref>, <xref rid="B69" ref-type="bibr">69</xref>).</td></tr></tbody></table><table-wrap-foot><fn id="TN1"><label>*</label><p><italic>31.6% of all JSLE patients presenting with an acute abdomen (<xref rid="B52" ref-type="bibr">52</xref>). JSLE, juvenile systemic lupus erythematosus; NA, not available; IVIG, intravenous immunoglobulin</italic>.</p></fn></table-wrap-foot></table-wrap></sec></sec><sec id="s4"><title>SLE Vasculitis vs. Anti-phospholipid Syndrome</title><p>In aSLE patients, APS is associated with cutaneous vasculitis (<xref rid="B30" ref-type="bibr">30</xref>, <xref rid="B70" ref-type="bibr">70</xref>). Differentiating between vasculopathy/thrombosis and vasculitis in SLE-associated APS can be difficult. However, it is of utmost importance as treatment approaches are different (anti-inflammatory treatment for vasculitis vs. anti-thrombotic therapy for vasculopathy) (<xref rid="B71" ref-type="bibr">71</xref>). As mentioned above, histological assessment of cutaneous lesions can differentiate vasculitis from cutaneous manifestations of APS. This is particularly relevant in the context of ischaemic lesions which are difficult to differentiate clinically. Where biopsy is not practical, it is important to consider the overall context of the presentation and potential evidence of other APS-related manifestations such as thrombosis. In cases with extra-cutaneous evidence of SLE disease flare or systemic vasculitis, immunosuppressive treatment should be favored (<xref rid="B13" ref-type="bibr">13</xref>).</p></sec><sec id="s5"><title>Vasculitis in the Context of “SLE-Like” Disease</title><p>Primary type-I interferonopathies are a group of Mendelian disorders that share the upregulation of type-I interferon signaling as key pathophysiological feature. These monogenic diseases include (but are not limited to) Aicardi-Goutières syndrome and syndromic forms of SLE-like disease (<xref rid="B72" ref-type="bibr">72</xref>, <xref rid="B73" ref-type="bibr">73</xref>). A common clinical feature across type I interferonopathies is skin involvement with chilblain lesions and/or vasculitis as well as systemic vasculopathy and/or vasculitis, all of which can also been seen in patients with “classical” JSLE. Though very rare and not fully reflecting the molecular and clinical phenotype of JSLE, primary type I interferonopathies and their pathophysiology are useful “models” for “classical” JSLE, which is also frequently characterized by type I interferon activation. Furthermore, a number of other SLE-like conditions, including complement deficiencies, are characterized by type I interferon responses that are not primarily caused by mutations in positive or negative regulators of interferons, but the result of immune complex accumulation, another key contributor to the pathophysiology of SLE (<xref rid="B74" ref-type="bibr">74</xref>, <xref rid="B75" ref-type="bibr">75</xref>).</p><p>Aicardi-Goutières syndrome manifests with progressive encephalopathy that is associated with calcification of the basal ganglia, mimicking congenital viral infections (<xref rid="B76" ref-type="bibr">76</xref>). Clinical skin manifestations include chilblain lesions, which are the name giving feature of familial chilblain Lupus, another monogenic SLE-like disorder characterized by type I interferon upregulation (<xref ref-type="fig" rid="F1">Figures 1D,E</xref>). Aicardi-Goutières syndrome, familial chilblain Lupus and other type I interferonopathies demonstrate vasculopathy, immune complex deposition, and lymphocytic vasculitis on skin biopsy (<xref rid="B73" ref-type="bibr">73</xref>, <xref rid="B77" ref-type="bibr">77</xref>). Indeed, in severe cases peripheral vasculopathy can result in atrophy and even digital auto-amputation in some patients (<xref ref-type="fig" rid="F1">Figure 1F</xref>) (<xref rid="B72" ref-type="bibr">72</xref>). This has particularly been reported in spondyloenchondrodysplasia with immune dysregulation (SPENCDI), a rare immuno-osseous dysplasia caused by biallelic mutations in the <italic>ACP5</italic> gene (<xref rid="B72" ref-type="bibr">72</xref>) and Stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI) that includes heterozygous mutations in <italic>TMEM173</italic> (<xref rid="B78" ref-type="bibr">78</xref>). Both disorders, in addition to increased activation of type I interferon responses, share clinical features with “classical” SLE including neurological and immune manifestations in SPENCDI, and pyrexia, vasculitis, microthrombotic angiopathy, and interstitial lung disease in SAVI (<xref rid="B78" ref-type="bibr">78</xref>).</p></sec><sec sec-type="conclusions" id="s6"><title>Conclusions</title><p>JSLE is a rare, heterogeneous and complex condition. This translates to difficulty in the recognition and management of disease, particularly when less common manifestations, such as vasculitis are involved. Cutaneous is more common than visceral vasculitis, and more prevalent in JSLE when compared to adult-onset disease. Reports on visceral vasculitis in JSLE are limited, which may be a reflection of difficulty in achieving the diagnosis and differentiating vasculitis from other JSLE-related complications. Early recognition and treatment of SLE-associated vasculitis are paramount to optimizing outcomes and preventing tissue and organ damage. Collaborative approaches are required to improve our knowledge on the demographics, clinical presentations, disease courses, and treatment options in JSLE-associated vasculitis.</p></sec><sec id="s7"><title>Author Contributions</title><p>ES, HL, and CH all participated in review and interpretation of the literature. All authors were involved in drafting the manuscript and revising it critically for important intellectual content. They have also all read and given final approval of the version to be published.</p><sec><title>Conflict of Interest Statement</title><p>CH's work is supported by the Fritz-Thyssen Foundation (research grant: SLE), Novartis Pharmaceuticals (research grant: psoriasis), LUPUS UK (research grant: SLE), the Hugh Greenwood Legacy Fund (research grant: bronchial inflammation), the Michael Davie Research Foundation (research grant: CNO/CRMO), and the FAIR charity (research grants: bronchial inflammation and SLE). CH received honoraria for advisory board activities and presentations from Novartis pharmaceuticals and Roche (systemic autoinflammatory disease). 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