Skip to main content

Advertisement

SpringerLink
Log in

Mesenteric vasculitis in children with systemic lupus erythematosus

  • Case Based Review
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

Lupus mesenteric vasculitis (LMV) is a severe and potentially fatal complication of systemic lupus erythematosus (SLE). Although LMV is always a consideration in adolescents and adult patients with SLE, who present with acute abdominal pain, diagnosis and management remain a great challenge. We describe the cases of five patients age 14 to 21 years old diagnosed with LMV. All five patients had active SLE and typical clinical presentation suspicious of LMV. Abdominal CT was the preferred imaging modality and was useful in four patients. Corticosteroids were the mainstay of treatment for all five patients. All five patients survived and complete remission of symptoms was achieved in four out of five patients with the addition of cyclophosphamide and in one out of five with rituximab. A review of the literature was performed including a systemic review of the case reports and case series published in the English literature over the last 20 years.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Ju JH, Min J-K, Jung C-K et al (2009) Lupus mesenteric vasculitis can cause acute abdominal pain in patients with SLE. Nat Rev Rheumatol 5:273–281. doi:10.1038/nrrheum.2009.53

    Article  PubMed  Google Scholar 

  2. Tu Y-L, Yeh K-W, Chen L-C et al (2011) Differences in disease features between childhood-onset and adult-onset systemic lupus erythematosus patients presenting with acute abdominal pain. Semin Arthritis Rheum 40:447–454. doi:10.1016/j.semarthrit.2010.06.011

    Article  PubMed  Google Scholar 

  3. Yuan S, Ye Y, Chen D et al (2014) Lupus mesenteric vasculitis: clinical features and associated factors for the recurrence and prognosis of disease. Semin Arthritis Rheum 43:759–766. doi:10.1016/j.semarthrit.2013.11.005

    Article  CAS  PubMed  Google Scholar 

  4. Richer O, Ulinski T, Lemelle I et al (2007) Abdominal manifestations in childhood-onset systemic lupus erythematosus. Ann Rheum Dis 66:174–178. doi:10.1136/ard.2005.050070

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. Bader-Meunier B, Armengaud JB, Haddad E et al (2005) Initial presentation of childhood-onset systemic lupus erythematosus: a French multicenter study. J Pediatr 146:648–653. doi:10.1016/j.jpeds.2004.12.045

    Article  CAS  PubMed  Google Scholar 

  6. Yuan S, Lian F, Chen D et al (2013) Clinical features and associated factors of abdominal pain in systemic lupus erythematosus. J Rheumatol 40:2015–2022. doi:10.3899/jrheum.130492

    Article  PubMed  Google Scholar 

  7. Lee C-K, Ahn MS, Lee EY et al (2002) Acute abdominal pain in systemic lupus erythematosus: focus on lupus enteritis (gastrointestinal vasculitis). Ann Rheum Dis 61:547–550

    Article  PubMed  PubMed Central  Google Scholar 

  8. Buck AC, Serebro LH, Quinet RJ (2001) Subacute abdominal pain requiring hospitalization in a systemic lupus erythematosus patient: a retrospective analysis and review of the literature. Lupus 10:491–495

    Article  CAS  PubMed  Google Scholar 

  9. Lian TY, Edwards CJ, Chan SP, Chng HH (2003) Reversible acute gastrointestinal syndrome associated with active systemic lupus erythematosus in patients admitted to hospital. Lupus 12:612–616

    Article  CAS  PubMed  Google Scholar 

  10. Turner HE, Myszor MF, Bradlow A, David J (1996) Lupus or lupoid hepatitis with mesenteric vasculitis. Br J Rheumatol 35:1309–1311

    Article  CAS  PubMed  Google Scholar 

  11. Hizawa K, Iida M, Aoyagi K et al (1998) Double-contrast radiographic assessment of lupus-associated enteropathy. Clin Radiol 53:825–829

    Article  CAS  PubMed  Google Scholar 

  12. Hiraishi H, Konishi T, Ota S et al (1999) Massive gastrointestinal hemorrhage in systemic lupus erythematosus: successful treatment with corticosteroid pulse therapy. Am J Gastroenterol 94:3349–3353. doi:10.1111/j.1572-0241.1999.01551.x

    Article  CAS  PubMed  Google Scholar 

  13. Alcocer-Gouyonnet F, Chan-Nuñez C, Hernández J et al (2000) Acute abdomen and lupus enteritis: thrombocytopenia and pneumatosis intestinalis as indicators for surgery. Am Surg 66:193–195

    CAS  PubMed  Google Scholar 

  14. Lin WY, Lan JL, Wang SJ (2001) Lupus mesenteric vasculitis detected by Ga-67 scan. Clin Nucl Med 26:726

    Article  CAS  PubMed  Google Scholar 

  15. Passam FH, Diamantis ID, Perisinaki G et al (2004) Intestinal ischemia as the first manifestation of vasculitis. Semin Arthritis Rheum 34:431–441

    Article  PubMed  Google Scholar 

  16. Kaneko Y, Hirakata M, Suwa A et al (2004) Systemic lupus erythematosus associated with recurrent lupus enteritis and peritonitis. Clin Rheumatol 23:351–354. doi:10.1007/s10067-004-0882-y

    Article  PubMed  Google Scholar 

  17. Nguyen VA, Pachinger O, Jaschke W et al. Severe mesenteric and coronary vasculitis: a late and uncommon complication in a patient with chronic discoid lupus erythematosus. Scand J Rheumatol 35:491–494

  18. Kishimoto M, Nasir A, Mor A, Belmont H (2007) Acute gastrointestinal distress syndrome in patients with systemic lupus erythematosus. Lupus 16:137–141. doi:10.1177/0961203306075739

    Article  CAS  PubMed  Google Scholar 

  19. Endo H, Kondo Y, Kawagoe K et al (2007) Lupus enteritis detected by capsule endoscopy. Intern Med 46:1621–1622

    Article  PubMed  Google Scholar 

  20. Waite L, Morrison E (2007) Severe gastrointestinal involvement in systemic lupus erythematosus treated with rituximab and cyclophosphamide (B-cell depletion therapy). Lupus 16:841–842. doi:10.1177/0961203307081118

    Article  CAS  PubMed  Google Scholar 

  21. Assimakopoulos SF, Dimitropoulou D, Liossis S-NC (2008) Education and imaging. Gastrointestinal: intestinal vasculitis associated with systemic lupus erythematosus. J Gastroenterol Hepatol 23:992. doi:10.1111/j.1440-1746.2008.05468.x

    Article  CAS  PubMed  Google Scholar 

  22. Kornu R, Oliver QZ, Reimold AM (2008) Recognizing concomitant lupus enteritis and lupus cystitis. J Clin Rheumatol 14:226–229. doi:10.1097/RHU.0b013e318181a8ec

    Article  PubMed  Google Scholar 

  23. Saito H, Kobayashi H, Watanabe K et al (2008) Usefulness of intraoperative esophagogastroduodenoscopy in a patient with lupus enteritis. Fukushima J Med Sci 54:38–42

    Article  PubMed  Google Scholar 

  24. Tu Y, Chen L, Ou L, Huang J (2009) Case report. Mesenteric vasculitis as the initial presentation in children with systemic lupus erythematosus. 4:251–253

  25. Akitake R, Nakase H, Ueno S et al (2009) Involvement of lupus enteritis in a patient with lupus cystitis and nephritis. Digestion 80:160–164. doi:10.1159/000227135

    Article  PubMed  Google Scholar 

  26. Huang D-F, Chen W-S (2009) Images in clinical medicine. Lupus-associated intestinal vasculitis. N Engl J Med 361:e3. doi:10.1056/NEJMicm0801958

    Article  CAS  PubMed  Google Scholar 

  27. De Carvalho JF (2010) Mesenteric vasculitis in a systemic lupus erythematosus patient with a low sledai: an uncommon presentation. Clinics (Sao Paulo) 65:337–340. doi:10.1590/S1807-59322010000300016

    Article  Google Scholar 

  28. Lee CK, Lee TH, Lee S-H et al (2010) GI vasculitis associated with systemic lupus erythematosus. Gastrointest Endosc 72:618–619. doi:10.1016/j.gie.2010.02.033

    Article  PubMed  Google Scholar 

  29. Marinello DK, Rafael D, Paiva EDS, Dominoni RL. Systemic lupus erythematosus complicated by intestinal vasculitis and pneumatosis intestinalis. Rev Bras Reumatol 50:596–602

  30. Oh JS, Kim Y-G, Lee SG et al (2010) Successful treatment of recurrent lupus enteritis with rituximab. Lupus 19:220–222. doi:10.1177/0961203309345787

    Article  CAS  PubMed  Google Scholar 

  31. Wakui H, Togashi M, Omokawa A et al (2010) Simultaneous herpes simplex virus esophagitis and lupus enteritis in a patient with systemic lupus erythematosus. Mod Rheumatol 20:98–101. doi:10.1007/s10165-009-0237-2

    Article  PubMed  Google Scholar 

  32. Moon S-J, Kwok S-K, Park K-S et al (2011) Simultaneous presentation of hemophagocytic syndrome and mesenteric vasculitis in a patient with systemic lupus erythematosus. Mod Rheumatol 21:330–333. doi:10.1007/s10165-010-0401-8

    Article  PubMed  Google Scholar 

  33. Lin H-P, Wang Y-M, Huo A-P (2011) Severe, recurrent lupus enteritis as the initial and only presentation of systemic lupus erythematosus in a middle-aged woman. J Microbiol Immunol Infect 44:152–155. doi:10.1016/j.jmii.2009.12.001

    Article  PubMed  Google Scholar 

  34. Rasalkar DD, Paunipagar BK (2011) Mesenteric vasculitis with genitourinary tract involvement in systemic lupus erythematosus. Postgrad Med J 87:503–504. doi:10.1136/pgmj.2011.118893

    Article  PubMed  Google Scholar 

  35. Al Balushi F, Humby F, Mahto A et al (2012) Mycophenolate mofetil inducing remission of lupus enteritis. Lupus 21:556–558. doi:10.1177/0961203311430219

    Article  CAS  PubMed  Google Scholar 

  36. Majithia R, Joy G, Liang J, Olden K (2012) Acute abdominal pain in systemic lupus erythematosus. Gastrointest Endosc 75:1267–1268. doi:10.1016/j.gie.2012.01.028

    Article  PubMed  Google Scholar 

  37. Tan T-C, Wansaicheong GK-L, Thong BY-H (2012) Acute onset of systemic lupus erythematosus with extensive gastrointestinal and genitourinary involvement. Lupus 21:1240–1243. doi:10.1177/0961203312455111

    Article  PubMed  Google Scholar 

  38. Li R, Chen Z (2012) Intestinal obstruction as the initial presentation of lupus mesenteric vasculitis. J Clin Rheumatol 18:319–320. doi:10.1097/RHU.0b013e3182685f27

    Article  PubMed  Google Scholar 

  39. Smith LW, Petri M (2013) Lupus enteritis: an uncommon manifestation of systemic lupus erythematosus. J Clin Rheumatol 19:84–86. doi:10.1097/RHU.0b013e318284794e

    Article  PubMed  PubMed Central  Google Scholar 

  40. Alsalameh S, Hauggaard A (2013) Mesenteric vasculitis in active systemic lupus erythematosus causing diffuse abdominal pain. Rheumatology (Oxford) 52:1889. doi:10.1093/rheumatology/ket281

    Article  Google Scholar 

  41. Janssens P, Arnaud L, Galicier L et al (2013) Lupus enteritis: from clinical findings to therapeutic management. Orphanet J Rare Dis 8:67. doi:10.1186/1750-1172-8-67

    Article  PubMed  PubMed Central  Google Scholar 

  42. Kamata Y, Minota S (2013) Three-pointed star sign of lupus enteritis. Rheumatology (Oxford) 52:1008. doi:10.1093/rheumatology/kes426

    Article  Google Scholar 

  43. Lin Y-J, Chen P-C, Chen H-A (2013) Mesenteric vasculitis causing ileocecal intussusception as the initial presentation of systemic lupus erythematosus: a case report. Clin Rheumatol 32(Suppl 1):S37–S40. doi:10.1007/s10067-010-1421-7

    Article  PubMed  Google Scholar 

  44. Ashouri JF, Davis JL, Farkas A et al (2012) A young woman with systemic lupus erythematosus and extensive mesenteric vasculitis involving small and medium vessels. Arthritis Care Res 64:1928–1933. doi:10.1002/acr.21833

    Article  Google Scholar 

  45. Byun JY, Ha HK, Yu SY et al (1999) CT features of systemic lupus erythematosus in patients with acute abdominal pain: emphasis on ischemic bowel disease. Radiology 211:203–209. doi:10.1148/radiology.211.1.r99mr17203

    Article  CAS  PubMed  Google Scholar 

  46. Ko SF, Lee TY, Cheng TT et al (1997) CT findings at lupus mesenteric vasculitis. Acta Radiol 38:115–120

    Article  CAS  PubMed  Google Scholar 

  47. Taourel PG, Deneuville M, Pradel JA et al (1996) Acute mesenteric ischemia: diagnosis with contrast-enhanced CT. Radiology 199:632–636. doi:10.1148/radiology.199.3.8637978

    Article  CAS  PubMed  Google Scholar 

  48. Shirato M, Hisa N, Fujikura Y et al (1992) Imaging diagnosis of lupus enteritis—especially about sonographic findings. Nihon Igaku Hoshasen Gakkai Zasshi 52:1394–1399

    CAS  PubMed  Google Scholar 

  49. Grimbacher B, Huber M, von Kempis J et al (1998) Successful treatment of gastrointestinal vasculitis due to systemic lupus erythematosus with intravenous pulse cyclophosphamide: a clinical case report and review of the literature. Br J Rheumatol 37:1023–1028

    Article  CAS  PubMed  Google Scholar 

  50. Kwok S-K, Seo S-H, Ju JH et al (2007) Lupus enteritis: clinical characteristics, risk factor for relapse and association with anti-endothelial cell antibody. Lupus 16:803–809. doi:10.1177/0961203307082383

    Article  CAS  PubMed  Google Scholar 

  51. Sultan SM, Ioannou Y, Isenberg DA (1999) A review of gastrointestinal manifestations of systemic lupus erythematosus. Rheumatology (Oxford) 38:917–932

    Article  CAS  Google Scholar 

  52. Martin-Suarez I, D’Cruz D, Mansoor M et al (1997) Immunosuppressive treatment in severe connective tissue diseases: effects of low dose intravenous cyclophosphamide. Ann Rheum Dis 56:481–487

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  53. Kim YG, Ha HK, Nah SS et al (2006) Acute abdominal pain in systemic lupus erythematosus: factors contributing to recurrence of lupus enteritis. Ann Rheum Dis 65:1537–1538. doi:10.1136/ard.2006.053264

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  54. Duxbury B, Combescure C, Chizzolini C (2013) Rituximab in systemic lupus erythematosus: an updated systematic review and meta-analysis. Lupus 22:1489–1503. doi:10.1177/0961203313509295

    Article  CAS  PubMed  Google Scholar 

  55. Medina F, Ayala A, Jara LJ et al (1997) Acute abdomen in systemic lupus erythematosus: the importance of early laparotomy. Am J Med 103:100–105

    Article  CAS  PubMed  Google Scholar 

  56. Koneru S, Kocharla L, Higgins GC et al (2008) Adherence to medications in systemic lupus erythematosus. J Clin Rheumatol 14:195–201. doi:10.1097/RHU.0b013e31817a242a

    PubMed  Google Scholar 

Download references

Conflict of interest

The authors declare they have no conflicts of interest.

Author information

Authors and Affiliations

  1. Pediatric Rheumatology Division, St. Louis Children’s Hospital, Washington University in St. Louis, One Children’s Place, St Louis, MO, 63110, USA

    Lampros Fotis, Kevin W. Baszis, Anthony R. French, Megan A. Cooper & Andrew J. White

Authors
  1. Lampros Fotis
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Kevin W. Baszis
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Anthony R. French
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Megan A. Cooper
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Andrew J. White
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Lampros Fotis.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Fotis, L., Baszis, K.W., French, A.R. et al. Mesenteric vasculitis in children with systemic lupus erythematosus. Clin Rheumatol 35, 785–793 (2016). https://doi.org/10.1007/s10067-015-2892-3

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-015-2892-3

Keywords

Search

Navigation