Lupus enteritis as the only active manifestation of systemic lupus erythematosus: A case report
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摘要
BACKGROUND Lupus enteritis is a rare manifestation of systemic lupus erythematosus(SLE).Diagnosis of this condition is difficult, especially in the absence of other symptoms related to active SLE. We present the case of a 25-year-old female with lupus enteritis as the sole initial manifestation of active SLE.CASE SUMMARY A 25-year-old African American female presented to the Emergency Department complaining of diffuse abdominal pain, diarrhea, nausea, and vomiting for 2 days. Her past medical history was significant for seasonal allergies and family history was pertinent for discoid lupus in her father and SLE in a cousin. The patient's vital signs on presentation were normal. Her physical exam was remarkable for significant lower abdominal tenderness without guarding or rigidity. A computed tomography of the abdomen and pelvis revealed marked circumferential wall thickening and edema of the proximal and mid small bowel predominantly involving the submucosa. Our main differential diagnoses were intestinal angioedema and mesenteric vein thrombosis. However, mesenteric vessels were patent, and laboratory testing for hereditary angioedema showed a normal C1 Esterase Inhibitor level and low C3 and C4 levels. Infectious work-up was negative. Autoimmune tests showed elevated anti-nuclear antibodies(ANA)(13.6), anti-Smith antibody, and anti-ribonucleoprotein(anti-RNP) antibody. The patient was diagnosed with SLE enteritis. She was maintained on bowel rest,given intravenous hydration, and started on methylprednisolone 60 mg IV daily.She had significant improvement in her abdominal pain, diarrhea, and emesis after 2 days of treatment. Steroids were tapered and maintained on Hydroxychloroquine with no relapses one year after presentation.CONCLUSION This case of lupus enteritis represents a rare manifestation of SLE. Diagnosis requires clinical suspicion, characteristic imaging and laboratory tests.Endoscopic appearance and biopsies usually yield non-specific findings. High dose steroids are the preferred treatment modality for moderate and severe cases.
BACKGROUND Lupus enteritis is a rare manifestation of systemic lupus erythematosus(SLE).Diagnosis of this condition is difficult, especially in the absence of other symptoms related to active SLE. We present the case of a 25-year-old female with lupus enteritis as the sole initial manifestation of active SLE.CASE SUMMARY A 25-year-old African American female presented to the Emergency Department complaining of diffuse abdominal pain, diarrhea, nausea, and vomiting for 2 days. Her past medical history was significant for seasonal allergies and family history was pertinent for discoid lupus in her father and SLE in a cousin. The patient's vital signs on presentation were normal. Her physical exam was remarkable for significant lower abdominal tenderness without guarding or rigidity. A computed tomography of the abdomen and pelvis revealed marked circumferential wall thickening and edema of the proximal and mid small bowel predominantly involving the submucosa. Our main differential diagnoses were intestinal angioedema and mesenteric vein thrombosis. However, mesenteric vessels were patent, and laboratory testing for hereditary angioedema showed a normal C1 Esterase Inhibitor level and low C3 and C4 levels. Infectious work-up was negative. Autoimmune tests showed elevated anti-nuclear antibodies(ANA)(13.6), anti-Smith antibody, and anti-ribonucleoprotein(anti-RNP) antibody. The patient was diagnosed with SLE enteritis. She was maintained on bowel rest,given intravenous hydration, and started on methylprednisolone 60 mg IV daily.She had significant improvement in her abdominal pain, diarrhea, and emesis after 2 days of treatment. Steroids were tapered and maintained on Hydroxychloroquine with no relapses one year after presentation.CONCLUSION This case of lupus enteritis represents a rare manifestation of SLE. Diagnosis requires clinical suspicion, characteristic imaging and laboratory tests.Endoscopic appearance and biopsies usually yield non-specific findings. High dose steroids are the preferred treatment modality for moderate and severe cases.
BACKGROUND Lupus enteritis is a rare manifestation of systemic lupus erythematosus(SLE).Diagnosis of this condition is difficult, especially in the absence of other symptoms related to active SLE. We present the case of a 25-year-old female with lupus enteritis as the sole initial manifestation of active SLE.CASE SUMMARY A 25-year-old African American female presented to the Emergency Department complaining of diffuse abdominal pain, diarrhea, nausea, and vomiting for 2 days. Her past medical history was significant for seasonal allergies and family history was pertinent for discoid lupus in her father and SLE in a cousin. The patient's vital signs on presentation were normal. Her physical exam was remarkable for significant lower abdominal tenderness without guarding or rigidity. A computed tomography of the abdomen and pelvis revealed marked circumferential wall thickening and edema of the proximal and mid small bowel predominantly involving the submucosa. Our main differential diagnoses were intestinal angioedema and mesenteric vein thrombosis. However, mesenteric vessels were patent, and laboratory testing for hereditary angioedema showed a normal C1 Esterase Inhibitor level and low C3 and C4 levels. Infectious work-up was negative. Autoimmune tests showed elevated anti-nuclear antibodies(ANA)(13.6), anti-Smith antibody, and anti-ribonucleoprotein(anti-RNP) antibody. The patient was diagnosed with SLE enteritis. She was maintained on bowel rest,given intravenous hydration, and started on methylprednisolone 60 mg IV daily.She had significant improvement in her abdominal pain, diarrhea, and emesis after 2 days of treatment. Steroids were tapered and maintained on Hydroxychloroquine with no relapses one year after presentation.CONCLUSION This case of lupus enteritis represents a rare manifestation of SLE. Diagnosis requires clinical suspicion, characteristic imaging and laboratory tests.Endoscopic appearance and biopsies usually yield non-specific findings. High dose steroids are the preferred treatment modality for moderate and severe cases.
引文
1 Helmick CG,Felson DT,Lawrence RC,Gabriel S,Hirsch R,Kwoh CK,Liang MH,Kremers HM,Mayes MD,Merkel PA,Pillemer SR,Reveille JD,Stone JH;National Arthritis Data Workgroup.Estimates of the prevalence of arthritis and other rheumatic conditions in the United States.Part I.Arthritis Rheum 2008;58:15-25[PMID:18163481 DOI:10.1002/art.23177]
2 Koo BS,Hong S,Kim YJ,Kim YG,Lee CK,Yoo B.Lupus enteritis:clinical characteristics and predictive factors for recurrence.Lupus 2015;24:628-632[PMID:25391541 DOI:10.1177/0961203314558858]
3 Brewer BN,Kamen DL.Gastrointestinal and Hepatic Disease in Systemic Lupus Erythematosus.Rheum Dis Clin North Am 2018;44:165-175[PMID:29149925 DOI:10.1016/j.rdc.2017.09.011]
4 Lin HP,Wang YM,Huo AP.Severe,recurrent lupus enteritis as the initial and only presentation of systemic lupus erythematosus in a middle-aged woman.J Microbiol Immunol Infect 2011;44:152-155[PMID:21439520 DOI:10.1016/j.jmii.2009.12.001]
5 Mushtaq H,Razzaque S,Ahmed K.Lupus Enteritis:An Atypical Initial Presentation of Systemic Lupus Erythematosus.J Coll Physicians Surg Pak 2018;28:160-161[PMID:29394979 DOI:10.29271/jcpsp.2018.02.160]
6 Anoosh F,Shariff R,Ambujakshan D,Nandipati KC,Turner JW,Mandava N.Acute abdomen as initial presentation in a patient with Systemic Lupus Erythematosus.Am J Case Rep 2009;10:55-58
7 Seyyedmajidi M,Vafaeimanesh J.Severe,Recurrent Mesenteric Vasculitis as the Initial Presentation of Systemic Lupus Erythematosus.Zahedan J Res Med Sci 2014;16:55-56
8 Shwarzbaum D,Rubinov J,Oikonomou I.P2472-On target:A rare case of Lupus Enteritis as the initial presentation of Systemic Lupus Erythematosus.World Congress of Gastroenterology at ACG2017Meeting Abstracts.Orlando,FL:American College of Gastroenterology.Available from:URL:https://eventscribe.com/2017/wcogacg2017/ajaxcalls/PosterInfo.asp?efp=S1lVTUxLQVozODMy&PosterID=116114&rnd=0.3164736
9 Chowichian M,Aanpreung P,Pongpaibul A,Charuvanij S.Lupus enteritis as the sole presenting feature of systemic lupus erythematosus:case report and review of the literature.Paediatr Int Child Health 2018;1-5[PMID:30191770 DOI:10.1080/20469047.2018.1504430]
10 Chung HV,Ramji A,Davis JE,Chang S,Reid GD,Salh B,Freeman HJ,Yoshida EM.Abdominal pain as the initial and sole clinical presenting feature of systemic lupus erythematosus.Can J Gastroenterol 2003;17:111-113[PMID:12605248 DOI:10.1155/2003/768184]
11 Tu YL,Chen LC,Ou LH,Huang JL.Mesenteric vasculitis as the initial presentation in children with systemic lupus erythematosus.J Pediatr Gastroenterol Nutr 2009;49:251-253[PMID:19543109 DOI:10.1097/MPG.0b013e31819f1df4]
12 Stoddard CJ,Kay PH,Simms JM,Kennedy A,Hughes P.Acute abdominal complications of systemic lupus erythematosus.Br J Surg 1978;65:625-628[PMID:698534]
13 Janssens P,Arnaud L,Galicier L,Mathian A,Hie M,Sene D,Haroche J,Veyssier-Belot C,HuynhCharlier I,Grenier PA,Piette JC,Amoura Z.Lupus enteritis:from clinical findings to therapeutic management.Orphanet J Rare Dis 2013;8:67[PMID:23642042 DOI:10.1186/1750-1172-8-67]
14 Petri M,Orbai AM,Alarcón GS,Gordon C,Merrill JT,Fortin PR,Bruce IN,Isenberg D,Wallace DJ,Nived O,Sturfelt G,Ramsey-Goldman R,Bae SC,Hanly JG,Sánchez-Guerrero J,Clarke A,Aranow C,Manzi S,Urowitz M,Gladman D,Kalunian K,Costner M,Werth VP,Zoma A,Bernatsky S,RuizIrastorza G,Khamashta MA,Jacobsen S,Buyon JP,Maddison P,Dooley MA,van Vollenhoven RF,Ginzler E,Stoll T,Peschken C,Jorizzo JL,Callen JP,Lim SS,Fessler BJ,Inanc M,Kamen DL,Rahman A,Steinsson K,Franks AG,Sigler L,Hameed S,Fang H,Pham N,Brey R,Weisman MH,McGwin G,Magder LS.Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus.Arthritis Rheum 2012;64:2677-2686[PMID:22553077 DOI:10.1002/art.34473]
15 Lee CK,Ahn MS,Lee EY,Shin JH,Cho YS,Ha HK,Yoo B,Moon HB.Acute abdominal pain in systemic lupus erythematosus:focus on lupus enteritis(gastrointestinal vasculitis).Ann Rheum Dis 2002;61:547-550[PMID:12006332 DOI:10.1136/ard.61.6.547]
16 Lee HA,Shim HG,Seo YH,Choi SJ,Lee BJ,Lee YH,Ji JD,Kim JH,Song GG.Panenteritis as an Initial Presentation of Systemic Lupus Erythematosus.Korean J Gastroenterol 2016;67:107-111[PMID:26907488 DOI:10.4166/kjg.2016.67.2.107]
17 Bodh V,Kalwar R,Sharma R,Sharma B,Mahajan S,Raina R,Jarial A.Lupus enteritis:An uncommon manifestation of systemic lupus erythematous as an initial presentation.J Dig Endosc 2017;8:134-136[DOI:10.4103/jde.JDE_36_16]
18 Patro PS,Phatak S,Zanwar A,Lawrence A.Presumptive Lupus Enteritis.Am J Med 2016;129:e277-e278[PMID:27235005 DOI:10.1016/j.amjmed.2016.04.032]
19 Demiselle J,Sayegh J,Cousin M,Olivier A,Augusto JF.An Unusual Cause of Abdominal Pain:Lupus Enteritis.Am J Med 2016;129:e11-e12[PMID:26841297 DOI:10.1016/j.amjmed.2016.01.011]
20 Tian XP,Zhang X.Gastrointestinal involvement in systemic lupus erythematosus:insight into pathogenesis,diagnosis and treatment.World J Gastroenterol 2010;16:2971-2977[PMID:20572299DOI:10.3748/wjg.v16.i24.2971]
21 Kim YG,Ha HK,Nah SS,Lee CK,Moon HB,Yoo B.Acute abdominal pain in systemic lupus erythematosus:factors contributing to recurrence of lupus enteritis.Ann Rheum Dis 2006;65:1537-1538[PMID:17038460 DOI:10.1136/ard.2006.053264]
2 Koo BS,Hong S,Kim YJ,Kim YG,Lee CK,Yoo B.Lupus enteritis:clinical characteristics and predictive factors for recurrence.Lupus 2015;24:628-632[PMID:25391541 DOI:10.1177/0961203314558858]
3 Brewer BN,Kamen DL.Gastrointestinal and Hepatic Disease in Systemic Lupus Erythematosus.Rheum Dis Clin North Am 2018;44:165-175[PMID:29149925 DOI:10.1016/j.rdc.2017.09.011]
4 Lin HP,Wang YM,Huo AP.Severe,recurrent lupus enteritis as the initial and only presentation of systemic lupus erythematosus in a middle-aged woman.J Microbiol Immunol Infect 2011;44:152-155[PMID:21439520 DOI:10.1016/j.jmii.2009.12.001]
5 Mushtaq H,Razzaque S,Ahmed K.Lupus Enteritis:An Atypical Initial Presentation of Systemic Lupus Erythematosus.J Coll Physicians Surg Pak 2018;28:160-161[PMID:29394979 DOI:10.29271/jcpsp.2018.02.160]
6 Anoosh F,Shariff R,Ambujakshan D,Nandipati KC,Turner JW,Mandava N.Acute abdomen as initial presentation in a patient with Systemic Lupus Erythematosus.Am J Case Rep 2009;10:55-58
7 Seyyedmajidi M,Vafaeimanesh J.Severe,Recurrent Mesenteric Vasculitis as the Initial Presentation of Systemic Lupus Erythematosus.Zahedan J Res Med Sci 2014;16:55-56
8 Shwarzbaum D,Rubinov J,Oikonomou I.P2472-On target:A rare case of Lupus Enteritis as the initial presentation of Systemic Lupus Erythematosus.World Congress of Gastroenterology at ACG2017Meeting Abstracts.Orlando,FL:American College of Gastroenterology.Available from:URL:https://eventscribe.com/2017/wcogacg2017/ajaxcalls/PosterInfo.asp?efp=S1lVTUxLQVozODMy&PosterID=116114&rnd=0.3164736
9 Chowichian M,Aanpreung P,Pongpaibul A,Charuvanij S.Lupus enteritis as the sole presenting feature of systemic lupus erythematosus:case report and review of the literature.Paediatr Int Child Health 2018;1-5[PMID:30191770 DOI:10.1080/20469047.2018.1504430]
10 Chung HV,Ramji A,Davis JE,Chang S,Reid GD,Salh B,Freeman HJ,Yoshida EM.Abdominal pain as the initial and sole clinical presenting feature of systemic lupus erythematosus.Can J Gastroenterol 2003;17:111-113[PMID:12605248 DOI:10.1155/2003/768184]
11 Tu YL,Chen LC,Ou LH,Huang JL.Mesenteric vasculitis as the initial presentation in children with systemic lupus erythematosus.J Pediatr Gastroenterol Nutr 2009;49:251-253[PMID:19543109 DOI:10.1097/MPG.0b013e31819f1df4]
12 Stoddard CJ,Kay PH,Simms JM,Kennedy A,Hughes P.Acute abdominal complications of systemic lupus erythematosus.Br J Surg 1978;65:625-628[PMID:698534]
13 Janssens P,Arnaud L,Galicier L,Mathian A,Hie M,Sene D,Haroche J,Veyssier-Belot C,HuynhCharlier I,Grenier PA,Piette JC,Amoura Z.Lupus enteritis:from clinical findings to therapeutic management.Orphanet J Rare Dis 2013;8:67[PMID:23642042 DOI:10.1186/1750-1172-8-67]
14 Petri M,Orbai AM,Alarcón GS,Gordon C,Merrill JT,Fortin PR,Bruce IN,Isenberg D,Wallace DJ,Nived O,Sturfelt G,Ramsey-Goldman R,Bae SC,Hanly JG,Sánchez-Guerrero J,Clarke A,Aranow C,Manzi S,Urowitz M,Gladman D,Kalunian K,Costner M,Werth VP,Zoma A,Bernatsky S,RuizIrastorza G,Khamashta MA,Jacobsen S,Buyon JP,Maddison P,Dooley MA,van Vollenhoven RF,Ginzler E,Stoll T,Peschken C,Jorizzo JL,Callen JP,Lim SS,Fessler BJ,Inanc M,Kamen DL,Rahman A,Steinsson K,Franks AG,Sigler L,Hameed S,Fang H,Pham N,Brey R,Weisman MH,McGwin G,Magder LS.Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus.Arthritis Rheum 2012;64:2677-2686[PMID:22553077 DOI:10.1002/art.34473]
15 Lee CK,Ahn MS,Lee EY,Shin JH,Cho YS,Ha HK,Yoo B,Moon HB.Acute abdominal pain in systemic lupus erythematosus:focus on lupus enteritis(gastrointestinal vasculitis).Ann Rheum Dis 2002;61:547-550[PMID:12006332 DOI:10.1136/ard.61.6.547]
16 Lee HA,Shim HG,Seo YH,Choi SJ,Lee BJ,Lee YH,Ji JD,Kim JH,Song GG.Panenteritis as an Initial Presentation of Systemic Lupus Erythematosus.Korean J Gastroenterol 2016;67:107-111[PMID:26907488 DOI:10.4166/kjg.2016.67.2.107]
17 Bodh V,Kalwar R,Sharma R,Sharma B,Mahajan S,Raina R,Jarial A.Lupus enteritis:An uncommon manifestation of systemic lupus erythematous as an initial presentation.J Dig Endosc 2017;8:134-136[DOI:10.4103/jde.JDE_36_16]
18 Patro PS,Phatak S,Zanwar A,Lawrence A.Presumptive Lupus Enteritis.Am J Med 2016;129:e277-e278[PMID:27235005 DOI:10.1016/j.amjmed.2016.04.032]
19 Demiselle J,Sayegh J,Cousin M,Olivier A,Augusto JF.An Unusual Cause of Abdominal Pain:Lupus Enteritis.Am J Med 2016;129:e11-e12[PMID:26841297 DOI:10.1016/j.amjmed.2016.01.011]
20 Tian XP,Zhang X.Gastrointestinal involvement in systemic lupus erythematosus:insight into pathogenesis,diagnosis and treatment.World J Gastroenterol 2010;16:2971-2977[PMID:20572299DOI:10.3748/wjg.v16.i24.2971]
21 Kim YG,Ha HK,Nah SS,Lee CK,Moon HB,Yoo B.Acute abdominal pain in systemic lupus erythematosus:factors contributing to recurrence of lupus enteritis.Ann Rheum Dis 2006;65:1537-1538[PMID:17038460 DOI:10.1136/ard.2006.053264]