Mesenteric Vasculitis as the Initial Presentation in Children With Systemic Lupus Erythematosus

Tu, Yu-Ling; Chen, Li‐Chen; Ou, Liang-Hsiao; Huang, Jing‐Long

doi:10.1097/mpg.0b013e31819f1df4

Cited by 13 publications

(6 citation statements)

References 11 publications

(13 reference statements)

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“…Abdominal vessel vasculitis, with or without bowel perforation is rare. 38 A sterile peritonitis occurs in fewer than 10% of patients, leading to abdominal pain and ascites, and is akin to pleuritis and pericarditis (i.e. “serositis”).…”

Section: Clinical Featuresmentioning

confidence: 99%

Systemic Lupus Erythematosus in Children and Adolescents

Levy¹,

Kamphuis²

2012

Pediatric Clinics of North America

275

237

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Synopsis Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease that can involve any organ system with a wide range of disease manifestations, and can lead to significant morbidity and even mortality. This article reviews the epidemiology, common clinical features, complications of disease, and briefly discusses the available treatment options. In addition, important medical and psychosocial issues relevant to the pediatrician caring for children and adolescents with SLE are discussed.

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Section: Clinical Featuresmentioning

confidence: 99%

Systemic Lupus Erythematosus in Children and Adolescents

Levy¹,

Kamphuis²

2012

Pediatric Clinics of North America

275

237

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“…Mean serum protein was 5.7 g/dL (17 patients). One patient had renal failure [43]; another had elevated transaminases and amylase [29]. Positive aPL antibodies were detected in 25/84 patients (30%).…”

Section: Introductionmentioning

confidence: 99%

“…Additional immunosuppression on initial treatment (n = 11) consisted of PO (n = 1) or IV cyclophosphamide (CYC) at 500 mg/m 2 to 750mg/m 2 . This was justified by concomitant severe organ involvement in 5 (central nervous system involvement in 2 [32,43], lupus nephritis in 3 [18,47]), associated intestinal necrosis in 4 [12,19,29,35,37] and persistent abdominal pain despite of IV steroids in one patient [12]. Seventeen patients underwent laparotomy, of which 10 (7%) had resection for necrosis or perforation.…”

Section: Introductionmentioning

confidence: 99%

“…On recurrent episodes, corticosteroids were used similar to first episodes. Additional immunosuppression consisted of IV CYC (n = 4) [1,25,43,44], MMF (n = 4) [25,34,44], AZA (n = 4) [25,34,43], Rituximab (n = 2) [34,44] and PO CYC (n = 1) [34]. Declared morbidity included short bowel syndrome with home total parenteral nutrition (n = 1) [37], recurrent abdominal bloating and dyspepsia (n = 1) [25] and recurrent abdominal pain without imaging abnormalities (n = 2).…”

Section: Introductionmentioning

confidence: 99%

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Lupus enteritis: from clinical findings to therapeutic management

Janssens

Arnaud

Galicier

et al. 2013

Orphanet J Rare Dis

130

181

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Lupus enteritis is a rare and poorly understood cause of abdominal pain in patients with systemic lupus erythematosus (SLE). In this study, we report a series of 7 new patients with this rare condition who were referred to French tertiary care centers and perform a systematic literature review of SLE cases fulfilling the revised ACR criteria, with evidence for small bowel involvement, excluding those with infectious enteritis. We describe the characteristics of 143 previously published and 7 new cases. Clinical symptoms mostly included abdominal pain (97%), vomiting (42%), diarrhea (32%) and fever (20%). Laboratory features mostly reflected lupus activity: low complement levels (88%), anemia (52%), leukocytopenia or lymphocytopenia (40%) and thrombocytopenia (21%). Median CRP level was 2.0 mg/dL (range 0–8.2 mg/dL). Proteinuria was present in 47% of cases. Imaging studies revealed bowel wall edema (95%), ascites (78%), the characteristic target sign (71%), mesenteric abnormalities (71%) and bowel dilatation (24%). Only 9 patients (6%) had histologically confirmed vasculitis. All patients received corticosteroids as a first-line therapy, with additional immunosuppressants administered either from the initial episode or only in case of relapse (recurrence rate: 25%). Seven percent developed intestinal necrosis or perforation, yielding a mortality rate of 2.7%. Altogether, lupus enteritis is a poorly known cause of abdominal pain in SLE patients, with distinct clinical and therapeutic features. The disease may evolve to intestinal necrosis and perforation if untreated. Adding with this an excellent steroid responsiveness, timely diagnosis becomes primordial for the adequate management of this rare entity.

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“…Treatment with low-dose cyclophosphamide (500 mg/week) followed by monthly pulses is widely used for the management of severe connective tissue diseases [52] and has been successful in multiple cases nonresponsive to steroid treatment [3,9,49]. In pediatric cases, the successful use of cyclophosphamide (0.5-1.0 g/m 2 ) is reported [24].…”

Section: Casementioning

confidence: 97%

Mesenteric vasculitis in children with systemic lupus erythematosus

et al. 2015

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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.

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Mesenteric Vasculitis as the Initial Presentation in Children With Systemic Lupus Erythematosus

Cited by 13 publications

References 11 publications

Systemic Lupus Erythematosus in Children and Adolescents

Systemic Lupus Erythematosus in Children and Adolescents

Lupus enteritis: from clinical findings to therapeutic management

Mesenteric vasculitis in children with systemic lupus erythematosus

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