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Lupus enteritis: from clinical findings to therapeutic management

Janssens, Peter ; Arnaud, Laurent ; Galicier, Lionel ; Mathian, Alexis ; Hie, Miguel ; Sene, Damien ; Haroche, Julien ; Veyssier-Belot, Catherine ; Huynh-Charlier, Isabelle ; Grenier, Philippe A ; Piette, Jean-Charles ; Amoura, Zahir

Orphanet journal of rare diseases, 2013-05, Vol.8 (1), p.67-67, Article 67 [Periódico revisado por pares]

England: BioMed Central Ltd

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Título:
Lupus enteritis: from clinical findings to therapeutic management
Autor: Janssens, Peter ; Arnaud, Laurent ; Galicier, Lionel ; Mathian, Alexis ; Hie, Miguel ; Sene, Damien ; Haroche, Julien ; Veyssier-Belot, Catherine ; Huynh-Charlier, Isabelle ; Grenier, Philippe A ; Piette, Jean-Charles ; Amoura, Zahir
Assuntos: Adolescent ; Adrenal Cortex Hormones - therapeutic use ; Adult ; Care and treatment ; Complications and side effects ; Diagnosis ; Enteritis ; Enteritis - drug therapy ; Enteritis - etiology ; Enteritis - physiopathology ; Female ; Genetics ; Humans ; Immunosuppressive Agents - therapeutic use ; Life Sciences ; Lupus ; Lupus Erythematosus, Systemic - complications ; Lupus Erythematosus, Systemic - drug therapy ; Lupus Erythematosus, Systemic - physiopathology ; Middle Aged ; Review ; Vasculitis - complications ; Vasculitis - drug therapy
É parte de: Orphanet journal of rare diseases, 2013-05, Vol.8 (1), p.67-67, Article 67
Notas: SourceType-Scholarly Journals-1
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ObjectType-Feature-6
ObjectType-Review-2
ObjectType-Case Study-4
ObjectType-Undefined-1
ObjectType-Report-3
ObjectType-Article-5
Descrição: 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.
Editor: England: BioMed Central Ltd
Idioma: Inglês

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Lupus enteritis: from clinical findings to therapeutic management

Janssens, Peter ; Arnaud, Laurent ; Galicier, Lionel ; Mathian, Alexis ; Hie, Miguel ; Sene, Damien ; Haroche, Julien ; Veyssier-Belot, Catherine ; Huynh-Charlier, Isabelle ; Grenier, Philippe A ; Piette, Jean-Charles ; Amoura, Zahir

England: BioMed Central Ltd

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