Lúpus eritematoso sistêmico complicado por vasculite intestinal e pneumatose intestinal

Marinello, Débora Karine; Rafael, Daniane; Paiva, Eduardo dos Santos; Dominoni, Robson Luiz

doi:10.1590/s0482-50042010000500011

Cited by 14 publications

(2 citation statements)

References 16 publications

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“…If a PCI is suspected, CT examination and ultrasound endoscopy should be performed when conditions permit, and the factors causing PCI should be explored. In addition to gastrointestinal diseases and emphysema, some rare events are associated to PCI, such as alpha-glucosidase inhibitors [7], sunitinib [20], lung transplantation [28], bone marrow transplantation [29], systemic lupus erythematosus [30], systemic sclerosis [11], myeloma [31], granulomatosis with polyangiitis [32]. Moreover, the mucosal damage caused by colonoscopy and biopsy may result in the gas entering the intestinal mucosa, thereby promoting the occurrence of PCI [33].…”

Section: Discussionmentioning

confidence: 99%

Pneumatosis cystoides intestinalis: a case report and literature review

2019

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BackgroundPneumatosis cystoides intestinalis (PCI) is a low-incidence disease that confuses many doctors. A vast number of factors are suspected to contribute to its pathogenesis, such as Crohn’s disease, intestinal stenosis, ulcerative colitis, drug use, extra-gastrointestinal diseases, and chronic obstructive pulmonary disease. Most consider its pathogenesis interrelated to an increase in intra-intestinal pressure and the accumulation of gas produced by aerogenic bacteria, and patients with atypical symptoms and imaging manifestations tend to be misdiagnosed.Case presentationA 64-year-old man complained of a 3-month history of bloody stool without mucopurulent discharge, abdominal pain, or diarrhea. Colonoscopy revealed multiple nodular projections into the segmental mucosa of the sigmoid colon. Crohn’s disease and malignant disease ware suspected first according to the patient’s history, but laboratory examinations did not confirm either. Endoscopic ultrasound (EUS) revealed multiple cystic lesions in the submucosa. Moreover, computer tomography scan showed multiple bubble-like cysts. Combined with ultrasonography, computed tomography, and pathology findings, we ultimately made a diagnosis of PCI. Instead of surgery, we recommended conservative treatment consisting of endoscopy and oral drug administration. His symptoms improved with drug therapy after discharge, and no recurrence was noted on follow-up.ConclusionsThe incidence of PCI is low. Due to a lack of specificity in clinical manifestations and endoscopic findings, it often misdiagnosed as intestinal polyps, tumors, inflammatory bowel disease, or other conditions. Colonoscopy, computed tomography, and ultrasonography have demonstrated benefit in patients with multiple nodular projections in colon. Compared to the treatment of the above diseases, PCI treatment is effective and convenient, and the prognosis is optimistic. Therefore, clinicians should increase their awareness of PCI to avoid unnecessary misdiagnosis.

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Section: Discussionmentioning

confidence: 99%

Pneumatosis cystoides intestinalis: a case report and literature review

2019

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“…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%

Lupus enteritis: from clinical findings to therapeutic management

Janssens

Arnaud

Galicier

et al. 2013

Orphanet J Rare Dis

124

170

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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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Clinical characteristics and prediction of the asymptomatic phenotype of pneumatosis intestinalis in critically ill patients: a retrospective observational study

Sato

Ohbe

Fujita

et al. 2020

Acute Medicine & Surgery

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Aim The differences in clinical characteristics between benign asymptomatic and symptomatic pneumatosis intestinalis (PI) remain unknown. This study aimed to reveal the clinical characteristics of PI in critically ill patients. Methods This was a retrospective observational study undertaken between 2013 and 2017 in a single facility. Patients with PI were enrolled. Pneumatosis intestinalis was diagnosed using computed tomography, and clinical data were collected. Pathologic PI refers to PI with bowel ischemia. Asymptomatic PI refers to PI with a benign etiology. Results There were 17 patients with pathologic PI and 31 with asymptomatic PI. Pathologic PI was detected at day 4 of hospital stay, and asymptomatic PI was detected at day 30 of hospital stay ( P < 0.01). The symptoms that were different between pathologic and asymptomatic PI were acute diarrhea (18% and 65%, P = 0.01), C‐reactive protein level elevation (9.9 and 2.1 mg/dL, P = 0.01), and systemic inflammatory reaction syndrome (100% and 13%, P < 0.01). Computed tomography findings showed a difference in the occurrence of ascites collection (94% versus 23%, P < 0.01) and PI of the ascending colon (47% versus 80%, P = 0.02). Hospital mortality of pathologic PI was 88%, whereas all patients with benign PI survived. The positive likelihood ratio of acute diarrhea with PI of the ascending colon to diagnose benign PI was 7.33 (1.11–48.5). Conclusions Pneumatosis intestinalis of the ascending colon that occurs in the post‐intensive care phase with a poor inflammatory reaction, acute diarrhea, and no ascites collection could be benign. In other cases, bowel ischemia should be promptly ruled out.

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Lúpus eritematoso sistêmico complicado por vasculite intestinal e pneumatose intestinal

Cited by 14 publications

References 16 publications

Pneumatosis cystoides intestinalis: a case report and literature review

Pneumatosis cystoides intestinalis: a case report and literature review

Lupus enteritis: from clinical findings to therapeutic management

Clinical characteristics and prediction of the asymptomatic phenotype of pneumatosis intestinalis in critically ill patients: a retrospective observational study

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