Antineutrophil Cytoplasmic Antibody–associated Glomerulonephritis Complicated by Pneumatosis Intestinalis

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Abstract

Pneumatosis intestinalis is a characteristic imaging phenomenon indicating the presence of gas in the bowel wall. The link between pneumatosis intestinalis and various kinds of autoimmune diseases has been reported anecdotally, while information regarding the cases with antineutrophil cytoplasmic antibodies (ANCA)–associated vasculitis complicated by concurrent pneumatosis intestinalis is lacking. In this report, we describe our serendipitous experience with one such case of pneumatosis intestinalis in a patient with ANCA-associated glomerulonephritis. We also discuss several therapeutic concerns that arose in the current case, which had an impact on the pathogenesis of the disease.

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Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, Wegener granulomatosis, Churg-Strauss syndrome, or rheumatoid arthritis-associated vasculitis.

Loïc Guillevin, Alfred Mahr, Christian Pagnoux … (2005)

We reviewed the medical records of 62 patients with systemic small and medium-sized vessel vasculitides and gastrointestinal tract involvement followed at our institution between 1981 and 2002. This group included 46 men and 16 women (male:female ratio, 2.9), with a mean age of 48 +/- 18 years. Vasculitides were distributed as follows: 38 polyarteritis nodosa (21 related to hepatitis B virus), 11 Churg-Strauss syndrome, 6 Wegener granulomatosis, 4 microscopic polyangiitis, and 3 rheumatoid arthritis-associated vasculitis. Gastrointestinal manifestations were present at or occurred within 3 months of diagnosis in 50 (81%) patients and were mainly abdominal pain in 61 (97%), nausea or vomiting in 21 (34%), diarrhea in 17 (27%), hematochezia or melena in 10 (16%), and hematemesis in 4 (6%). Gastroduodenal ulcerations were detected endoscopically in 17 (27 %) patients, esophageal in 7 (11%), and colorectal in 6 (10%), but histologic signs of vasculitis were found in only 3 colon biopsies. Twenty-one (34%) patients had a surgical abdomen; 11 (18%) developed peritonitis, 9 (15%) had bowel perforations, 10 (16%) bowel ischemia/infarction, 4 (6%) intestinal occlusion, 6 (10%) acute appendicitis, 5 (8%) cholecystitis, and 3 (5%) acute pancreatitis. (Some patients had more than 1 condition.) Sixteen (26%) patients died.The respective 10-month and 5-year survival rates were 71% (95% confidence interval [CI], 52-90) and 56% (95% CI, 35-77) for the 21 surgical patients; and 94% (95% CI, 87-101) and 82% (95% CI, 70-94) for the 41 patients without surgical abdomen (p = 0.08). Peritonitis (hazard ratio [HR] = 4.3, p < 0.01), bowel perforations (HR = 5.7, p < 0.01), gastrointestinal ischemia or infarctions (HR = 4.1, p < 0.01), and intestinal occlusion (HR = 5.5, p < 0.01) were the only gastrointestinal manifestations significantly associated with increased mortality in multivariate analysis. For this subgroup of 15 patients, 6-month and 5-year survival rates were 60% (95% CI, 35-85) and 46% (95% CI, 19-73), respectively (p = 0.003). None of the other gastrointestinal or extraintestinal vasculitis-related symptoms, or angiographic abnormalities (seen in 67% of the 39 patients who underwent angiography), was predictive of surgical complications or poor outcome. However, prognosis has dramatically improved during the past 30 years, probably owing to better management of these more severely ill patients, with prompt surgical intervention when indicated, and the combined use of steroids and immunosuppressants.

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Natural history, clinical pattern, and surgical considerations of pneumatosis intestinalis

PN Khalil, S. Huber‐Wagner, R Ladurner … (2009)

Objective Pneumatosis intestinalis has been increasingly detected in recent years with the more frequent use of computed tomography for abdominal imaging of the intestine. The underlying causes of the gas found during radiographic studies of the bowel wall can vary widely and different hypotheses regarding its pathophysiology have been postulated. Pneumatosis intestinalis often represents a benign condition and should not be considered an argument for surgery. However, it can also require life-threatening surgery in some cases, and this can be a difficult decision in some patients. Methods The spectrum of pneumatosis intestinalis is discussed here based on various computed tomographic and surgical findings in patients who presented at our University Medical Centre in 2003-2008. We have also systematically reviewed the literature to establish the current understanding of its aetiology and pathophysiology, and the possible clinical conditions associated with pneumatosis intestinalis and their management. Results Pneumatosis intestinalis is a primary radiographic finding. After its diagnosis, its specific pathogenesis should be ascertained because the appropriate therapy is related to the underlying cause of pneumatosis intestinalis, and this is sometimes difficult to define. Surgical treatment should be considered urgent in symptomatic patients presenting with an acute abdomen, signs of ischemia, or bowel obstruction. In asymptomatic patients with otherwise inconspicuous findings, the underlying disease should be treated first, rather than urgent exploratory surgery considered. Extensive and comprehensive information on the pathophysiology and clinical findings of pneumatosis intestinalis is provided here and is incorporated into a treatment algorithm. Conclusions The information presented here allows a better understanding of the radiographic diagnosis and underlying aetiology of pneumatosis intestinalis, and may facilitate the decision-making process in this context, thus providing fast and adequate therapy to particular patients.

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Spontaneous pneumomediastinum: analysis of 62 consecutive adult patients.

Vivek Iyer, Avni Y Joshi, Jay H Ryu (2009)

To clarify the clinical presentation and course of patients with spontaneous pneumomediastinum (SP) and to determine the usefulness of diagnostic testing in these patients. We conducted a retrospective review of 62 consecutive adult patients (age >or=18 years) diagnosed as having SP during an 11-year period from July 1, 1997, to June 30, 2008. The study cohort included 41 men and 21 women (median age, 30 years; interquartile range, 20-69 years). Among the 62 study patients, the most common presenting symptoms were chest pain (39 patients [63%]), cough (28 [45%]), and dyspnea (27 [44%]). Preexisting lung diseases were identified in 27 patients (44%) and included interstitial lung disease, asthma, lung malignancies, bronchiolitis obliterans syndrome, chronic obstructive pulmonary disease, bronchiectasis, and cystic lung lesions. The initial diagnosis of SP was achieved by chest radiography in 52 patients (84%); the remaining cases were diagnosed by computed tomography. Forty-seven patients (76%) were hospitalized for a median duration of 2.5 days. Additional diagnostic procedures were performed in 27 patients (44%) and included contrast esophagography, bronchoscopy, and esophagogastroduodenoscopy; however, they did not yield a pathologic cause in any patient. Pneumothorax was identified in 20 patients (32%), but less than one-third of these patients underwent chest tube thoracostomy. No episodes of mediastinitis or sepsis occurred. Recurrence of SP was seen in 1 patient, and thoracoscopic surgery was performed in 1 patient for persistent air leak (pneumothorax). Spontaneous pneumomediastinum was associated with a relatively benign clinical course; however, pneumothorax was seen in 32% of cases. Diagnostic testing to determine a pathologic cause yielded little clinically relevant information in these patients.

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Journal

Journal ID (nlm-ta): Clin Med Insights Case Rep

Journal ID (iso-abbrev): Clin Med Insights Case Rep

Journal ID (publisher-id): Clinical Medicine Insights: Case Reports

Title: Clinical Medicine Insights. Case Reports

Publisher: Libertas Academica

ISSN (Electronic): 1179-5476

Publication date Collection: 2015

Publication date (Electronic): 10 August 2015

Volume: 8

Pages: 65-70

Affiliations

Division of Nephrology, Department of Internal Medicine, Jichi Medical University, Tochigi, Japan.

Author notes

CORRESPONDENCE: tetsu-a@ 123456jichi.ac.jp

Article

Publisher ID: ccrep-8-2015-065

DOI: 10.4137/CCRep.S26155

PMC ID: 4533850

PubMed ID: 26309421

SO-VID: 8c929273-3cd8-4bbd-9c1f-7ce600b2a495

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This is an open-access article distributed under the terms of the Creative Commons CC-BY-NC 3.0 License.

History

Date received : 20 March 2015

Date revision received : 17 June 2015

Date accepted : 19 June 2015

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Antineutrophil Cytoplasmic Antibody–associated Glomerulonephritis Complicated by Pneumatosis Intestinalis

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Most cited references 22

Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, Wegener granulomatosis, Churg-Strauss syndrome, or rheumatoid arthritis-associated vasculitis.

Natural history, clinical pattern, and surgical considerations of pneumatosis intestinalis

Spontaneous pneumomediastinum: analysis of 62 consecutive adult patients.

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