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      Systemic Lupus Erythematosus-associated Acute Acalculous Cholecystitis Successfully Treated by a Corticosteroid Combined with Azathioprine

      case-report

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          Abstract

          A 69-year-old Japanese woman was transferred to our hospital due to pancytopenia with a fever. She had Murphy's sign, and computed tomography showed pleural effusion and a swollen gallbladder without gallstones. We diagnosed her with systemic lupus erythematosus (SLE)-associated acute acalculous cholecystitis (AAC). Partly because her clinical and laboratory findings were not serious enough to warrant immediate surgical intervention, and partly because her poor general condition made her ineligible for surgery, surgical therapy was not selected. Corticosteroid therapy was performed with azathioprine, and the swelling in her gallbladder improved. As a conservative therapy for SLE-associated AAC, corticosteroid therapy combined with azathioprine might be beneficial.

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          EULAR recommendations for the management of large vessel vasculitis.

          C Mukhtyar, L Guillevin, M C Cid … (2009)
          To develop European League Against Rheumatism (EULAR) recommendations for the management of large vessel vasculitis. An expert group (10 rheumatologists, 3 nephrologists, 2 immunolgists, 2 internists representing 8 European countries and the USA, a clinical epidemiologist and a representative from a drug regulatory agency) identified 10 topics for a systematic literature search through a modified Delphi technique. In accordance with standardised EULAR operating procedures, recommendations were derived for the management of large vessel vasculitis. In the absence of evidence, recommendations were formulated on the basis of a consensus opinion. Seven recommendations were made relating to the assessment, investigation and treatment of patients with large vessel vasculitis. The strength of recommendations was restricted by the low level of evidence and EULAR standardised operating procedures. On the basis of evidence and expert consensus, management recommendations for large vessel vasculitis have been formulated and are commended for use in everyday clinical practice.
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            Acute acalculous cholecystitis: a review.

            Jason Huffman, Steven Schenker (2010)
            Although recognized for more than 150 years, acute acalculous cholecystitis (AAC) remains an elusive diagnosis. This is likely because of the complex clinical setting in which this entity develops, the lack of large prospective controlled trials that evaluate various diagnostic modalities, and thus dependence on a small data base for clinical decision making. AAC most often occurs in critically ill patients, especially related to trauma, surgery, shock, burns, sepsis, total parenteral nutrition, and/or prolonged fasting. Clinically, AAC is difficult to diagnose because the findings of right upper-quadrant pain, fever, leukocytosis, and abnormal liver tests are not specific. AAC is associated with a high mortality, but early diagnosis and intervention can change this. Early diagnosis is the crux of debate surrounding AAC, and it usually rests with imaging modalities. There are no specific criteria to diagnose AAC. Therefore, this review discusses the imaging methods most likely to arrive at an early and accurate diagnosis despite the complexities of the radiologic modalities. A pragmatic approach is vital. A timely diagnosis will depend on a high index of suspicion in the appropriate patient, and the combined results of clinical findings (admittedly nonspecific), plus properly interpreted imaging. Sonogram (often sequential) and hepatic iminodiacetic acid scans are the most reliable modalities for diagnosis. It is generally agreed that cholecystectomy is the definitive therapy for AAC. However, at times a diagnostic/therapeutic drainage via interventional radiology/surgery may be necessary and life-saving, and may be the only treatment needed. Copyright (c) 2010 AGA Institute. Published by Elsevier Inc. All rights reserved.
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              Clinical predictors of severe gallbladder complications in acute acalculous cholecystitis.

              Shuu-Jiun Wang, Shou-Ping Shih, En-Duo Wang … (2003)
              To evaluate the relationship between clinical information (including age, laboratory data, and sonographic findings) and severe complications, such as gangrene, perforation, or abscess, in patients with acute acalculous cholecystitis (AAC). The medical records of patients hospitalized from January 1997 to December 2002 with a diagnosis of acute cholecystitis were retrospectively reviewed to find those with AAC, confirmed at operation or by histologic examination. Data collected included age, sex, white blood cell count, AST, total bilirubin, alkaline phosphatase, bacteriology, mortality, and sonographic findings. The sonographic findings were recorded on a 3-point scale with 1 point each for gallbladder distention, gallbladder wall thickness >3.5 mm, and sludge. The patients were divided into 2 groups based on the presence (group A) or absence (group B) of severe gallbladder complications, defined as perforation, gangrene, or abscess. There were 52 cases of AAC, accounting for 3.7% of all cases of acute cholecystitis. Males predominated. Most patients were diagnosed by ultrasonography (48 of 52) or computed tomography (17 of 52). Severe gallbladder complications were present in 27 patients (52%, group A) and absent in 25 (group B). Six patients died with a mortality of 12%. Four of the 6 who died were in group A. Patients in group A were significantly older than those in group B (mean 60.88 y vs. 54.12 y, P=0.04) and had a significantly higher white blood cell count (mean 15,885.19 vs. 9,948.40, P=0.0005). All the 6 patients who died had normal white blood cell counts with an elevated percentage of band forms. The most commonly cultured bacteria in both blood and bile were E. coli and Klebsiella pneumoniae. The cumulative sonographic points did not reliably distinguish between groups A and B, even though group A tended to have more points. Older patients with a high white cell count are more likely to have severe gallbladder complications. In these patients, earlier surgical intervention should be considered if the sonographic findings support the diagnosis of AAC.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Intern Med
                Journal ID (iso-abbrev): Intern. Med
                Title: Internal Medicine
                Publisher: The Japanese Society of Internal Medicine
                ISSN (Print): 0918-2918
                ISSN (Electronic): 1349-7235
                Publication date (Electronic): 27 June 2019
                Publication date (Print): 1 October 2019
                Volume: 58
                Issue: 19
                Pages: 2879-2885
                Affiliations
                [1 ]Comprehensive Clinical Education, Training and Development Center, Kumamoto University Hospital, Japan
                [2 ]Section of Internal Medicine and Rheumatology, Kumamoto Shinto General Hospital, Japan
                [3 ]Division of Hematology, Kumamoto Shinto General Hospital, Japan
                [4 ]Department of Hepatology and Gastroenterology, Kumamoto Shinto General Hospital, Japan
                Author notes

                Correspondence to Dr.　Noritaka Kudo, kudo.noritaka@ 123456gmail.com

                Article
                DOI: 10.2169/internalmedicine.2820-19
                PMC ID: 6815888
                PubMed ID: 31243208
                SO-VID: 4917d55c-8191-4cf5-9417-fa4f181e462f
                Copyright © Copyright © 2019 by The Japanese Society of Internal Medicine
                License:

                The Internal Medicine is an Open Access journal distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit ( https://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                Date received : 12 February 2019
                Date accepted : 1 April 2019
                Categories
                Subject: Case Report

                Keywords: acute acalculous cholecystitis,systemic lupus erythematosus,corticosteroid,azathioprine
                Data availability:
                Keywords: acute acalculous cholecystitis, systemic lupus erythematosus, corticosteroid, azathioprine

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