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Review
. 2023 May 29:11:20503121231178046.
doi: 10.1177/20503121231178046. eCollection 2023.

Clinical presentation and organ-based outcomes of Multifocal fibrosclerosis: A systematic review

Ahmed Safi Vahidy  1 , Sameer Saleem Tebha  2 , Yeny Chavarria  3 , Sandip Kc  4 , Shristi Sharma  4
Affiliations
Review

Clinical presentation and organ-based outcomes of Multifocal fibrosclerosis: A systematic review

Ahmed Safi Vahidy et al. SAGE Open Med. .

Abstract

Objectives: Multifocal fibrosclerosis is a rare disorder causing progressive fibrosis of multiple organ systems. Existing data on the disease show that there are multiple manifestations of the disease, with different outcomes. However, quantitative data are scarce, prompting the need for our investigation.

Method: A comprehensive systematic review was performed from inception to November 16, 2022, with the restriction of English language, not including review articles. Article screening and extraction was performed independently, and shortlisted articles were assessed for bias. Analysis was performed using SPSS Version 25 (IBM® SPSS® Statistics; Chicago, IL, USA). Data were presented as frequencies and percentages, with a confidence interval of 95%.

Result: This review included 134 patients, with 78 (58.2%) males. Mean age was 53.6 years and included two pediatric patients. The most common comorbidity was diabetes (9.7%). Prevalent presenting symptoms included pain (47.8%) and swelling (35.1%). A mean of 2.51 organs or anatomical sites was affected, retroperitoneum (64.2%) being most affected. The pancreas (30.0%) and digestive system (47.0%) were the organs/organ systems most affected. Patients had favorable outcomes in 79.1% of cases, 87.3% had no relapse, and 91.8% of patients survived the condition.

Conclusion: The findings in this study provide a quantitative measurement of the demographics, presentations, organ manifestations, and outcomes of multifocal fibrosclerosis. We found the disease to be prevalent in males in Japan or the United States, with the abdomen and its organs being commonly involved.

Keywords: Multifocal fibrosclerosis; autoimmune pancreatitis; idiopathic fibrosis; retroperitoneal fibrosis.

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Conflict of interest statement

The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1.
Figure 1.
PRISMA diagram of included studies (n = 116). PRISMA: Preferred Reporting Items for Systematic Reviews and Meta-Analysis.
See this image and copyright information in PMC

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