Systemic multifocal fibrosclerosis

Johal, Simran; Manjunath, Srikantaiah; Allen, C. M. Van; Trash, D. B.

doi:10.1136/pgmj.74.876.608

Cited by 3 publications

(1 citation statement)

References 4 publications

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“…The full texts of 530 articles were read, and 116 articles were shortlisted for the final extraction, 112 of which were case reports and 4 were case series. 3,18...…”

Section: Resultsmentioning

confidence: 99%

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

et al. 2023

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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.

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“…The full texts of 530 articles were read, and 116 articles were shortlisted for the final extraction, 112 of which were case reports and 4 were case series. 3,18...…”

Section: Resultsmentioning

confidence: 99%

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

et al. 2023

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Adhäsionen, sklerosierende Peritonitis und Mesenteritis

Offner

2013

Pathologie

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Subglottic and tracheal stenosis associated with multifocal fibrosclerosis

Orabi

Negam

Bulman

2007

Acta Oto-Laryngologica

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This is the first case report in the literature of idiopathic subglottic and tracheal stenosis in a patient diagnosed with multifocal fibrosclerosis (MFS). The patient had unilateral orbital pseudotumour, bilateral pretibal cutaneous manifestations and raised thyroid antibodies, which might indicate subclinical thyroid involvement. Urgent permanent tracheostomy was needed. The patient also underwent tracheal dilatation for her dysphonia. There was no evidence of brain, lung, liver or renal involvement. Although extremely rare, laryngologists should consider the possibility of such a diagnosis in a stridulous patient with multifocal fibrotic manifestations.

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Systemic multifocal fibrosclerosis

Cited by 3 publications

References 4 publications

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

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

Adhäsionen, sklerosierende Peritonitis und Mesenteritis

Subglottic and tracheal stenosis associated with multifocal fibrosclerosis

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