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Review
. 2021 May 3;10(9):1963.
doi: 10.3390/jcm10091963.

Syndrome of Undifferentiated Recurrent Fever (SURF): An Emerging Group of Autoinflammatory Recurrent Fevers

Riccardo Papa  1 , Federica Penco  1 , Stefano Volpi  1 , Diana Sutera  1 , Roberta Caorsi  1 , Marco Gattorno  1
Affiliations
Review

Syndrome of Undifferentiated Recurrent Fever (SURF): An Emerging Group of Autoinflammatory Recurrent Fevers

Riccardo Papa et al. J Clin Med. .

Abstract

Syndrome of undifferentiated recurrent fever (SURF) is a heterogeneous group of autoinflammatory diseases (AID) characterized by self-limiting episodes of systemic inflammation without a confirmed molecular diagnosis, not fulfilling the criteria for periodic fever, aphthous stomatitis, pharyngitis and adenopathy (PFAPA) syndrome. In this review, we focused on the studies enrolling patients suspected of AID and genotyped them with next generation sequencing technologies in order to describe the clinical manifestations and treatment response of published cohorts of patients with SURF. We also propose a preliminary set of indications for the clinical suspicion of SURF that could help in everyday clinical practice.

Keywords: FMF; NGS; SURF; anakinra; autoinflammatory diseases; colchicine.

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Conflict of interest statement

The Authors declared no conflict of interest for this study.

Figures

Figure 1
Figure 1
Original English studies found in the PubMed database (https://pubmed.ncbi.nlm.nih.gov; accessed on 2 February 2020) with the queries: “periodic/recurrent fever/s” AND “NGS/Sanger”; “undefined/undifferentiated” AND “autoinflammatory”; “NGS/Sanger” AND “autoinflammatory”. AID, autoinflammatory diseases.
Figure 2
Figure 2
Trend line of studies in Table 1.
Figure 3
Figure 3
Correlation between the numbers of enrolled patients and analyzed genes of studies in Table 1 except the two using whole exome sequencing.
Figure 4
Figure 4
Predictive software of studies in Table 1. SIFT, Sorting Intolerant From Tolerant; PP2, Polymorphism Phenotyping version 2; MT, Mutation Taster; CADD, Combined Annotation Dependent Depletion software; HSF, human splicing finder; NNSplice, Splice Site Prediction by Neural Network; GERP, Genomic Evolutionary Rate Profiling; MetaSVM, Meta-analytic Support Vector Machine; PROVEAN, Protein Variation Effect Analyzer; SSF, Splice Site Finder; REVEL, Rare Exome Variant Ensemble Learner; UMD, Universal Mutation Database; MES, Manufacturing Execution System; GVGD, Grantham Variation and Grantham Deviation.
Figure 5
Figure 5
Classification tools of studies in Table 1. HGMD, Human Gene Mutation Database; EMGQN, European Molecular Genetics Quality Network; ACGS, Association for Clinical Genetics Society.
Figure 6
Figure 6
Clinical manifestations of SURF patients reported by at least two studies of Table 2. SURF, syndrome of undifferentiated recurrent fever.
Figure 7
Figure 7
Treatment efficacy in SURF patients. SURF, syndrome of undifferentiated recurrent fever; NSAIDs, non-steroidal anti-inflammatory drugs; DMARDs, disease-modifying anti-rheumatic drug.
See this image and copyright information in PMC

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