Familial Mediterranean fever and periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: shared features and main differences

Rheumatology International
Amra AdrovicOzgur Kasapcopur

Abstract

Autoinflammatory diseases are characterized by fever attacks of varying durations, associated with variety of symptoms including abdominal pain, lymphadenopathy, polyserositis, arthritis, etc. Despite the diversity of the clinical presentation, there are some common features that make the differential diagnosis of the autoinflammatory diseases challenging. Familial Mediterranean fever (FMF) is the most commonly seen autoinflammatory conditions, followed by syndrome associated with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA). In this review, we aim to evaluate disease characteristics that make a diagnosis of FMF and PFAPA challenging, especially in a regions endemic for FMF. The ethnicity of patient, the regularity of the disease attacks, and the involvement of the upper respiratory systems and symphonies could be helpful in differential diagnosis. Current data from the literature suggest the use of biological agents as an alternative for patients with FMF and PFAPA who are non-responder classic treatment options. More controlled studies are needed to evaluate the efficacy and safety of this strategy.

References

Sep 1, 1989·The Pediatric Infectious Disease Journal·G S MarshallA R Lawton
Jan 1, 1987·The Journal of Pediatrics·G S MarshallA R Lawton
Jul 7, 1999·The Journal of Pediatrics·K T ThomasK M Edwards
Apr 29, 2000·QJM : Monthly Journal of the Association of Physicians·D R BoothP N Hawkins
Jan 10, 2002·Annals of the Rheumatic Diseases·C KorkmazH Yazici
Nov 24, 2007·The Journal of Pediatrics·Fatoş YalçinkayaSeza Ozen
Oct 10, 2008·The Journal of Rheumatology·Z Birsin OzçakarMesiha Ekim
Feb 6, 2009·Rheumatology·Fatos YalçinkayaAysin Bakkaloglu
Apr 12, 2011·Proceedings of the National Academy of Sciences of the United States of America·Silvia StojanovDaniel L Kastner
Jul 30, 2011·Rheumatology International·Armen Yuri GasparyanGeorge D Kitas
Sep 26, 2012·The Journal of Allergy and Clinical Immunology·Laeticia KollyMichaël Hofer
Jul 13, 2013·The Open Rheumatology Journal·Shoichiro TaniuchiKazunari Kaneko
Nov 20, 2013·Nature Reviews. Rheumatology·Seza Ozen, Yelda Bilginer
Feb 27, 2014·Annals of the Rheumatic Diseases·Seza OzenUNKNOWN FMF Arthritis Vasculitis and Orphan disease Research in pediatric rheumatology (FAVOR) and Turkish FMF study group
Sep 13, 2014·Clinical Rheumatology·Z Birsin OzçakarFatoş Yalçınkaya
Feb 1, 2015·Annals of the Rheumatic Diseases·Silvia FedericiUNKNOWN Paediatric Rheumatology International Trials Organisation (PRINTO) and Eurofever Project
Mar 12, 2015·Current Medicinal Chemistry·Betul Sozeri, Ozgur Kasapcopur
Jan 30, 2016·Indian Journal of Pediatrics·Ruth J Pepper, Helen J Lachmann
Apr 14, 2016·Joint, Bone, Spine : Revue Du Rhumatisme·Perrine DusserIsabelle Koné-Paut
Feb 17, 2017·Pediatrics International : Official Journal of the Japan Pediatric Society·Muhammed GunesSara Sebnem Kilic
Jun 21, 2017·Current Rheumatology Reports·Erdal SagSeza Ozen
Aug 23, 2017·Rheumatology International·Kenan BarutOzgur Kasapcopur
Oct 17, 2017·The Journal of Pediatrics·Esra PehlivanOzgur Kasapcopur
Dec 6, 2017·Brazilian Journal of Otorhinolaryngology·Ozturk AktasAhmet Adnan Cirik
May 17, 2018·The New England Journal of Medicine·Fabrizio De BenedettiGuido Junge

❮ Previous
Next ❯

Citations

Feb 16, 2021·Frontiers in Cellular and Infection Microbiology·Mysore V TejesviMarjo Renko
Jul 25, 2019·Journal of Pediatric Hematology/oncology·Rabia Miray Kisla EkinciEmine Kocabas
Feb 23, 2021·Frontiers in Medicine·Mehmet YildizOzgur Kasapcopur
Dec 14, 2021·Frontiers in Pediatrics·Tatjana WelzelJasmin B Kuemmerle-Deschner

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.

Related Papers

Proceedings of the National Academy of Sciences of the United States of America
Kelly L BrownStefan Berg
Archives of Otolaryngology--head & Neck Surgery
Greg LicameliFatma Dedeoglu
The Journal of Pediatrics
K T ThomasKathryn M Edwards
© 2022 Meta ULC. All rights reserved