Independent risk factors for resolution of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome within 4 years after the disease onset

Yıldız, Mehmet; Haşlak, Fatih; Adroviç, Amra; Gücüyener, Neslihan; Ülkersoy, İpek; Köker, Oya; Şahin, Sezgin; Ünlü, Gülçin; Barut, Kenan; Kasapçopur, Özgür

doi:10.1007/s10067-020-05466-w

Cited by 9 publications

(5 citation statements)

References 30 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“… 16 In one of our group’s previous studies on the natural course of the PFAPA syndrome, our group showed that later age of disease onset, positive family history for PFAPA syndrome, and the absence of headache may be predictors for resolution of PFAPA syndrome within 4 years after the onset. 26 …”

Section: Discussionmentioning

confidence: 99%

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome: A Single-Center Experience

Yıldız

Haşlak

Adroviç

et al. 2021

Turk Arch Pediatrics

Self Cite

View full text Add to dashboard Cite

Objective: The purpose of this study is to share our experience about clinical findings, natural course, and treatment response rates of a large cohort of patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome. Materials and Methods: Medical records of patients who were diagnosed with PFAPA syndrome between January 2010 and May 2021 at Istanbul University-Cerrahpasa Cerrahpasa Medical Faculty pediatric rheumatology department were reviewed retrospectively. Results: A total of 607 patients (females: 277, males: 330) with PFAPA syndrome were included. The median duration of episodes was 3 (1-15; interquartile range (IQR) 3-5) days, and the median interval between episodes was 20 days (5-120; IQR 15-30). The median age at the last attack and median disease duration were 66 (24-168; IQR 48-84) months and 40 (4-132; IQR 27.5-60) months, respectively. Fever (100%) was the most common clinical finding, followed by pharyngitis/exudative tonsillitis in 594 (97.9%), aphthous stomatitis in 308 (50.7%), cervical lymphadenopathy in 278 (45.8%), abdominal pain in 249 (41%), and arthralgia in 228 (37.6%) of the patients. Among the clinical findings, there was no statistical difference according to gender, except for cervical lymphadenitis being higher in males ( P < .001). Of the patients who were given steroids during attacks, 94.6% were responsive. Colchicine was effective in 93 (63.7%) patients. The disease episodes ceased in 313 (95.4%) of patients who had tonsillectomy/adenoidectomy. Conclusions: Clinicians should be alert for additional symptoms such as abdominal pain, arthralgia, and headache apart from the cardinal signs. Although tonsillectomy is highly effective, its use is controversial. Colchicine may be a good alternative for prophylaxis.

show abstract

Section: Discussionmentioning

confidence: 99%

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome: A Single-Center Experience

Yıldız

Haşlak

Adroviç

et al. 2021

Turk Arch Pediatrics

Self Cite

View full text Add to dashboard Cite

show abstract

“…Treatment with leukotriene receptor antagonists empirically reduces febrile attacks in PFAPA, 30 supporting this idea. A recent study reported presence of headache as an independent risk factor for long‐term morbidity of PFAPA syndrome of more than 4 years 31 . Therefore, patients with headaches may be good candidates for tonsillectomy to avoid long‐term morbidity.…”

Section: Discussionmentioning

confidence: 99%

“…Complete resolution rate for each combination of headache status and age at onset of more than 4 years. 31 Therefore, patients with headaches may be good candidates for tonsillectomy to avoid long-term morbidity.…”

Section: Ta B L Ementioning

confidence: 99%

Can the effectiveness of tonsillectomy for PFAPA syndrome be predicted based on clinical factors

et al. 2023

View full text Add to dashboard Cite

Objectives: To evaluate the clinical factors associated with the outcome of tonsillectomy in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome, thereby clarifying who would most likely benefit from that surgery. Methods:This was a case-control study of 53 PFAPA patients who underwent tonsillectomy and were divided into a complete-resolution group and a postoperativefever group. Logistic regression analyses were performed using 17 clinical factors as variables to identify factors associated with the surgical outcome. Hierarchical cluster analysis was also performed to evaluate for relationships between phenotypes and surgical outcomes.Results: Thirty-nine (73.6%) patients had complete resolution after tonsillectomy. In simple logistic regression analysis, the surgical outcome showed significant positive trends with late-onset (odds ratio [OR] 7.1, P = 0.02) and presence of headache (OR 6.5, P = 0.01). In stepwise multiple logistic regression analysis adjusted for age at onset, presence of headache was significantly associated with complete resolution (OR 6.5, P = 0.01). The complete resolution rates for each combination of headache status and age at onset were as follows: presence of headache/age at onset ≥36 months, 100% (14/14); presence of headache/age at onset <36 months, 76.9% (10/13); absence of headache/age at onset ≥36 months, 75.0% (6/8); and absence of headache/age at onset <36 months, 43.8% (7/16). In hierarchical cluster analysis, complete resolution, age at onset, and headache were in the same cluster.Conclusions: PFAPA patients with headache and late onset responded well to tonsillectomy. The mechanisms underlying this association may warrant further investigation.

show abstract

“…A recently published report evaluating the predicting factors for resolution of PFAPA within 4 years of disease onset in a cohort of 466 Turkish PFAPA patients found that age at disease onset, positive family history of PFAPA, myalgia, and headache were all found to be related to early resolution of PFAPA syndrome ( 18 ). These findings support our hypothesis that those with PFAPA who have a positive family history of the disorder may comprise a separate group from those with PFAPA with no reported family history of the disorder and may more commonly report myalgia and headache during PFAPA flares.…”

Section: Discussionmentioning

confidence: 99%

Familial Periodic Fever, Aphthous Stomatitis, Pharyngitis and Adenitis Syndrome; Is It a Separate Disease?

et al. 2022

View full text Add to dashboard Cite

IntroductionPeriodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA) is the most common periodic fever syndrome in the pediatric population, yet its pathogenesis is unknown. PFAPA was believed to be sporadic but family clustering has been widely observed.ObjectiveTo identify demographic and clinical differences between patients with PFAPA and a positive family history (FH+) as compared to those with no family history (FH−).MethodsIn a database comprising demographic and clinical data of 273 pediatric PFAPA patients treated at two tertiary centers in Israel, 31 (14.3%) had FH+. Data from patients with FH+ were compared to data from those with FH−. Furthermore, family members (FMs) of those with FH+ were contacted via telephone for more demographic and clinical details.ResultsThe FH+ group as compared to the FH− group had more myalgia (56 vs. 19%, respectively, p = 0.001), headaches (32 vs. 2%, respectively, p = 0.016), and a higher carrier frequency of M694V mutation (54% vs. 25%, respectively, p = 0.05). Colchicine was seen to be a more beneficial treatment for the FH+ group as compared to the FH− group; however, with no statistical significance (p = 0.096). FMs displayed almost identical characteristics to patients in the FH+ group except for greater arthralgia during flares (64 vs. 23%, respectively, p = 0.008), and compared to the FH− group they had more oral aphthae (68 vs. 43%, respectively, p = 0.002), myalgia/arthralgia (64 vs. 19%/16%, respectively, p < 0.0001), and higher rates of FH of Familial Mediterranean fever (FMF) (45 vs.15%, respectively, p = 0.003).ConclusionsOur findings suggest that patients with a FH+ likely experience a different subset of disease with higher frequency of family history of FMF, arthralgia, myalgia, and might have a better response to colchicine compared to FH−. Colchicine prophylaxis for PFAPA should be considered in FH+.

show abstract

Independent risk factors for resolution of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome within 4 years after the disease onset

Cited by 9 publications

References 30 publications

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome: A Single-Center Experience

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome: A Single-Center Experience

Can the effectiveness of tonsillectomy for PFAPA syndrome be predicted based on clinical factors

Familial Periodic Fever, Aphthous Stomatitis, Pharyngitis and Adenitis Syndrome; Is It a Separate Disease?

Contact Info

Product

Resources

About