Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome – Is It Related to Ethnicity? An Israeli Multicenter Cohort Study

Amarilyo, Gil; Harel, Liora; Ahmad, Sabreen Abu; Rumi, Maryam Abu; Brik, Riva; Hezkelo, Nofar; Bar‐Yoseph, Ronen; Mei-Zahav, Meir; Ohana, Orly; Levinsky, Yoel; Chodick, Gabriel; Butbul-Aviel, Yonatan

doi:10.1016/j.jpeds.2020.08.033

Cited by 13 publications

(10 citation statements)

References 20 publications

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“…Similar results about the effect of underlying MEFV mutations on the response rate to colchicine treatment in patients with PFAPA syndrome were also found in different studies. 10 , 14 , 22 Supporting the prior findings, Amarilyo et al, 11 in their study evaluating the relationship between ethnicity and PFAPA syndrome, reported that the colchicine-responsive patients among patients of Mediterranean descent were higher than in patients with multi-ethnic descent. In contrast to earlier findings, there are studies in the literature suggesting similar rates of colchicine responders between patients carrying MEFV mutations and patients without MEFV mutations.…”

Section: Discussionsupporting

confidence: 60%

“…Consistent with the studies reporting the efficacy on decreasing the episodes of the colchicine treatment in 45.5-85% of PFAPA patients, 63.7% of our patients were responsive to colchicine. 10 , 11 , 14 , 15 , 18 , 22 In contrast to the previous studies, Berkun et al 16 stated that none of their PFAPA patients who tried colchicine were responsive. The remarkable difference between the rates of colchicine-responsive PFAPA patients reported in the studies and the fact that the high responsiveness rates were generally from communities around the Mediterranean made the effect of underlying MEFV mutations on the effectiveness of colchicine treatment a matter of interest.…”

Section: Discussionmentioning

confidence: 87%

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

Yıldız

Haşlak

Adroviç

et al. 2021

Turk Arch Pediatrics

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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.

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Section: Discussionsupporting

confidence: 60%

Section: Discussionmentioning

confidence: 87%

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

Yıldız

Haşlak

Adroviç

et al. 2021

Turk Arch Pediatrics

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“…These results suggest that even within a multi-ethnic population, particular ethnicities may enhance the risk for certain SAIDs. Along these lines, in a multi-ethnic French population, disease risk in 79 individuals with Behçet's disease was more strongly associated with ethnicity than the geographic environment (19), and Mediterranean descent was more common in individuals in Israel with PFAPA, a SAID of unknown aetiology (20). In summary, our results demonstrate that children with SAIDs in British Columbia have complex, multi-ethnic origins.…”

Section: Discussionmentioning

confidence: 53%

Children with systemic autoinflammatory diseases have multiple, mixed ethnicities that reflect regional ethnic diversity

Tucker

Niemietz

Mangat

et al. 2021

Clinical and Experimental Rheumatology

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Objective. To evaluate the ethnic diversity of children with a systemic autoinflammatory disease (SAID) in a multiethnic Canadian province. Methods. Self-reported ethnicity of 149 children and adolescents with a SAID in British Columbia, Canada, was analysed for ethnic representation among individual patients, across the cohort, within particular SAIDs, and compared to provincial census data on ethnic diversity. Results. Half of reported cases had a diagnosis of either PFAPA (23.5%) or an unclassifiable autoinflammatory syndrome (31.5%), with a monogenic SAID diagnosed in only 12.8% of cases. The majority of participants (73.1%) were mixed ethnicity with European and Asian heritage reported most frequently (57.0% and 23.0% of all responses, respectively). Ethnic diversity reflected regional diversity except for West Asian, Arabic, Jewish, and Eastern European heritage, which were over-represented in SAID patients, and Chinese descent, which was under-represented in our cohort compared to the general population of British Columbia. Conclusion. Results from this study show extensive multi-ethnic diversity in individual patients and across the various SAIDs inclusive of monogenic SAIDs that are frequently associated with particular ethnicities. Although not disproportionately represented, this is the first report of systemic autoinflammatory disease in Canadian children of Indigenous heritage.

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“…5 Department of Pediatrics B, Ruth Rappaport Children's Hospital, Rambam Medical Center, Haifa, Israel. 6 Department of Pediatrics A, Schneider Children's Medical Center, Petah Tikva, Israel. • fast, convenient online submission • thorough peer review by experienced researchers in your field • rapid publication on acceptance…”

Section: Discussionmentioning

confidence: 99%

“…Medications may reduce the frequency of attacks, especially glucocorticoids therapy where a single dose usually halts the attack within hours, but in about half of patients frequency of attacks increases [5]. Our group recently found that a Mediterranean ancestry may play a role in the development of the disease [6]; however, other factors that affect the development or severity of the disease remain unknown.…”

Section: Introductionmentioning

confidence: 99%

PFAPA flares observed during COVID outbreak: can emotional stress trigger PFAPA attacks? A multicenter cohort study

et al. 2022

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Objective It is common knowledge among clinicians who treat PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis) patients that emotional stress can trigger PFAPA attacks similarly to other autoinflammatory diseases. However, it has never been proved scientifically. Our aim was to examine whether emotional stress serves as a trigger for PFAPA attacks. Methods Patients aged 3-12 years, with active PFAPA, from two Israeli medical centers were enrolled to this study. Patient's parents were reached via phone calls in two occasions: a stressful period related to the COVID-19 pandemic restrictions and a less stressful period. In both times they were asked to report occurrence of PFAPA attacks in the preceding 2 weeks. The relative stress levels of the two periods were validated by an emotional distress scale questionnaire. The significance level was set at 0.05. Results Mean age was 7.28 ± 2.7 for the 99 paediatric patients enrolled in the study. Scores for the mean emotional distress questionnaire were statistically significant higher in the stressful period compared to the less stressful period (35.6 ± 8.1 vs. 32.1 ±7.7, respectively, P = 0.047). In the stressful period, 41 (38.7%) reported at least one attack during the preceding 2 weeks, compared to 24 (22.6%) in the less stressful period (p = 0.017). Conclusion PFAPA flares during COVID-19 outbreak are described. This study is the first to suggest that emotional stress is associated with PFAPA attacks.

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Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome – Is It Related to Ethnicity? An Israeli Multicenter Cohort Study

Cited by 13 publications

References 20 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

Children with systemic autoinflammatory diseases have multiple, mixed ethnicities that reflect regional ethnic diversity

PFAPA flares observed during COVID outbreak: can emotional stress trigger PFAPA attacks? A multicenter cohort study

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