Inflammasome and immune disorders (11)
Autoimmune and Inflammatory Disorders Research (7)
Ocular Diseases and Behçet’s Syndrome (6)
Inflammation biomarkers and pathways (5)
Vasculitis and related conditions (4)
Yabanci Erten ES, Koru L, Kaya F, Kucuk E, Aydin Z , et al.
Pediatric research •
Data on prophylaxis in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) are limited for probiotics. We aimed to evaluate clinical responses of probiotic and colchicine. We included PFAPA patients receiving colchicine or probiotic (Lactobacillus plantarum HEAL9 (5×10 cfu/tablet), Lactobacillus paracasei 8700:2 (5 × 10 cfu/tablet) and a vitamin mixture (0.75 μg/tablet vitamin D3, 0.38 μg/tablet, vitamin B12, 30 μg/tablet folic acid)) for ≥3 months. Disease activity was assessed using Auto-Inflammatory Disease Activity Index (AIDAI). Monthly AIDAI scores from 3-month periods before and after prophylaxis were compared retrospectively. Early clinical response was defined as AIDAI score change from the month before to after prophylaxis. The time to first attack was compared. The study included 130 patients (colchicine: n = 97; probiotics: n = 33). Significant early clinical response occurred in both groups, with median AIDAI scores decreasing from the month before to after prophylaxis (colchicine: p < 0.001; probiotics: p < 0.001). Time to first attack was longer in the colchicine group than probiotic group (p = 0.048). Colchicine and probiotics showed similar short-term improvement in PFAPA activity. However, differences exist in time to first attack and adverse effects and should be discussed with families when selecting prophylaxis. This study provides one of the first evaluations of clinical responses to colchicine and probiotic prophylaxis in children with PFAPA syndrome using objective disease activity scoring. By demonstrating clinical improvements in both groups, the study contributes to the growing body of evidence supporting probiotic use. The identification of gastrointestinal symptoms as a factor affecting colchicine response may contribute to a more personalized treatment approach. These findings contribute valuable data to the limited literature on PFAPA prophylaxis and support shared decision-making in clinical practice.
Koru L, Erten ESY, Dursun HK, Dizman EN, Kaya F , et al.
European journal of pediatrics •
We aimed to evaluate the efficacy of prophylactic colchicine treatment in periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) using the Auto-Inflammatory Diseases Activity Index (AIDAI) score measured before and during treatment. We also aimed to determine the optimal treatment duration for observing clinical response and to identify factors predicting response to treatment. Children with PFAPA receiving colchicine were included in the study. We utilized AIDAI scoring system by evaluating the sore throat, tonsillitis, and oral aphthae under the general symptoms heading. This form of AIDAI was given to the families, and they were asked to document their symptoms on a daily basis. AIDAI scores were calculated for the month before colchicine initiation and for the first and third months after. Data were obtained retrospectively. The mean age of patients was 4.4 ± 2.13 years, and almost two third of them (50/76) were male. The difference in AIDAI scores between the month before colchicine treatment and the 1st month after the treatment was significant, while the difference between the 1st and 3rd month after the treatment was not (p = 0.002 vs. p = 0.463). Patient treatment responses were: 44 (57.9%) complete response, 15 (19.7%) partial response, and 17 (22.4%) unresponsive to colchicine. Based on this finding, we can conclude that in the case of partial response to colchicine treatment after the first month, dose escalation, review of treatment compliance and the need for prophylaxis should be reevaluated. • Colchicine prophylaxis is recommended in PFAPA patients; however, the optimal time point for response assessment remains unclear. • The AIDAI score is suggested for measuring disease activity in autoinflammatory conditions. • This is the first repeated-measure study evaluating colchicine response in PFAPA patients using the AIDAI score. • Clinical response predominantly emerged within the first month, suggesting this period may be critical for evaluation.
Aydin Z, Kaya F, Kucuk E, Koru L, Dizman EN , et al.
European journal of pediatrics •
Recurrent aphthous stomatitis (RAS) is a common condition in pediatric age. Since there are significant genetic commonalities among idiopathic cases, periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome (PFAPA), and Behçet's disease (BD), they were proposed to be considered in one clinical spectrum termed Behçet's Spectrum Disorders (BSDs). This retrospective study aimed to evaluate the efficacy of colchicine on oral ulcers in children with BSD related RAS (BSD-RAS). Secondary aim was to compare the disease groups in terms of clinical and laboratory benefits. Data at admission and one year-after the treatment initiation were compared. A total of 317 patients with RAS were evaluated, and 186 patients classified as BSD-RAS (Female: n = 105, 56%). The median age at symptom onset was 5 (0.5-16.5) years. Colchicine was started to 142 (76%) patients. We observed a significant reduction in the frequency and duration of oral ulcers, as well as in the visual analogue scale (VAS) scores reported by patients/families and physicians, and the erythrocyte sedimentation rate during symptom-free periods across all three groups. In patients with PFAPA, both difference between initial and final recorded values (Δ) patient/families and Δ physician VAS scores were significantly higher than in other groups (p < 0.001). Furthermore, both Δ duration (p = 0.005) and Δ frequency of oral aphthae (p = 0.012) were significantly higher in idiopathic cases than those with PFAPA. These findings demonstrate a substantial reduction in both clinical symptoms and laboratory markers following colchicine treatment, supporting its effectiveness as a therapeutic option for BSD-RAS. •Recurrent aphthous stomatitis, PFAPA, and Behçet's disease may manifest with similar oral ulcers in pediatric patients. •Colchicine is frequently used in this clinical spectrum known as Behçet's Spectrum Disorders, although there is a scarcity of pediatric data. •Colchicine treatment significantly improved clinical and laboratory outcomes across all groups. •Idiopathic group had greater reductions in oral ulcer frequency and duration while PFAPA associated cases showed highest improvement in visual analogue scale scores.