Most of the published data relate to classical forms of rheumatic diseases (RD) and information on rare inflammatory disorders such as Behçet’s syndrome (BS) and familial Mediterranean fever (FMF) is limited. We studied the frequency of side effects and disease flares after COVID-19 vaccination with either Pfizer/BioNTech or Sinovac/CoronaVac in 256 patients with BS, 247 with FMF, and 601 with RD. Telephone interviews were conducted using a questionnaire survey in a cross-sectional design in patients with BS, FMF, and RD followed by a single university hospital. Study participants were vaccinated either with CoronaVac (BS:109, FMF: 90, and RD: 343,) or BioNTech (BS: 147, FMF: 157 and RD: 258). The majority have received double dose (BS: 94.9%, FMF 92.3% and RD: 86.2%). BioNTech ensured a significantly better efficacy than CoronaVac against COVID-19 in all patient groups (BS: 1.4% vs 10.1%; FMF: 3.2% vs 12.2%, RD:2.7% vs 6.4%). Those with at least one adverse event (AE) were significantly more frequent among those vaccinated with BioNTech than those with CoronaVac (BS: 86.4% vs 45%; FMF: 83.4% vs 53.3%; and RD: 83.3% vs 45.5%). The majority of AEs were mild to moderate and transient and this was true for either vaccine. There were also AEs that required medical attention in all study groups following CoronaVac (BS: 5.5%, FMF: 3.3%, and RD:2.9%) or BioNTech (BS: 5.4%, FMF: 1.9%, and RD: 4.7%). The main causes for medical assistance were disease flare and cardiovascular events. Patients with BS (16.0%) and FMF (17.4%) were found to flare significantly more frequently when compared to those with RD (6.0%) ( p < 0.001). This was true for either vaccine. BS patients reported mainly skin-mucosa lesions; there were however, 11 (4.3%) who developed major organ attack such as uveitis, thrombosis or stroke. Flare in FMF patients were associated mainly with acute serositis with or without fever. Arthralgia/arthritis or inflammatory back pain were observed mainly in the RD group. Our study demonstrates that BS and FMF patients vaccinated with either CoronaVac or BioNTech demonstrated similar AE profile and frequency compared to RD patients. AEs that required physician consultation or hospitalization occurred in all study groups after either CoronaVac or BioNTech. Increased frequency of flares in BS and FMF compared to that seen in RD might reflect defects in innate immunity and deserves further investigation. Caution should be required when monitoring these patients after vaccination.

Recently, haploidentical transplantations have been performed with unmanipulated BM or PBSC. This approach is becoming more widely adopted with the use of PTCY. However, there is limited evidence about this approach in children. We present 15 children who received 16 haploidentical HSCT with unmanipulated BM or PBSC using PTCY for GVHD prophylaxis. Post-transplant CY(50 mg/kg IV) was given on the third and fifth day, and CsA or tacrolimus with MMF or MP was also used for GVHD prophylaxis. All patients engrafted at a median of 16 and 18 days for neutrophil and thrombocyte recovery, respectively. Grades II-III acute GVHD developed in seven patients, and mild chronic GVHD was found in two patients. Two patients died within the first 100 days due to sepsis (TRM 12.5%). Eleven patients are currently alive, with a median follow-up of 12 months (range 6-22 months). The 12-month OS and DFS were 75 ± 10.8% and 68.8 ± 11.6%, respectively. Our results with these high-risk patients are encouraging for haploidentical HSCT in pediatric patients. Future studies should continue to assess haploidentical HSCT, including comparison of other modalities, in a primary pediatric population.

No abstract

No abstract

Background:Familial Mediterranean Fever (FMF) can cause various muscle diseases. Because it is a chronic auto inflammatory disease, painful trigger points may be encountered in the examination due to a decrease in the pain threshold (1-3).Objectives:The aim of this study was to determine the prevalence of Fibromiyalgia in patients with FMF, at the same time to identify the relationship between fatigue and quality of life.Methods:Sixtyseven patients (38 female, 29 male) with FMF were enrolled in the study. They were diagnosed with FMF based on the Livneh diagnostic criteria (4). Fibromyalgia involvement of the patients was evaluated according to the Fibromyalgia Impact Questionnaire (FIQ). Patients with diagnose with other chronic disease were excluded. Fatigue Severity Scale (FSS) was used to evaluate fatigue. Quality of life was evaluated with Short Form-36 (SF-36).Results:Respectively, the mean age, disease duration and body mass index were 34.46±12.69 years, 12.66±7.86 years and 24.96±5.42 kg/m2. In addition, 65% of the patients had no rheumatic disease in their family history. The mean of scores of FIQ was 38.66±25.14, the mean of FSS was 38.07±17.56, the mean of SF-36-PCS was 45.55±10.54 and SF36-MCS was 30.93±17.39. Patients were categorized as mild (n=28), moderate (n=24) and severe (n=15) affected according to their FİQ score. The relationships of scores of FIQ, FSS and SF-36 were demonstrated Table 1.Conclusion:Fibromyalgia symptoms can be seen in FMF. According to our results, it has been shown that patients with moderate and severe symptoms have increased fatigue levels and decreased quality of life. In the light of these results, we can say that also the fibromyalgia symptom of patients with FMF should be considered in the treatment.References:[1]Sari, Ismail; Birlik, Merih; Kasifoglu, Timucin. Familial Mediterranean fever: an updated review. European journal of rheumatology, 2014, 1.1: 21.[2]Alayli G, Durmus D, Ozkaya O, Sen HE, Genc G, Kuru O. Frequency of juvenile fibromyalgia syndrome in children with familial Mediterranean fever: effects on depression and quality of life. Clin Exp Rheumatol 2011; 29: S127-32.[3]Langevitz P, Buskila D, Finkelstein R, Zaks N, Neuman L, Sukenik S, et al. Fibromyalgia in familial Mediterranean fever. J Rheumatol 1994; 21: 1335-7.[4]Bashardoust, Bahman. Familial Mediterranean fever; diagnosis, treatment, and complications. Journal of nephropharmacology, 2015, 4.1: 5.Table 1.The correlations of FIQ, FSS and SF-36 scores.FSSSF-36 PCSSF-36 MCSFIQ-mildmean±sd23.78±14.8853.34±7.0140.98±13.73r0.595**-0.014-0.551**p0.0010.9440.002FIQ-moderatemean±sd45.75±10.8341.09±8.8938.13±9.19r0.053-0.379-0.145p0.8060.0680.498FIQ-severemean±sd52.46±10.1138.13±9.1920.32±15.68r0.622*-0.548*-0.268p0.0130.0350.333-Pearson CorrelationDisclosure of Interests:None declared