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.
Initial case series of small number of patients at the beginning of the pandemic reported a rather guarded prognosis for Behçet's syndrome (BS) patients infected with SARS-CoV-2. In this prospective study, we describe the incidence, clinical characteristics, disease course, management, and outcome in a large cohort of BS patients with laboratory-confirmed infection of SARS-CoV-2. We defined a cohort of 1047 registered BS patients who were aged between 16 and 60 years and seen routinely before the pandemic at the multidisciplinary outpatient clinic. We followed prospectively this cohort from beginning of April 2020 until the end of April 2021. During 13 months of follow-up, of the 1047 (599 M/448 F) patients, 592 (56.5%) were tested for SARS-CoV-2 PCR at least once and 215 (20.5%; 95% CI 0.18-0.23) were tested positive. We observed 2 peaks which took place in December 2020 and April 2021. Of the 215 PCR positive patients, complete information was available in 214. Of these 214, 14 (6.5%) were asymptomatic for COVID-19. In the remaining, the most common symptoms were anosmia, fatigue, fever, arthralgia, and headache. A total of 40 (18.7%) had lung involvement, 25 (11.7%) were hospitalized, 1 was admitted to the intensive care unit while none died. Favipiravir was the most prescribed drug (74.3%), followed by colchicine (40.2%), and hydroxychloroquine (20.1%) in the treatment of COVID-19. After COVID-19, 5 patients (2.3%) were given supplemental O 2 and 31 (14.5%) antiaggregant or anticoagulants. During COVID-19, of the 214 PCR positive patients, 116 (54.2%) decreased the dose of their immunosuppressives or stopped taking completely; 36 (16.8%) experienced a BS flare which was mostly oral ulcers (10.3%). None of the patients reported a thrombotic event. A total of 93 (43.5%) patients reported BS flares after a median 45 days of COVID-19 infection and this was found to be significantly associated with immunosuppressive drug discontinuation. Multiple regression analysis adjusted for age and gender indicated that smoking and using interferon-alpha decreased the likelihood of getting COVID-19. The incidence and severity of COVID-19 did not differ between those who were using colchicine or not. The cumulative incidence of COVID-19 in this prospectively followed cohort of BS patients was almost two folds of that estimated for the general population living in Istanbul, Turkey, however, the clinical outcome of COVID-19 was not severe and there was no mortality. The protective effect of smoking and interferon deserves further investigation. On the other hand, colchicine did not have any positive or negative effect against COVID-19. Significant number of patients flared after COVID-19, however, this was significantly associated with immunosuppressive discontinuation during the infection. Contrary to our previous observations, COVID-19 did not seem to exacerbate thrombotic events during or after the infection.
There are limited data about humoral response to vaccine in Behçet's syndrome (BS). We compared SARS-CoV-2 antibody response after two doses of inactivated (Sinovac/CoronaVac) or mRNA (Pfizer/BioNTech) vaccines in patients with BS and healthy controls (HCs). We studied 166 (92M/74F) patients with BS (mean age: 42.9 ± 9.6 years) and 165 (75M/90F) healthy controls (mean age: 42.4 ± 10.4 years), in a single-center cross-sectional design between April 2021 and October 2021. A total of 80 patients with BS and 89 HCs received two doses of CoronaVac, while 86 patients with BS and 76 HCs were vaccinated with BioNTech. All study subjects had a negative history for COVID-19. Serum samples were collected at least 21 days after the second dose of the vaccine. Anti-spike IgG antibody titers were measured quantitatively using a commercially available immunoassay method. We found that the great majority in both patient and HC groups had detectable antibodies after either CoronaVac (96.3% vs 100%) or BioNTech (98.8% vs 100%). Among those vaccinated with CoronaVac, BS patients had significantly lower median (IQR) titers compared to ) vs 102 (59-180), p < 0.001]. On the other hand, antibody titers did not differ among patients with BS and HCs who were vaccinated with BioNTech [1648BioNTech [ .5 (527.0-3693.8) vs 1516BioNTech [ .0 (836.3-2599), p = 0.512). Among different treatment regimen subgroups in both vaccine groups, those who were using anti-TNF-based treatment had the lowest antibody titers. However, the difference was statistically significant only among those vaccinated with CoronaVac. Among patients vaccinated with BioNTech, there was no statistically significant difference between different treatment regimen groups. Compared to inactivated COVID-19 vaccine, mRNA-based vaccine elicited higher antibody titers among BS patients. Only in the CoronaVac group, patients especially those using anti-TNF agents were found to have low titers compared to healthy subjects. BS patients vaccinated with BioNTech were found to have similar seroconversion rates and antibody levels compared to healthy controls. Further studies should assess whether the low antibody titers are associated with diminished protection against COVID-19 in both vaccine groups.
Background Compared to the general population, the risk of death is substantially higher in renal transplant recipients than in age and sex matched individuals in the general population. In the general population, coronary artery calcification (CAC) predicts all-cause and cardiovascular mortality. In this study, we aimed to analyze these relationships in renal transplant recipients. Methods We examined 178 renal transplant patients in this prospective observational cohort study. We measured coronary artery calcification with multidetector spiral computed tomography using Agatston score at multiple time points. Overall, 411 scans were performed in 178 patients over an average 12.8 years follow up. The clinical end point was a composite including all cause death and non-fatal cardiovascular events. Data analysis was performed by the joint model. Results During a follow-up of 12.8 ± 2.4 years, coronary calcification progressed over time (P < 0.001) and the clinical endpoint occurred in 54 patients. In the analysis by the joint model both the baseline CAC score and the CAC score progression were strongly associated with the incidence rate of the composite event (HR: 1.261, 95% CI: 1.119-1.420, p = 0.0001) Conclusion Coronary artery calcification at baseline and coronary calcification progression robustly predict the risk of death and cardiovascular events in renal transplant recipients. These findings support the hypothesis that the link between the calcifying arteriopathy of renal transplant patients and clinical end points in these patients is causal in nature.
BACKGROUND AND AIMS Sarcopenia is characterized by an involuntary loss of skeletal muscle mass, strength, and function and is usually associated with older age. However, sarcopenia may also be seen at younger ages in patients with chronic kidney disease. The European Working Group on Sarcopenia in Older People (EWGSOP) recently proposed a standardized definition of sarcopenia. We aimed to investigate the incidence of sarcopenia and associated factors in renal transplant recipients. METHOD We examined consecutive adult (age >18 years) renal transplant recipients under regular follow-up in our outpatient clinic during December 2021. We assessed the muscle strength with a handgrip test using a dynamometer and with a chair stand test. Using the Sergi formula, we used bioimpedance analysis to estimate the appendicular skeletal mass. Finally, we measured the gait speed to assess physical performance. Probable sarcopenia was defined as the presence of low muscle strength. Following that, sarcopenia was diagnosed with low muscle quantity in patients with probable sarcopenia according to the revised criteria by the EWGSOP. We retrieved the clinical and laboratory data from the patients’ medical records. RESULTS We recruited a total of 93 kidney transplant recipients (mean age: 59 ± 1.4, male gender 58.1%). About 15.0% of the patients were cadaveric transplants. Probable sarcopenia was found in 31 patients (33.3%), of which 14 (15.0%) were diagnosed with sarcopenia. Diabetes mellitus and lower albumin levels were the significant factors associated with the presence of probable sarcopenia (P = 0.01, P = 0.015, respectively; Table 1). On the other hand, sarcopenia was significantly associated with cadaveric transplantation (P = 0.02; Table 2). CONCLUSION We found that probable sarcopenia and sarcopenia were highly prevalent in our relatively young renal transplant recipients. We recommend active screening for the presence of sarcopenia in renal transplant recipients, especially in the cadaveric ones.
Background and Aims Fabry disease is a rare, systemic, genetic, and metabolic lysosomal storage disease. Phenotypic heterogeneity prevents accurate predictions for the disease progression. We aimed to evaluate the association of multiple plasma biomarkers with disease severity in Fabry disease. Method In a cross-sectional study, we examined 76 Fabry, 46 CKD patients and 41 healthy controls. We studied KIM-1, MCP-1, YKL-40, TNFR-1, TNFR-2, cystatin-C with enzyme linked immunoassay. Fabry patients on renal replacement therapy were excluded from the analysis. Results Demographic, clinical and laboratory data of the study participants are shown in Table 1. eGFR was calculated with creatinine and creatinine-cystatin C using CKD-EPI formula. While there was no difference between Fabry patients and healthy controls regarding eGFR(cr); eGFR(cr-cys) was significantly lower in Fabry patients. Biomarker levels in three groups were shown in Table 2. After performing age, sex and BMI adjusted analysis, MCP-1 was significantly lower in Fabry patients compared to CKD group (p:0.01). MCP-1 was significantly higher in Fabry patients with cardiac involvement than in those without cardiac involvement (p:0.039). KIM-1 was significantly higher in Fabry patients comparing to healthy controls (p:0.01). There was no significant difference regarding biomarker levels between Fabry patients with and without kidney involvement. YKL-40 was significantly lower in Fabry patients without kidney involvement compared to both control groups (p:0.025), probably reflecting the effect of ERT. Conclusion MCP-1, KIM-1, YKL-40 and Cystatin C seems to be useful markers for the management of Fabry patients. Each biomarker is associated with different aspect of the disease. MCP-1 might be a useful regarding Fabry disease activity especially in patients with cardiac involvement. KIM-1 might be an early sign in Fabry disease. Cystatin-C should be used for better management of patients since it shows renal involvement better than creatinine.
Background and Aims: Thirst, is the main driver for fluid consumption. Abundant water intake is advised for patients with autosomal dominant polycystic kidney disease (ADPKD). However, daily routines may limit water intake. There is no established tool to quantify the drivers of water intake in ADPKD patients. The aim of this study was to modify and validate a thirst distress scale (TDS) to identify the factors that influence water intake in ADPKD patients. Methods The TDS-heart failure questionnaire was first modified to TDS-PKD to adapt to patients with ADPKD to assess (a) the intensity of thirst, (b) the consequences if the quench for thirst is not satisfied enough and (c) the disturbing effects of thirst on quality of life. Then, the TDS-PKD questionnaire was translated to Turkish using the guidelines of the World Health Organization. An electronic survey with the SurveyMonkey platform was used to collect data. Thirst intensity was evaluated using a visual analog scale (VAS). Results The questionnaire was filled in by 186 ADPKD patients, of which 126 were on tolvaptan therapy. The TDS-PKD questionnaire showed good internal consistency, with a Cronbach's alpha value of 0.859. According to the exploratory factor analysis, a three-factor structure was obtained. Three factors explained 60.7% of the total variance. There was a positive and statistically significant correlation between the total TDS and VAS scores (r = 0.589, p < 0.001). The average TDS-PDK score was 39.0 ± 1.4. Conclusion The TDS-PKD questionnaire is a valid and reliable tool for evaluating the thirst distress in ADPKD patients.
BACKGROUND AND AIMS Abundant water intake is advised for patients with autosomal dominant polycystic kidney disease (ADPKD) Meijer and Casteleijn (Riding the waves: evidence for a beneficial effect of increased water intake in autosomal dominant polycystic kidney disease patients? Nephrol Dialysis Transpl. 2014;29(9):1615–1617). Thirst, which can be defined as a sensation of dryness in the mouth and throat associated with a desire for liquids, is the main driver for fluid consumption Fitzsimons (Thirst. Physiol Rev 1972;52(2):468–561). However, daily routines may limit water intake. There is no established tool to quantify the drivers of water intake in ADPKD patients. In this study, it was aimed to develop and validate a thirst distress scale (TDS) to quantify the factors of water intake in ADPKD patients. METHOD The TDS-heart failure questionnaire was first (i) translated to Turkish using the approach recommended by the guidelines of the World Health Organization and finally (ii) modified to adapt to patients with ADPKD with totally 12 questions assessing (a) what may affect water intake, (b) what limit water intake and (c) what disturbing effects thirst may have on quality of life. The guidelines of the World Health Organization were used to translate the questionnaire to Turkish. An electronic survey with SurveyMonkey platform was used to collect data on thirst with TDS-PKD and thirst intensity (visual analog scale, VAS). Internal consistency of the TDS-PKD was evaluated with Cronbach’s alpha. Exploratory factor analysis was conducted to reveal the validity and the factor structure of the questions in the questionnaire. RESULTS The questionnaire was filled in by 154 ADPKD patients, of which 116 were on tolvaptan therapy. The time taken to answer the questions was about 4 min. The TDS-PKD questionnaire showed good internal consistency with a Cronbach's alpha value of 0.867. According to the exploratory factor analysis, a three-factor structure was obtained, meaning that there are three constructs about thirst in the TDS-PKD. Three factors explained 62.57% of the total variance. Factor one consisted of questions 1–7, factor two 8–10 and factor three 11–12. All three factors were significantly associated with thirst intensity (r = 0.505, r = 0.244 and r = 197, respectively). The average score of TDS-PDK was 40.2 ± 10.5, and the average VAS score was 8.5 ± 0.7. CONCLUSION The TDS-PKD questionnaire is a valid and reliable tool for evaluating the thirst distress in ADPKD patients.
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