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1.
Francesca Ingegnoli Tommaso Schioppo Yannick Allanore Roberto Caporali Michele Colaci Oliver Distler Daniel E. Furst Nicolas Hunzelmann Florenzo Iannone Dinesh Khanna Marco Matucci-Cerinic 《Seminars in arthritis and rheumatism》2019,48(4):686-693
Background
Systemic sclerosis (SSc) is an autoimmune chronic disease characterized by vascular impairment, immune dysfunction and collagen deposition. Raynaud’s phenomenon (RP) and digital ulcers (DU) are prominent features of SSc. Intravenous (IV) iloprost (ILO), according to the recently updated EULAR recommendations, is indicated for RP after failure of oral therapy. Moreover, IV ILO could be useful in DU healing. IV ILO is currently available mainly on the European market approved for RP secondary to SSc with 3–5 days infusion cycle. Unfortunately, data published varies regarding regimen (dosage, duration and frequency). Up to now, ILO has been studied in small cohorts of patients and in few randomized controlled trials.Methods
A systematic review of studies on IV ILO in patients with SSc complicated by DU and RP was performed. Insufficient data were available to perform a meta-analysis according to the GRADE system. We performed a three-stage internet-based Delphi consensus exercise.Results
Three major indications were identified for IV ILO usage in SSc: RP non-responsive to oral therapy, DU healing, and DU prevention. IV ILO should be administered between 0.5 and 2.0 ng/kg/min according to patient tolerability with a frequency depending on the indication.Conclusions
Although these suggestions are supported by this expert group to be used in clinical setting, it will be necessary to formally validate the present suggestions in future clinical trials. 相似文献2.
Jinmi Kim Woonhyoung Lee Geun-Tae Kim Hyon-Suk Kim Soyoung Ock In-Soo Kim Seri Jeong 《Seminars in arthritis and rheumatism》2019,48(4):728-735
Objective
This study aimed to review and compare the analytical and clinical performance of automated indirect immunofluorescence (AIIF) and manual indirect immunofluorescence (MIIF) as anti-nuclear antibody screening assays for patients with systemic rheumatic diseases (SRDs), such as systemic lupus erythematosus (SLE) and systemic sclerosis (SSc).Methods
A systematic literature search was performed in the Medline, Embase, Cochrane, Web of Science, and Scopus databases for studies published before August 2017. A bivariate random effects model was used to calculate the summary diagnostic values.Results
Twenty-two studies involving 6913 positive and 1818 negative samples of MIIF, as well as 524 combined SRD, 132 SLE, and 104 SSc patients, and 520 controls were available for meta-analysis. The summary positive concordance (PC) of qualitative result between AIIF and MIIF was 93.7%, whereas PCs of total pattern (68.5%; homogeneous, 52.3%; speckled, 56.5%; nucleolar, 52.7%; centromere, 51.4%; nuclear dot, 11.7%) and titer (77.8%) exhibited significantly lower values. The summary clinical sensitivities of AIIF vs. MIIF were 84.7% vs 78.2% for combined SRDs, 95.5% vs. 93.9% for SLE, and 86.5% vs. 83.7% for SSc, respectively. Meanwhile, the summary specificities of AIIF vs. MIIF were 75.6% vs. 79.6% for combined SRDs, 74.2% vs. 83.3% for SLE, and 74.2% vs. 83.3% for SSc, respectively. Although the differences in sensitivity and specificity between AIIF and MIIF were not significant in most subgroups, the summary specificity of SLE and SSc showed statistically significant changes.Conclusions
Our systematic meta-analysis demonstrates that AIIF is comparable to MIIF in distinguishing between the positive and negative results, and screening SRDs based on clinical sensitivities and standardization. However, improvements in the pattern and titer recognition and clinical specificities are necessary. 相似文献3.
4.
Tetsuya Hanaka Takashi Kido Hiroshi Ishimoto Keishi Oda Shingo Noguchi Aya Nawata Shingo Nakayamada Noriho Sakamoto Yoshiya Tanaka Kazuhiro Yatera Hiroshi Mukae 《Respiratory investigation》2019,57(2):172-182
Background
There have been no reports on the relationship between lung radiological patterns and rheumatoid arthritis (RA) disease activity or RA treatment response in patients with RA-associated lung disease (RA-LD).Methods
Patients with RA-LD who underwent treatment for RA from April 2005 to March 2015 were retrospectively evaluated. RA-LD patients were divided into three groups based on high-resolution computed tomography (HRCT) patterns [usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), and bronchiolitis]. The disease activity score of 28 joints-erythrocyte sedimentation rate (DAS28-ESR) and the response of RA to treatment, as measured by the European League Against Rheumatism (EULAR) response criteria, were investigated.Results
A total of 77 patients (21 with UIP, 23 with NSIP, and 33 with bronchiolitis) were enrolled. Median scores (interquartile range) on the DAS28-ESR at baseline were 5.27 (4.76–5.74), 5.48 (4.24–6.34), and 5.04 (3.90–5.66) for UIP, NSIP, and bronchiolitis, respectively; there were no statistical differences between the three groups (p = 0.412). One year after baseline, 19 (90%), 14 (61%), and 19 (58%) of patients in the UIP, NSIP, and bronchiolitis groups, respectively, were considered good or moderate responders, as evaluated using the EULAR response criteria; there was a significant difference between these three groups (p = 0.014). Multiple logistic regression analysis revealed that the UIP pattern was significantly associated with good or moderate response to RA treatment 1 year after baseline (p = 0.012).Conclusions
These results suggest that NSIP and bronchiolitis HRCT patterns may be risk factors for resistance to RA therapy. 相似文献5.
Evgeni Aizenberg Robin M. ten Brinck Monique Reijnierse Annette H.M. van der Helm-van Mil Berend C. Stoel 《Seminars in arthritis and rheumatism》2019,48(4):579-586
Objective
MRI-detected inflammation is considered of diagnostic value for rheumatoid arthritis (RA), but its evaluation involves a time-consuming scoring of 61 joint-level features. It is not clear, however, which of these features are specific for RA and whether evaluating a subset of specific features is sufficient to differentiate RA patients. This study aimed to identify a subset of RA-specific features in a case–control setting and validate them in a longitudinal cohort of arthralgia patients.Methods
The difference in frequency of MRI-detected inflammation (bone marrow edema, synovitis, and tenosynovitis) between 199 RA patients and 193 controls was studied in 61 features across the wrist, metacarpophalangeal, and metatarsophalangeal joints. A subset of RA-specific features was obtained by applying a cutoff on the frequency difference while maximizing discriminative performance. For validation, this subset was used to predict arthritis development in 225 clinically suspect arthralgia (CSA) patients. Diagnostic performance was compared to a reference method that uses the complete set of 61 features normalized for inflammation levels in age-matched controls.Results
Subset of 30 features, mainly (teno)synovitis, was obtained from the case–control setting. Validation in CSA patients yielded an area of 0.69 (95% CI: 0.59–0.78) under the ROC curve and a positive predictive value (PPV) of 31%, compared to 0.68 (95% CI: 0.60–0.77) and 29% PPV of the reference method with 61 features.Conclusion
Subset of 30 MRI-detected inflammatory features, dominated by (teno)synovitis, offers a considerable reduction of scoring efforts without compromising accuracy for prediction of arthritis development in CSA patients. 相似文献6.
Xuan Luo Chuiwen Deng Yunyun Fei Wen Zhang Yongzhe Li Xuan Zhang Yan Zhao Xiaofeng Zeng Fengchun Zhang 《Seminars in arthritis and rheumatism》2019,48(4):626-631
Background
Psoriatic arthritis (PsA) is a chronic and seronegative inflammatory arthritis occurring in patients with psoriasis. The current knowledge about the risk of malignancy associated with psoriatic arthritis (PsA) patients undergoing therapy is controversial. We focused on the relationship between malignancy and therapy and undertook a meta-analysis to address this issue.Methods
A systematic literature search of the PubMed, EMBASE, and Web of Science databases was performed to identify relevant studies and trials. Statistical analysis was conducted using STATA 11.2 software.Results
Nine cohort studies were included, corresponding to a total of 43,115 PsA patients undergoing therapy. A significant positive association between therapy and increased risk for overall malignancy was found relative to the general population as the reference group (pooled RR, 1.29; 95% CI: 1.04–1.60). High heterogeneity was found (I2 = 71.37%). Subgroup analysis reported that PsA patients treated with conventional synthetic disease modifying antirheumatic drugs (csDMARDs) presented increased cancer risk (pooled RR, 1.75; 95% CI: 1.40–2.18) but patients treated with biological disease modifying antirheumatic drugs (bDMARDs) did not (pooled RR, 0.957; 95% CI: 0.80–1.14). Compared to controls, patients with PsA undergoing treatment specifically are at increased risk for non-melanoma skin cancers (pooled RR, 2.46; 95% CI: 1.84–3.28).Conclusions
This study allowed the estimation of cancer risk in PsA patients during therapy. Large-scale longitudinal studies will be essential to draw firm conclusions regarding PsA-associated risk for treatment-induced malignancy. 相似文献7.
Objective
Gout is the osteoarticular expression of hyperuricemia, resulting from excessive production and/or insufficient elimination of uric acid. Emerging case reports described the deposition of mono sodium urate in the spine as a rare manifestation of gout, we aimed in revealing the full picture of reported axial joint gout (AJG).Methods
We performed a systemic patient level review focused on characteristics of reported cases of axial joint involvement in gout.Results
A total of 127 studies (142 cases) were identified as axial joint gout. Most of the cases were reported by neurosurgeons and orthopedic surgeons (19.7% and 17.6%, respectively),low back and neck pain and weakness of limbs were presented in 113 cases, most of the cases (77.5%) were diagnosed via operation or aspiration. Although CT and MRI was the most popular imaging method, 8 cases underwent DECT avoided surgery had marked improvement.Conclusions
The incidence of AJG was underestimated and the IAJG exist independent of peripheral arthritis. AJG should be suspected when back pain and neurological involvement occurred in the risky populations. DECT would be a promising technique to initiate the earlier intervention complimentary to invasive procedures or operations. 相似文献8.
Cayetano Alegre 《Reumatología clinica》2019,15(3):170-172
Objective
To establish the percentage of Catalonian rheumatologists who attended to private patients, to understand the most common processes in private practice, to determine the dedication to patient care and the necessary activities to guarantee proper care, such as continuing education and office management, and to know what techniques were most widely used.Material and methods
A personalized survey of Catalonian rheumatologists whose e-mail addresses were made available to us. Fifty-two responses were analyzed.Results
The percentage of men (52%) and women (48%) was similar; 33% worked exclusively in private rheumatology (PR), most of them in hospitals or medical teams; 11% worked alone; 27% were in training. The disease most frequently treated was osteoarthritis; 51% were involved in studies or clinical trials in the field of RP.Conclusions
The concept of RP as a private business is changing and is progressively being transformed into working units with a structure similar to public hospitals with formal training.This report provides new data on the activities and characteristics of private rheumatologists. 相似文献9.
Yukihiro Minegishi Sumito Inoue Kento Sato Koya Abe Hiroaki Murano Kodai Furuyama Sujeong Yang Hiroyoshi Machida Hiroshi Nakano Masamichi Sato Takako Nemoto Chisa Sato Michiko Nishiwaki Tomomi Kimura Keiko Yamauchi Akira Igarashi Yoshikane Tokairin Yoko Shibata Masafumi Watanabe 《Respiratory investigation》2019,57(2):191-197
Background
Elderly patients who are hospitalized due to pneumonia experience deterioration of their activities of daily living (ADL) during this period; in some cases, this loss of ADL is not recovered at the end of antibiotic treatment. In this study, we examined whether erector spinae muscle cross-sectional area (ESMCSA) measured by computed tomography (CT) could predict a low level of ADL at the end of antibiotic treatment for pneumonia.Methods
Eighty patients (mean age 74.8 years) with pneumonia, who were admitted to Yamagata university hospital between 2015 and 2016, were analyzed retrospectively. In all cases, chest CT was performed on admission and ESMCSA was measured at the level of the 12th thoracic vertebra. Patient levels of ADL were also measured, both on admission and at the end of treatment, using the Barthel Index.Results
Patients with lower levels of ADL at the end of treatment were significantly older and tended to have a lower body mass index, poorer nutritional status, and more severe pneumonia than did patients who were self-reliant. Significantly smaller ESMCSAs were noted in patients who required assistance at the end of treatment than in those who were self-reliant. In multivariate logistic regression analysis, smaller ESMCSA was significantly associated with a lower level of ADL at the end of treatment, independent of age, sex, severity of pneumonia, nutritional status, or dehydration status.Conclusion
These results suggest that ESMCSA can predict ADL level after antibiotic treatment of pneumonia. 相似文献10.
Shin Tanaka Kentaroh Miyoshi Hisao Higo Takeshi Kurosaki Shinji Otani Seiichiro Sugimoto Masaomi Yamane Katsuyuki Kiura Shinichi Toyooka Takahiro Oto 《Respiratory investigation》2019,57(2):165-171
Background
Idiopathic pulmonary fibrosis (IPF) is a chronically progressive lung disease with exceptionally poor prognosis. While lung transplantation (LTx) is considered the last-resort therapeutic option, dismal waitlist mortality still hampers the salvage of patients with IPF. Pirfenidone, originally designed for IPF treatment, has increasingly been utilized. This study aimed to evaluate whether Pirfenidone could influence outcomes of patients with IPF on the Japanese LTx waitlist.Methods
This retrospective single-center cohort study included 25 consecutive patients with IPF who were registered as LTx candidates at our institution between July 1999 and August 2016. Patients with a history of pretransplant Pirfenidone therapy (Pirfenidone group) were compared with those with no history (non-Pirfenidone group).Results
In total, 6 (24%) patients received Pirfenidone as pretransplant therapy for 45.2 (range, 18.6–66.8) months. During the treatment period, the Pirfenidone group achieved a significant reduction in the decline rate of the forced vital capacity (-6.2% vs. -0.3%, p = 0.04) and a lower lung allocation score (31 vs. 41, p = 0.013) compared with the non-Pirfenidone group. The Pirfenidone group exhibited 100% waitlist survival three years after registration that was comparable to other indications, and 66% of the patients were still alive at the time of organ availability. No patient in the Pirfenidone group developed Pirfenidone-related surgical complications postoperatively.Conclusions
Patients with IPF successfully managed with long-term Pirfenidone therapy achieved favorable outcomes after LTx registration, comparable to other patients with LTx indications. The tolerability to antifibrotic therapy can be a predictor of waitlist survival. 相似文献11.
Mohsin Shah Jean H. Tayar Noha Abdel-Wahab Maria E. Suarez-Almazor 《Seminars in arthritis and rheumatism》2019,48(4):736-740
Objectives
Immune checkpoint inhibitors (ICIs) can successfully treat cancer, but their use can be hindered by serious immune-related adverse events. We report six patients receiving ICIs who presented with de novo myositis.Methods
We identified patients with myositis who were receiving ICIs between January 2004 and September 2016 at The University of Texas MD Anderson Cancer Center.Results
Six patients developed de novo myositis. The mean age was 64.3 years and five patients were male. Cancer types included melanoma, urothelial carcinoma, renal cell carcinoma, and prostate cancer. ICI regimens included single-agent ipilimumab (n = 1), pembrolizumab (n = 1), or atezolizumab (n = 1); nivolumab and ipilimumab (n = 3). The median time to development of de novo myositis from first infusion was 5.4 weeks (range: 2.1–17.1 weeks). All patients with myositis had elevated levels of creatinine kinase, ranging from 514 to 13,710 U/L. Two of them developed rhabdomyolysis, one with concurrent myocarditis. Five patients were treated with 1–2 mg/kg corticosteroids, with variable response rates; one patient received nonsteroidal anti-inflammatory drugs. Two patients with myositis died as a result of cancer progression.Conclusion
We found several occurrences of de novo myositis following ICI therapy. These preliminary data suggest that myositis can occur early after onset of ICI therapy with serious adverse outcomes. 相似文献12.
Pablo Olivera Silvio Danese Lieven Pouillon Stefanos Bonovas Laurent Peyrin-Biroulet 《Digestive and liver disease》2019,51(3):327-334
Background
Biologics against tumor necrosis factor (anti-TNF) have dramatically changed the management of moderate-to-severe ulcerative colitis (UC). In pivotal clinical trials, golimumab showed efficacy as induction and maintenance therapy in anti-TNF naïve UC patients. However, confirmatory data on effectiveness in the real world setting are needed.Aim
to summarize recent evidence on the effectiveness of golimumab in observational real-world studies.Methods
A literature search was conducted using Medline, Embase, and congresses databases for English language articles or abstracts on the effectiveness of golimumab published between January 1, 2014 and May 15, 2018. Pooled short-term (6–14 weeks) and mid- and long-term (24–54 weeks) clinical response and remission rates were calculated.Results
24 abstracts were included; of those 8 were published full-text articles and 16 were abstracts from medical conferences. Overall, pooled short-term clinical response and remission rates were 59.3% (range 35–85.5%; 13 studies; 1429 patients) and 35.9% (range 14–51.7%; 9 studies; 666 patients), respectively. Pooled mid- and long-term clinical response and remission rates were 60.3% (range 37.1–89.5%; 4 studies; 356 patients) and 39.2% (range 12–84%; 8 studies; 822 patients), respectively.Conclusions
Results: of observational studies confirm that golimumab is an effective therapy for UC in clinical practice. 相似文献13.
Jinbo Hu Chuan Peng Jiayu Li Rufei Gao Aipin Zhang Linqiang Ma Linkun Zhang Yi Yang Qingfeng Cheng Yue Wang Ting Luo Zhihong Wang Hua Qing Shumin Yang Qifu Li 《Seminars in arthritis and rheumatism》2019,48(4):644-648
Objective
This study aims to evaluate whether serum Bisphenol A (BPA) is a risk factor for hyperuricemia.Methods
In this prospective study, a total of 482 participants without hyperuricemia were enrolled at baseline and followed up for 6 years. Clinical characteristics were recorded, and serum levels of uric acid and BPA were measured. Participants were stratified into tertiles according to low, median, and high baseline serum BPA levels. Regression models were used to analyze associations of serum BPA with the change in uric acid and the risk of developing hyperuricemia.Results
At baseline, serum concentrations of BPA was 0.51 (0.24–2.37) ng/mL. After 6 years of follow-up, the change in serum uric acid concentration from baseline to the 6-year mark was significantly higher in subjects with higher baseline BPA concentration (0.03 ± 0.19, 0.07 ± 0.21, and 0.11 ± 0.25 mg/dL for low, median, and high tertiles, respectively, P = 0.006). When adjusted for potential confounders, such as age, renal function, and history of diabetes and hypertension, multivariable logistic analyses showed that subjects in the median or high baseline BPA tertiles exhibited a twofold higher risk of 6-year hyperuricemia incidence compared to subjects in the low baseline BPA tertile [odds ratio (OR) = 2.28 (95% CI: 1.05–4.95) for the median tertile; 2.42 (1.07–5.48) for the high tertile, Pfor Trend = 0.043].Conclusion
In conclusion, serum BPA is an independent risk factor for hyperuricemia. 相似文献14.
Aiko Ogawa Seiichiro Sakao Nobuhiro Tanabe Hiromi Matsubara Koichiro Tatsumi 《Respiratory investigation》2019,57(2):183-190
Background
There are several medications available to treat pulmonary arterial hypertension (PAH): PAH-targeted drugs. However, in patients with pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis (PVOD/PCH), rare diseases that cause pulmonary hypertension, the effectiveness and safety of vasodilators, including PAH-targeted drugs, are unclear.Methods
We searched English-language publications listed in three electronic databases (PubMed, Cochrane Library, and the Japan Medical Abstracts Society). Reports with efficacy outcomes (survival, improvement in 6-minute walk distance, and pulmonary vascular resistance) and data on development of pulmonary edema after administration of vasodilators to patients with PVOD/PCH were selected (1966 to August 2015).Results
We identified 20 reports that met our criteria. No randomized controlled or prospective controlled studies were reported. The survival time ranged from 71 minutes to 4 years or more after initiation of vasodilators. Most of the reported cases showed an improvement in the 6-minute walk distance and pulmonary vascular resistance. Pulmonary edema was reported in 15 articles, some cases of which were lethal.Conclusions
The present study demonstrates the potential efficacy and difficulties in the use of vasodilators in patients with PVOD/PCH; however, drawing a firm conclusion was difficult because of the lack of randomized controlled trials. Further research is needed to ascertain if vasodilator use is beneficial and safe in patients with PVOD/PCH. 相似文献15.
Alessandro Loglio Mauro Viganò Glenda Grossi Sara Labanca Maria Goldaniga Alessandra Pompa Lucia Farina Mariagrazia Rumi Paolo Corradini Floriana Facchetti Giovanna Lunghi Luca Baldini Pietro Lampertico 《Digestive and liver disease》2019,51(3):419-424
Backgound
A significant proportion of hepatitis B surface antigen (HBsAg) negative/anti-hepatitis B core antigen (anti-HBc) positive patients with non-Hodgkin lymphoma (NHL) undergoing rituximab-based chemotherapy (R-CT) may suffer hepatitis B virus (HBV) reactivation.Aims
We wanted to assess efficacy and safety of lamivudine (LMV) prophylaxis to prevent this complication.Methods
Eighty-five consecutive HBsAg negative/anti-HBc positive NHL patients (71 years, 100% serum HBV DNA undetectable, 74% anti-HBs positive) received LMV coadministered with R-CT and for 18 months after the end of R-CT. Serum ALT, HBsAg, anti-HBs and HBV DNA were assessed every 4 months during and after end of LMV.Results
During 39 (2–108) months of study period, including 21 months of LMV and 27 additional months after LMV discontinuation, one patient (2%) had HBV reactivation, 31 months after stopping LMV and during administration of new immunosuppressive regimens, without LMV prophylaxis, owing to incomplete oncological response. A 50% decline of anti-HBs titers occurred in 22/63 (35%) patients, including 12 who became anti-HBs seronegative. Five (6%) patients had ALT increase during R-CT but none required R-CT discontinuation. Seventeen (20%) patients died, all for tumour progression.Conclusion
LMV prophylaxis is safe and effective in preventing HBV reactivation in HBsAg negative/anti-HBc positive NHL patients receiving R-CT. 相似文献16.
Anna Viola Daniela Pugliese Sara Renna Federica Furfaro Flavio Caprioli Renata D’Incà Fabrizio Bossa Stefano Mazza Giuseppe Costantino Massimo Claudio Fantini Gionata Fiorino Angela Alibrandi Ambrogio Orlando Alessandro Armuzzi Walter Fries 《Digestive and liver disease》2019,51(4):510-515
Background
Anti-TNF therapies infliximab (IFX), adalimumab (ADA), and golimumab (GOL) are approved for treating moderate to severe ulcerative colitis (UC). In UC, only the switch from IFX to ADA has been investigated, reaching no more than 10–43% remission rates at 12 months.Aim
Of the present study was to investigate disease outcome after a switch from subcutaneous (SC) agents to the intravenous (IV) agent (IFX).Methods
In this retrospective multicentre study, we analysed the charts of UC patients unresponsive/intolerant or with secondary loss of response (LOR) to ADA or GOL who were switched to IFX. We evaluated clinical response and remission together with adverse events at 3, 6, and 12 months follow-up.Results
Seventy-six patients were included; 38 patients started ADA and 38 started GOL for a mean therapy duration of 6?±?6 months. Indications for switch were adverse events in 3%, primary failure in 79%, and LOR in 18% of patients. Clinical remission was reached by 47%, 50%, and 77% of patients, respectively. Patients that switched for LOR did numerically, but not statistically, better than patients who switched for primary failure.Conclusions
Our data show a superior remission rate in SC to IV anti-TNF switch in UC compared to the IV to SC switch reported in literature. 相似文献17.
Savino Sciascia Massimo Radin Irene Cecchi Elena Rubini Mario Bazzan Dario Roccatello 《Seminars in arthritis and rheumatism》2019,48(4):741-744
Objectives
To investigate the long-term effect of B-cell depletion therapy with Rituximab (RTX) alone as rescue therapy in primary antiphospholipid syndrome (PAPS) patients with severe thrombocytopenia.Methods
We retrospectively retrieved data from patients who met the following inclusion criteria: (a) persistent antiphospholipid antibodies (aPL) positivity and fulfilled the Sydney criteria for PAPS (b) presented with severe thrombocytopenia (platelets <50,000/mm3) (c) were treated with RTX as a rescue therapy (d) had at least 1 year of follow-up after B-cells depletion therapy.Results
This retrospective study included 6 consecutive female PAPS patients [median age 49.5 (range 38–66)] who presented with severe thrombocytopenia (platelets <50,000/mm3, mean value 31,000 ± 9000/mm3). We observed a full response (defined as >150,000 platelets/mm3) after treatment with RTX in 5 out of 6 patients (83.3%). Among responders, after a median follow-up of more than 4 years, we observed a median time free from relapse of 43 months (range 12–97). One patient did not respond to the B-cell depletion therapy and was treated with a splenectomy 1 month after RTX therapy and platelets levels normalized after 3 months. No adverse events were reported, no patients developed significant infections. Importantly, the patients required no further maintenance therapy for the thrombocytopenia.Conclusion
In one of the longest-term observational (median 43 months) studies, sustained clinical remission of severe thrombocytopenia without immunosuppressive maintenance therapy was obtained by RTX alone in patients with PAPS and severe thrombocytopenia intolerant or refractory to conventional therapy. 相似文献18.
Elena Montecatine-Alonso María-Victoria Gil-Navarro Cecilia M. Fernández-Llamazares Aurora Fernández-Polo Pere Soler-Palacín Jesús Llorente-Gutiérrez María-Teresa Gómez-Travecedo Calvo María-Dolores Esquivel-Mora Icíar Pérez-Rodrigo José M. Cisneros Walter-Alfredo Goycochea-Valdivia Olaf Neth 《Enfermedades infecciosas y microbiología clínica》2019,37(5):301-306
Introduction
Antimicrobial defined daily dose (DDD), has limitations for antimicrobial consumption measurement in paediatrics. An alternative DDD design applicable for children is proposed.Methods
Children (<16 years-old) from 10 Spanish hospitals during a 12-months period were included. Weight for age (50th percentile) was calculated for the median age of the cohort using standardized World Health Organization tables. DDD (g) for each antimicrobial was calculated by multiplying the obtained weight times the recommended dose (mg/kg) of the antimicrobial for the most common infectious indication.Results
A total of 40,575 children were included. Median age was 4.17 (IQR: 1.36–8.98) and 4.81 (IQR: 1.42–9.60) years for boys and girls, respectively. Mean weight for this age was 17.08 kg. Standardized DDD for representative antimicrobials were calculated.Conclusions
A useful method for antimicrobial DDD measurement in paediatrics has been proposed and should be validated in future studies for its use in paediatric antimicrobial stewardship programmes. 相似文献19.
Pietro Leccese Yesim Ozguler Robin Christensen Sinem Nihal Esatoglu Dongsik Bang Bahram Bodaghi Aykut Ferhat Celik Farida Fortune Julien Gaudric Ahmet Gül Ina Kötter Alfred Mahr Robert J. Moots Jutta Richter David Saadoun Carlo Salvarani Francesco Scuderi Petros P. Sfikakis Gulen Hatemi 《Seminars in arthritis and rheumatism》2019,48(4):752-762
Objectives
The aim of this systematic review was to inform the update of European League Against Rheumatism (EULAR) Recommendations for the management of Behçet’s syndrome (BS), on the evidence for the treatment of skin, mucosa and joint involvement of BS.Methods
A systematic literature search, data extraction, statistical analyses and assessment of the quality of evidence were performed according to a pre-specified protocol using the PRISMA guidelines. Studies that assessed the efficacy of an intervention in comparison to an active comparator or placebo for oral ulcers, genital ulcers, papulopustular lesions, nodular lesions or arthritis were included. Where possible, risk ratios were calculated for binary outcomes and mean difference for continuous outcomes.Results
Among the 3927 references that were screened, 37 were included in the analyses. Twenty-seven of these assessed mucocutaneous and 17 assessed joint involvement. Twenty-one of these studies were randomised controlled trials (RCTs). RCTs with colchicine, azathioprine, interferon-alpha, thalidomide, etanercept and apremilast showed beneficial results with some differences according to lesion type and gender. These agents were generally well tolerated with few adverse events causing withdrawal from the study.Conclusions
RCTs comprised more than a half (21/37, 57%) of the sources included in the evidence synthesis related to skin, mucosa and joint involvement applicable for the EULAR Recommendations for the management of BS. Differences in the outcome measures that were used across the included studies often made it difficult to combine and compare the results. 相似文献20.
Angelique Rondags Suzanne Arends Freke R. Wink Barbara Horváth Anneke Spoorenberg 《Seminars in arthritis and rheumatism》2019,48(4):611-617