scholarly journals Long-term effectiveness and safety of secukinumab for treatment of refractory mucosal and articular Behçet’s phenotype: a multicentre study

2020 ◽  
Vol 79 (8) ◽  
pp. 1098-1104 ◽  
Author(s):  
Filippo Fagni ◽  
Alessandra Bettiol ◽  
Rosaria Talarico ◽  
Giuseppe Lopalco ◽  
Elena Silvestri ◽  
...  
Keyword(s):  
Complete Response ◽  
Term Treatment ◽  
Behçet’S Syndrome ◽  
Biologic Treatment ◽  
Behçet's Syndrome ◽  
Long Term Treatment ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

ObjectiveTo evaluate the effectiveness and safety of secukinumab in patients with a mucosal and articular Behçet’s phenotype resistant to conventional and biologic treatment.MethodsA multicentre retrospective study was performed on 15 patients with a mucosal and articular phenotype of Behçet’s syndrome fulfilling the International Criteria for Behçet’s Disease and refractory to treatment with colchicine, disease-modifying antirheumatic drugs and at least one antitumour necrosis factor-α agent. Minimum follow-up was set at 6 months. Six patients with a polyarticular involvement were treated with secukinumab 300 mg/month, while all other cases received secukinumab 150 mg/month. Dose increase from 150 to 300 mg per month and shortening of administration frequency were allowed for poor disease control. Response evaluation was based on the number of oral ulcers in the previous 28 days and Disease Activity Score-28 for articular manifestations.ResultsAt 3 months of follow-up, nine (66.7%) patients achieved a response (complete or partial), and this proportion further increased to 86.7% at 6 months, 76.9% at 12 months, 90.0% at 18 months and 100.0% after 24 months. Notably, all patients who started with secukinumab 300 mg/month achieved complete response by month 6. Seven (46.7%) patients could achieve a response only after switching to a higher dosage.ConclusionsOur study suggests that secukinumab at a dose of 150 and 300 mg per month is safe and effective for the long-term treatment of patients with Behçet’s syndrome with a mucosal and articular phenotype refractory to previous treatments. Notably, secukinumab 300 mg/month resulted in superior complete mucosal and articular responses with no serious or dose-related adverse effects.

Long term follow-up of Behçet’s syndrome patients treated with cyclophosphamide

Rheumatology ◽  
2019 ◽  
Vol 59 (9) ◽  
pp. 2264-2271 ◽  
Author(s):  
Mert Gurcan ◽  
Sinem Nihal Esatoglu ◽  
Vedat Hamuryudan ◽  
Didem Saygin ◽  
Serdal Ugurlu ◽  
...  
Keyword(s):  
Adverse Events ◽  
Standard Form ◽  
Treatment Modalities ◽  
Behçet’S Syndrome ◽  
Short Term ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Abstract Objectives CYC remains an important treatment option for Behçet’s syndrome (BS) patients with life-threatening manifestations. However, adverse events may occur with CYC and this has led to increased use of biologic agents in other vasculitides. We investigated short and long term adverse events associated with CYC use in BS patients. Methods We conducted a retrospective chart review of all BS patients treated with CYC between 1972 and 2006. Patients were called in and a standard form was used for collecting demographic characteristics, indication for CYC, its cumulative dose and short term adverse events, defined as those causing discontinuation of CYC, hospitalization and/or death, long term adverse events, including infertility and malignancy, and outcome. Results Of 5790 BS patients, 198 (3.4%) had used at least one dose of CYC. Main indications were vascular or neurological involvement. After a median follow-up of 17 years, 52 (26%) patients had died, 113 (57%) could be contacted, and 33 (17%) were lost to follow-up. Vascular involvement was the leading cause of death (n = 27). Seventeen (9%) patients experienced short term adverse events with haemorrhagic cystitis being the most common. After a median follow-up of 25 years (interquartile range: 15–26 years), 17 malignancies occurred in 15 (8%) patients. Infertility was experienced by 26 (30%) patients. Conclusion Long term adverse events such as malignancy and infertility were major problems in our BS patients treated with CYC. These results underline the need for safer treatment modalities that are at least as effective as CYC.

scholarly journals Apremilast in Refractory Behçet’s Syndrome: A Multicenter Observational Study

2021 ◽  
Vol 11 ◽  
Author(s):  
Matheus Vieira ◽  
Solène Buffier ◽  
Mathieu Vautier ◽  
Alexandre Le Joncour ◽  
Yvan Jamilloux ◽  
...  
Keyword(s):  
Adverse Events ◽  
Complete Response ◽  
Joint Disease ◽  
Behçet’S Syndrome ◽  
Multicenter Observational Study ◽  
Entire Cohort ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

ObjectiveMucocutaneous and joint disorders are the most common manifestations in Behçet’s syndrome (BS) and are frequently clustered in the so-called minor forms of BS. There remains a need for safe and effective treatment for joint lesions in BS. We report the long-term safety and effectiveness of apremilast in refractory joint and mucocutaneous manifestations of BS.MethodsFrench nationwide multicenter study including 50 BS patients with either active joint and/or mucocutaneous manifestations resistant to colchicine and/or DMARDs. Patients received apremilast 30 mg twice a day. Primary effectiveness endpoint was the proportion of patients with complete response (CR) of articular symptoms at month 6 (M6), defined as resolution of inflammatory arthralgia and arthritis, with joint count equal to zero.ResultsAt inclusion, the median tender and swollen joint count was of 4 [2-6] and 2 [1-2], respectively. The proportion of CR in joint disease at M6 was 65% (n = 15/23), and 17% (n = 4/23) were partial responders. CR of oral and genital ulcers, and pseudofolliculitis at M6 was 73% (n = 24/33), 94% (n = 16/17) and 71% (n = 10/14), respectively. The overall response at M6 was 74% for the entire cohort and 70% for the mucocutaneous-articular cluster (n = 27). The median Behçet’s syndrome activity score significantly decreased during study period [50 (40–60) vs. 20 (0–40); p <0.0001]. After a median follow-up of 11 [6-13] months, 27 (54%) patients were still on apremilast. Reasons for apremilast withdrawal included adverse events (n = 15, 30%) and treatment failure (n = 8, 16%). Thirty-three (66%) patients experienced adverse events, mostly diarrhea (n = 19, 38%), nausea (n = 17, 34%) and headache (n = 16, 32%).ConclusionApremilast seems effective in BS-related articular disease refractory to colchicine and DMARDs. Discontinuation rates were significantly higher than that reported in clinical trials.

AB0481 EFFICACY OF APREMILAST FOR THE TREATMENT OF GENITAL ULCERS ASSOCIATED WITH ACTIVE BEHÇET’S SYNDROME: A COMBINED ANALYSIS OF TWO RANDOMIZED CONTROLLED TRIALS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1538-1538
Author(s):  
G. Hatemi ◽  
A. Mahr ◽  
M. Takeno ◽  
D. Kim ◽  
M. Melikoglu ◽  
...  
Keyword(s):  
Phase Ii ◽  
Phase Ii Study ◽  
Complete Response ◽  
Pooled Analysis ◽  
Behçet’S Syndrome ◽  
Research Support ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
The Mean ◽  
Behcet's Syndrome

Background:Behçet’s syndrome is a chronic, multi-system inflammatory disorder characterized by painful, recurrent oral ulcers (OU) and genital ulcers (GU).1The GU associated with Behçet’s syndrome can contribute to difficulties with sexual activity, walking, and sitting2; may cause scarring1; and may impair quality of life.1,2Apremilast (APR), an oral phosphodiesterase 4 inhibitor, has demonstrated efficacy in the treatment of the OU associated with Behçet’s syndrome in the phase III, randomized RELIEF study (BCT-002).3Objectives:To describe the efficacy of APR for the treatment of GU associated with active Behçet’s syndrome in the RELIEF study and in a pooled data analysis of RELIEF and the phase II study.Methods:Adult patients (≥18 years of age) with active Behçet’s syndrome and ≥3 OU at randomization or ≥2 OU at screening and randomization, without active major organ involvement, were randomized (1:1) to APR 30 mg twice daily or placebo (PBO). In RELIEF, clinical improvement in GU was assessed by evaluating the time to the first GU recurrence after loss of complete response, the mean number of GU in patients without GU at baseline, and the proportion of patients who were GU-free (complete response) at Week 12 (regardless of baseline GU status). A pooled analysis of patients in RELIEF and a randomized, phase II study4were conducted to assess achievement of GU complete response in patients with GU at baseline. In patients with GU complete response before Week 12, the median time to the first GU recurrence after loss of complete response was based on Kaplan-Meier estimates. The mean number of GU was summarized descriptively using data as observed. Between-group differences in the proportion of patients who were GU-free at Week 12 were analyzed by Cochran-Mantel-Haenszel test using non-responder imputation to handle missing data. Statistical tests were 2 sided (α=0.05).Results:A total of 207 patients were randomized and received ≥1 dose of study medication (APR: n=104; PBO: n=103). In all, 17 patients in the APR group and 17 in the PBO group had GU at baseline, with mean GU counts of 2.9 (APR) and 2.6 (PBO). Among patients with GU at baseline in RELIEF, 12/17 (70.6% [APR]) and 7/17 (41.2% [PBO]) achieved GU complete response at Week 12 (P=0.110). The median time to first GU recurrence in these patients occurred earlier with PBO (6.1 weeks) vs. APR (not calculable). In the pooled analysis of RELIEF and the phase II study, a significantly greater proportion of patients with GU at baseline achieved GU complete response at Week 12 with APR vs. PBO (21/27 [77.8%] vs. 9/23 [39.1%];P=0.011) (Figure 1). The proportion of patients who were GU-free was significantly greater with APR (92/104 [88.5%]) vs. PBO (72/101 [71.3%]), regardless of baseline number of GU (P=0.002) (Figure 2).Conclusion:The number of patients with GU was low, but the totality of the data shows a favorable trend in the treatment effect of APR on GU. Greater proportions of APR-treated patients were GU-free at Week 12 vs. patients receiving PBO, and the time to the first GU recurrence occurred earlier with PBO vs. APR.References:[1]Kokturk A. Patholog Res Int. 2012;2012:690390. 2. Senusi A, et al. Orphanet J Rare Dis. 2015;10:117. 3. Hatemi G, et al. N Engl J Med. 2019;381:1918-1928. 4. Hatemi G, et al. N Engl J Med. 2015;372:1510-1518.Disclosure of Interests:Gulen Hatemi Grant/research support from: BMS, Celgene Corporation, Silk Road Therapeutics – grant/research support, Consultant of: Bayer, Eli Lilly – consultant, Speakers bureau: AbbVie, Mustafa Nevzat, Novartis, UCB – speaker, Alfred Mahr Consultant of: Celgene, Speakers bureau: Roche, Chugai, Mitsuhiro Takeno Speakers bureau: Esai, Tanabe-Mitsubishi – speaker; Celgene Corporation – advisory board, Doyoung Kim: None declared, Melike Melikoglu: None declared, Sue Cheng Employee of: Amgen Inc. – employment; Celgene Corporation – employment at the time of study conduct, Shannon McCue Employee of: Amgen Inc. – employment; Celgene Corporation – employment at the time of study conduct, Sven Richter Employee of: Amgen Inc. – employment; Celgene Corporation – employment at the time of study conduct, Michele Brunori Employee of: Amgen Inc. – employment; Celgene Corporation – employment at the time of study conduct, Maria Paris Employee of: Amgen Inc. – employment; Celgene Corporation – employment at the time of study conduct, Mindy Chen Employee of: Amgen Inc. – employment; Celgene Corporation – employment at the time of the conduct, Yusuf Yazici Consultant of: BMS, Celgene Corporation, Genentech, Sanofi – consultant, Consultant of: BMS, Celgene Corporation, Genentech, Sanofi – consultant

scholarly journals Tocilizumab in the treatment of severe and refractory parenchymal neuro-Behçet’s syndrome: case series and literature review

2020 ◽  
Vol 12 ◽  
pp. 1759720X2097190
Author(s):  
Jinjing Liu ◽  
Dong Yan ◽  
Zhimian Wang ◽  
Yunjiao Yang ◽  
Shangzhu Zhang ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Interleukin 6 ◽  
Case Series ◽  
Interquartile Range ◽  
Behçet’S Syndrome ◽  
Serious Adverse Events ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Objectives: This study aimed to investigate the efficacy and safety of tocilizumab (TCZ) in severe and refractory parenchymal neuro-Behçet’s syndrome (p-NBS). Methods: We retrospectively analyzed five patients with p-NBS treated with TCZ in our center between 2013 and 2020, and six cases from literature research with the index terms “neuro-Behçet’s syndrome” and “tocilizumab” on PubMed NCBI. Results: A total of 11 patients with p-NBS were enrolled (5 males, 6 females), with a mean age of 34.5 ± 8.0 years at the onset. All the patients had parenchymal neurological lesions, six patients (54.5%) suffered from multiple lesions, and nine patients (81.8%) were disabled. Before TCZ administration, all the patients had failed conventional therapy, eight patients (72.7%) received two or more immunosuppressants, and five patients showed insufficient response or intolerance to other biologics. TCZ was administrated at 8 mg/kg every 4 weeks, with background glucocorticoids (GCs) and immunosuppressants. After a median follow-up of 13 (interquartile range, 3.5–23.5) months, all the patients achieved both clinical and radiological improvements, and the Behçet’s Disease Current Activity Form score improved significantly (3 versus 0, median, p = 0.004), the Rankin score also decreased (4 versus 2, median, p = 0.005). Levels of interleukin-6 in the cerebrospinal fluid decreased significantly in five patients (533.4 ± 389.7 pg/ml versus 34.5 ± 27.1 pg/ml, p = 0.048), after a median of two (interquartile range, 1–4) times of TCZ infusions. Furthermore, the GC dosage ( per os) reduced from 69.2 ± 16.9 mg/d to 16.4 ± 16.2 mg/d ( p = 0.000), and immunosuppressants were tapered in number and dosage in seven (63.6%) and four (36.3%) patients, respectively. No serious adverse events or deaths were observed during follow-up. Conclusions: TCZ is well tolerated and effective in severe and refractory p-NBS, with a favorable GC- and immunosuppressant-sparing effect. Cerebrospinal fluid interleukin-6 might be used to monitor the effects of TCZ in p-NBS.

C 35 Seven years follow-up of neurological involvement in Behçet's syndrome

1993 ◽  
Vol 14 ◽  
pp. 41s ◽  
Author(s):  
G. Akman-Demir ◽  
B. Baykan-Kurt ◽  
P. Serdaroglu ◽  
H. Gürvit ◽  
H. Yazici ◽  
...  
Keyword(s):  
Neurological Involvement ◽  
Behçet’S Syndrome ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Long-term salvage therapy with cyclosporin A in refractory idiopathic thrombocytopenic purpura

Blood ◽  
2002 ◽  
Vol 99 (4) ◽  
pp. 1482-1485 ◽  
Author(s):  
Giovanni Emilia ◽  
Monica Morselli ◽  
Mario Luppi ◽  
Giuseppe Longo ◽  
Roberto Marasca ◽  
...  
Keyword(s):  
Cyclosporin A ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Complete Response ◽  
Term Treatment ◽  
Thrombocytopenic Purpura ◽  
Long Term Treatment ◽  
Life Threatening ◽  
Difficult Challenge

Treatment of severe, chronic idiopathic thrombocytopenic purpura (ITP) refractory to most usual therapies is a difficult challenge. Little information exists on the clinical use of cyclosporin A (CyA) in the treatment of ITP. This report describes long-term treatment with CyA (median, 40 months) and follow-up (median, 36.8 months) in 12 adult patients with resistant ITP. CyA used in relatively low doses (2.5-3 mg/kg of body weight per day) led to a clinical improvement in 10 patients (83.3%). Five had a complete response (41.1%), 4 a complete response to maintenance therapy (33.3%), and one a partial response (8.3%). Two patients had no response. Most patients with a response (60%) had a long-term remission (mean, 28.6 months) after discontinuation of CyA. One patient had a relapse of ITP 4 years after CyA therapy was stopped. Side effects were moderate and transient, even in patients dependent on continued CyA treatment. CyA seems to represent reasonable salvage treatment in severe, potentially life-threatening, refractory ITP.

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