scholarly journals Effect of rituximab or tumour necrosis factor inhibitors on lung infection and survival in rheumatoid arthritis-associated bronchiectasis

Rheumatology ◽  
2020 ◽  
Vol 59 (10) ◽  
pp. 2838-2846 ◽  
Author(s):  
Md Yuzaiful Md Yusof ◽  
Kundan Iqbal ◽  
Michael Darby ◽  
Giovanni Lettieri ◽  
Edward M Vital ◽  
...  
Keyword(s):  
Therapeutic Choice ◽  
Antitrypsin Deficiency ◽  
Long Term Follow Up ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Observational Cohort ◽  
Alpha 1 Antitrypsin ◽  
Retrospective Observational Cohort Study ◽  
Necrosis Factor

Abstract Objective To evaluate rituximab (RTX) in patients with RA-associated bronchiectasis (RA-BR) and compare 5-year respiratory survival between those treated with RTX and TNF inhibitors (TNFi). Methods A retrospective observational cohort study of RA-BR in RTX or TNFi-treated RA patients from two UK centres over 10 years. BR was assessed using number of infective exacerbation/year. Respiratory survival was measured from therapy initiation to discontinuation either due to lung exacerbation or lung-related deaths. Results Of 800 RTX-treated RA patients, 68 had RA-BR (prevalence 8.5%). Post-RTX, new BR was diagnosed in 3/735 patients (incidence 0.4%). At 12 months post-Cycle 1 RTX, 21/68 (31%) patients had fewer exacerbations than the year pre-RTX, 36/68 (53%) remained stable and 11/68 (16%) had increased exacerbations. The rates of exacerbation improved after Cycle 2 and stabilized up to 5 cycles. Of patients who received ≥2 RTX cycles (n = 60), increased exacerbations occurred in 7/60 (12%) and were associated with low IgG, aspergillosis and concurrent alpha-1-antitrypsin deficiency. Overall, 8/68 (11.8%) patients discontinued RTX while 15/46 (32.6%) discontinued TNFi due to respiratory causes. The adjusted 5-year respiratory survival was better in RTX-treated compared with TNFi-treated RA-BR patients; HR 0.40 (95% CI 0.17, 0.96); P =0.041. Conclusion The majority of RTX-treated RA-BR patients had stable/improved pulmonary symptoms in this long-term follow-up. In isolated cases, worsening of exacerbation had definable causes. Rates of discontinuation due to adverse lung outcomes were better for RTX than a matched TNFi cohort. RTX is an acceptable therapeutic choice for RA-BR if a biologic is needed.

scholarly journals O12 Effect of rituximab on RA-associated bronchiectasis and comparison of 5-year respiratory survival with TNFi

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Md Yuzaiful ◽  
Md Yusof ◽  
Kundan Iqbal ◽  
Michael Darby ◽  
Giovanni Lettieri ◽  
...  
Keyword(s):  
Research Support ◽  
Severe Arthritis ◽  
Therapeutic Choice ◽  
Increased Risk ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Observational Cohort ◽  
Alpha 1 Antitrypsin ◽  
Respiratory Causes

Abstract Background Bronchiectasis (BR) is a significant pulmonary morbidity common in people with rheumatoid arthritis (RA). Patients with RA and bronchiectasis (RA-BR) often have severe arthritis but the use of biologics may be difficult in this group of patient due to concerns over safety. There is no data comparing the use of rituximab (RTX) and tumour necrosis factor inhibitors (TNFi) in RA-BR. The objectives of this study were to evaluate effect of rituximab (RTX) in patients with RA-BR and compare 5-year respiratory survival between those treated with RTX and TNFi. Methods A retrospective observational cohort study of RA-BR was conducted in RTX or TNFi-treated RA patients from two UK centres over 10 years. Identical data collection methodologies were used between centres. BR was assessed using number of infective exacerbations/year. Respiratory survival was defined as time from therapy initiation to discontinuation either due to lung exacerbation or lung-related deaths. Results Of 800 RTX-treated RA patients, 68 had RA-BR (prevalence=8.5%). Post-RTX, new BR was diagnosed in 3/735 patients (incidence=0.4%). At 12 months post-Cycle 1 RTX, 21/68 (31%) patients had fewer exacerbations than the year pre-RTX, 36/68 (53%) remained stable and 11/68 (16%) had increased exacerbations. Pseudomonas colonisation at RTX baseline was associated with increased risk of this initial exacerbation [OR 7.23 (95% CI 1.28-40.80)] while older age reduced risk [OR 0.44 (95% CI 0.21-0.90) per 10 years of age]. The rates of exacerbation improved after Cycle 2 RTX and were stable up to 5 cycles. Of patients who received ≥2 RTX cycles (n = 60), increased exacerbations occurred in 7/60 (12%) and were associated with low IgG, aspergillosis and concurrent alpha-1-antitrypsin deficiency. Respiratory survival was compared between RA-BR patients treated with RTX (N = 68) or TNFi (N = 46). Most characteristics were matched between these two groups. However, median (IQR) number of infective exacerbations/year in the previous 12 months pre-bDMARDs was higher in those treated with RTX than TNFi; 3.0 (1-4) and 0 (0-2) respectively. Overall, 8/68 (11.8%) patients discontinued RTX while 15/46 (32.6%) discontinued TNFi due to respiratory causes. Moreover, the 5-year respiratory survival was better in RTX-treated compared to TNFi-treated RA-BR patients; HR 0.40 (95% CI 0.17-0.96); p = 0.041, adjusted for age, gender and centre effect. Distribution of TNFi in those who discontinued therapy due to respiratory causes was etanercept=6, infliximab=6, adalimumab=2 and certolizumab=1. Conclusion The majority RA-BR patients had stable or improved pulmonary symptoms during RTX therapy over a prolonged follow-up period. In isolated cases, worsening of exacerbation after RTX therapy had definable causes. Despite a higher rate of exacerbations pre-biologic, rates of discontinuation or deaths due to respiratory causes were better for RTX than a matched TNFi cohort. RTX appears to be an acceptable therapeutic choice for RA-BR if a biologic is needed. Disclosures M. Md Yusof: None. K. Iqbal: None. M. Darby: None. G. Lettieri: None. E. Vital: Honoraria; Roche. Grants/research support; Roche. P. Beirne: None. S. Dass: Honoraria; Roche. P. Emery: Consultancies; Pfizer, MSD, Abbott. Grants/research support; Roche, Pfizer, MSD, Abbott. C. Kelly: Consultancies; Boehringer-Ingelheim. Honoraria; Boehringer-Ingelheim.

Long-term follow-up of a cohort of children with alpha-1-antitrypsin deficiency

1990 ◽  
Vol 116 (2) ◽  
pp. 248-251 ◽  
Author(s):  
Michael Wall ◽  
Esther Moe ◽  
Jay Eisenberg ◽  
Michael Powers ◽  
Neil Buits ◽  
...  
Keyword(s):  
Antitrypsin Deficiency ◽  
Long Term Follow Up ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

scholarly journals Protocol for the EARCO Registry: a pan-European observational study in patients with α1-antitrypsin deficiency

2020 ◽  
Vol 6 (1) ◽  
pp. 00181-2019 ◽  
Author(s):  
Timm Greulich ◽  
Alan Altraja ◽  
Miriam Barrecheguren ◽  
Robert Bals ◽  
Jan Chlumsky ◽  
...  
Keyword(s):  
Natural History ◽  
Research Collaboration ◽  
Medical Decision ◽  
Case Report Form ◽  
Increased Risk ◽  
Antitrypsin Deficiency ◽  
Study Team ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

Rationale and objectivesAlpha-1 antitrypsin deficiency (AATD) is a genetic condition that leads to an increased risk of emphysema and liver disease. Despite extensive investigation, there remain unanswered questions concerning the natural history, pathophysiology, genetics and the prognosis of the lung disease in association with AATD. The European Alpha-1 Clinical Research Collaboration (EARCO) is designed to bring together researchers from European countries and to create a standardised database for the follow-up of patients with AATD.Study design and populationThe EARCO Registry is a non-interventional, multicentre, pan-European, longitudinal observational cohort study enrolling patients with AATD. Data will be collected prospectively without interference/modification of patient's management by the study team. The major inclusion criterion is diagnosed severe AATD, defined by an AAT serum level <11 µM (50 mg·dL−1) and/or a proteinase inhibitor genotype ZZ, SZ or compound heterozygotes or homozygotes of other rare deficient variants. Assessments at baseline and during the yearly follow-up visits include lung function testing (spirometry, body plethysmography and diffusing capacity of the lung), exercise capacity, blood tests and questionnaires (symptoms, quality of life and physical activity). To ensure correct data collection, there will be designated investigator staff to document the data in the case report form. All data will be reviewed by the EARCO database manager.SummaryThe EARCO Registry aims to understand the natural history and prognosis of AATD better with the goal to create and validate prognostic tools to support medical decision-making.

scholarly journals Long-term follow-up of secondary amyloidosis patients treated with tumor necrosis factor inhibitor therapy

Medicine ◽  
2017 ◽  
Vol 96 (34) ◽  
pp. e7859 ◽  
Author(s):  
Sinem Nihal Esatoglu ◽  
Gulen Hatemi ◽  
Serdal Ugurlu ◽  
Aycan Gokturk ◽  
Koray Tascilar ◽  
...  
Keyword(s):  
Tumor Necrosis Factor ◽  
Tumor Necrosis ◽  
Tumor Necrosis Factor Inhibitor ◽  
Inhibitor Therapy ◽  
Secondary Amyloidosis ◽  
Factor Inhibitor ◽  
Long Term Follow Up ◽  
Necrosis Factor

scholarly journals Long-term evolution of lung function in individuals with alpha-1 antitrypsin deficiency from the Spanish registry (REDAAT)

2018 ◽  
Vol Volume 13 ◽  
pp. 1001-1007 ◽  
Author(s):  
Cristina Esquinas ◽  
Sonia Serreri ◽  
Miriam Barrecheguren ◽  
Esther Rodríguez ◽  
Alexa Nuñez ◽  
...  
Keyword(s):  
Lung Function ◽  
Long Term Evolution ◽  
Term Evolution ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin
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