scholarly journals S18 Rate Of Decline In Lung Density May Predict Long-term Outcome In Patients With Alpha 1 Antitrypsin Deficiency (aatd)

Thorax ◽  
2014 ◽  
Vol 69 (Suppl 2) ◽  
pp. A12-A12 ◽  
Author(s):  
C. Green ◽  
D. Parr ◽  
R. Stockley ◽  
A. Turner
2018 ◽  
Vol Volume 13 ◽  
pp. 1001-1007 ◽  
Author(s):  
Cristina Esquinas ◽  
Sonia Serreri ◽  
Miriam Barrecheguren ◽  
Esther Rodríguez ◽  
Alexa Nuñez ◽  
...  

Author(s):  
Olivier Guillaud ◽  
Emmanuel Jacquemin ◽  
Eduardo Couchonnal ◽  
Claire Vanlemmens ◽  
Claire Francoz ◽  
...  

Rheumatology ◽  
2020 ◽  
Vol 59 (10) ◽  
pp. 2838-2846 ◽  
Author(s):  
Md Yuzaiful Md Yusof ◽  
Kundan Iqbal ◽  
Michael Darby ◽  
Giovanni Lettieri ◽  
Edward M Vital ◽  
...  

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.


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