title: Rituximab in autoimmune connective tissue disease-associated interstitial lung disease.
abstract: CTD-associated interstitial lung disease (ILD) often fails to respond to conventional immunomodulatory agents. There is now considerable interest in the use of rituximab in systemic autoimmune CTD in patients refractory to standard treatments. The aim of this study was to review the experience of North Bristol NHS Trust managing patients with CTD-associated ILD with rituximab and explore possible associations with treatment response.
  
  
  
  We conducted a retrospective analysis of all patients who received rituximab under the Bristol CTD-ILD service, having failed to respond to other immunomodulatory treatments. Results were collated for pulmonary function and radiological outcomes before and after treatment.
  
  
  
  Twenty-four patients were treated with rituximab. Their physiological parameters had failed to improve despite other immunomodulatory agents, with a mean change in forced vital capacity (FVC) prior to therapy of - 3.3% (95% CI - 5.6, -1.1) and mean change in diffusing capacity of carbon monoxide of - 4.3% (95% CI - 7.7, -0.9). After rituximab, radiology remained stable or improved for 11 patients, while worsening was observed in 9 patients. The decline in FVC was halted following treatment, with a mean change of + 4.1% (95% CI 0.9, 7.2), while diffusing capacity of carbon monoxide was stable [mean change +2.1% (95% CI - 1.0, 5.2)]. Patients with myositis overlap or antisynthetase syndrome appeared to respond well to treatment, with four patients showing clinically significant improvement in FVC >10%.
  
  
  
  Rituximab is a therapeutic option in treatment-refractory CTD-associated ILD. Some disease subgroups may respond better than others, however, more work is needed to define its role in managing these patients.
authors.affiliation: Southmead Hospital, Academic Respiratory Unit, University of Bristol, Bristol.
authors.forename: Charles
authors.initials: C
authors.lastname: Sharp
authors.affiliation: Department of Geography, London School of Economics, Houghton Street, London.
authors.forename: Melanie
authors.initials: M
authors.lastname: McCabe
authors.affiliation: Bristol Interstitial Lung Disease Service.
authors.forename: Nick
authors.initials: N
authors.lastname: Dodds
authors.affiliation: Bristol Interstitial Lung Disease Service.
authors.forename: Anthony
authors.initials: A
authors.lastname: Edey
authors.affiliation: Bristol Interstitial Lung Disease Service.
authors.forename: Lloyd
authors.initials: L
authors.lastname: Mayers
authors.affiliation: Bristol Interstitial Lung Disease Service.
authors.forename: Huzaifa
authors.initials: H
authors.lastname: Adamali
authors.affiliation: Southmead Hospital, Academic Respiratory Unit, University of Bristol, Bristol.
authors.forename: Ann B
authors.initials: AB
authors.lastname: Millar
authors.affiliation: Bristol Interstitial Lung Disease Service Academic Rheumatology, North Bristol NHS Trust, Southmead Hospital, Bristol, UK harsha.gunawardena@nbt.nhs.uk.
authors.forename: Harsha
authors.initials: H
authors.lastname: Gunawardena
mesh_terms: D000328:Adult; D000368:Aged; D001327:Autoimmune Diseases / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology; D003240:Connective Tissue Diseases / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D008168:Lung / Q000000981:diagnostic imaging / Q000503:physiopathology; D017563:Lung Diseases, Interstitial / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000209:etiology; D008297:Male; D008875:Middle Aged; D011871:Radiology / Q000379:methods / Q000706:statistics & numerical data; D012189:Retrospective Studies; D000069283:Rituximab / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D007155:Immunologic Factors; D000069283:Rituximab
keywords: connective tissue disease; interstitial lung disease; rituximab; treatment