title: Effects of rituximab in connective tissue disorders related interstitial lung disease.
abstract: OBJECTIVE
  Interstitial lung disease (ILD) is a key prognostic factor in connective tissue disorders (CTDs). The aim of our study was to assess the changes in pulmonary functional tests (PFTs) in various CTDs, including anti-synthetase syndrome (SYN), systemic sclerosis (SSc) and mixed connective tissue disorder (MCTD), following the use of rituximab therapy.
  
  
  METHODS
  A multicentre retrospective analysis of patients with ILD secondary to SYN (n=15), MCTD (n=6) and SSc (n=23). PFTs were performed at baseline and at 1 and 2 years of follow-up. The primary outcome was the change in forced vital capacity (FVC) at 1 year.
  
  
  RESULTS
  In the SYN population, median FVC changed from 53.0% (42.0-90.0) at baseline to 51.4% (45.6-85.0) at 1 year and 63.0 (50-88) (p=0.6) at 2 years (p=0.14). In SSc, FVC changed from 81.0% (66.0-104.0) at baseline to 89.0% (65.0-113.0) at 1 year (p=0.1) and 74.5 (50-91) at 2 years (p=0.07). In the MCTD population, FVC changed from 64.5% (63.0-68.0) at baseline to 63.0% (59.0-71.0) at 1 year (p=0.6) and 61 (59-71) after 2 years (p=0.8). DLCO showed a trend for improvement in the SYN population (p=0.06 at 1 year and 0.2 at years) while changes remain non-significant in the SSc and MCTD patients. In SYN patients, the percentage of responders at 1 year for FVC (33.3%) was greater than in SSc (9.5%) (p=0.07) and MCTD (17%) (p=0.45). RTX showed a satisfactory safety profile.
  
  
  CONCLUSIONS
  A trend of improvement of PFTs was observed in SYN patients although not reaching significance, while SSc and MCTD patients were stabilised.
authors.affiliation: Dept. of Clinical and Experimental Medicine, AOUC, University of Florence, Italy; and Paris Descartes University, Rheumatology A Dept., APHP, Cochin Hospital, Paris, France.
authors.forename: Gemma
authors.initials: G
authors.lastname: Lepri
authors.affiliation: Paris Descartes University, Rheumatology A Department, APHP, Cochin Hospital, Paris, France.
authors.forename: Jerome
authors.initials: J
authors.lastname: Avouac
authors.affiliation: Spedali Civili di Brescia, Service of Rheumatology and Clinical Immunology, University of Brescia, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Airò
authors.affiliation: Hospital Clinico San Cecilio, Granada, Spain; on behalf of GEAS-SEMI (Grupo Enfermedades Autoinmune Sistémicas)-(Sociedad Española de Medicina Interna).
authors.forename: Francisco
authors.initials: F
authors.lastname: Anguita Santos
authors.affiliation: Department of Clinical and Experimental Medicine, AOUC, University of Florence, Italy.
authors.forename: Silvia
authors.initials: S
authors.lastname: Bellando-Randone
authors.affiliation: Department of Clinical and Experimental Medicine, AOUC, University of Florence, Italy.
authors.forename: Jelena
authors.initials: J
authors.lastname: Blagojevic
authors.affiliation: Hospital Virgen del Rocio, Department of Internal Medicine, Sevilla, Spain; on behalf of GEAS-SEMI (Grupo Enfermedades Autoinmune Sistémicas)-(Sociedad Española de Medicina Interna).
authors.forename: Francisco
authors.initials: F
authors.lastname: Garcia Hernàndez
authors.affiliation: Hospital Can Misses, Autoimmune Disease Unit, Internal Medicine, Ibiza, Spain; on behalf of GEAS-SEMI (Grupo Enfermedades Autoinmune Sistémicas)-(Sociedad Española de Medicina Interna).
authors.forename: Jose Antonio
authors.initials: JA
authors.lastname: Gonzalez Nieto
authors.affiliation: Department of Clinical and Experimental Medicine, AOUC, University of Florence, Italy.
authors.forename: Serena
authors.initials: S
authors.lastname: Guiducci
authors.affiliation: Department of Rheumatology, University Hospital Zurich, Switzerland.
authors.forename: Suzana
authors.initials: S
authors.lastname: Jordan
authors.affiliation: Royal Adelaide Hospital, University of Adelaide, Australia.
authors.forename: Vidya
authors.initials: V
authors.lastname: Limaye
authors.affiliation: Department of Rheumatology, University Hospital Zurich, Switzerland.
authors.forename: Britta
authors.initials: B
authors.lastname: Maurer
authors.affiliation: Vall D'Hebron General Hospital, Autonoma Univeristy of Barcelona, Internal Medicine, Barcelona, Spain; on behalf of GEAS-SEMI (Grupo Enfermedades Autoinmune Sistémicas)-(Sociedad Española de Medicina Interna).
authors.forename: Albert
authors.initials: A
authors.lastname: Selva-O'Callaghan
authors.affiliation: Sapienza University of Rome, Department of Internal Medicine and Medical Specialities, Rome, Italy.
authors.forename: Valeria
authors.initials: V
authors.lastname: Riccieri
authors.affiliation: Department of Rheumatology, University Hospital Zurich, Switzerland.
authors.forename: Oliver
authors.initials: O
authors.lastname: Distler
authors.affiliation: Department of Clinical and Experimental Medicine, AOUC, University of Florence, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Matucci-Cerinic
authors.affiliation: Paris Descartes University, Rheumatology A Department, APHP, Cochin Hospital, Paris, France.
authors.forename: Yannick
authors.initials: Y
authors.lastname: Allanore
mesh_terms: D000328:Adult; D000368:Aged; D003240:Connective Tissue Diseases / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D018450:Disease Progression; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D008168:Lung / Q000187:drug effects* / Q000503:physiopathology; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D020127:Recovery of Function; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D000069283:Rituximab / Q000009:adverse effects / Q000627:therapeutic use*; D013997:Time Factors; D016896:Treatment Outcome; D014797:Vital Capacity
chemical_list: D007166:Immunosuppressive Agents; D000069283:Rituximab