PMID- 32432411
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20210123
IS  - 2578-5745 (Electronic)
IS  - 2578-5745 (Linking)
VI  - 2
IP  - 6
DP  - 2020 Jun
TI  - Treatment With Mycophenolate and Cyclophosphamide Leads to Clinically Meaningful 
      Improvements in Patient-Reported Outcomes in Scleroderma Lung Disease: Results of 
      Scleroderma Lung Study II.
PG  - 362-370
LID - 10.1002/acr2.11125 [doi]
AB  - OBJECTIVE: Our objective was to determine if treatment with cyclophosphamide 
      (CYC) and mycophenolate mofetil (MMF) improves patient-reported outcomes (PROs) 
      among patients with systemic sclerosis-related interstitial lung disease 
      (SSc-ILD). METHODS: This study examined PROs in patients with SSc-ILD (N = 142) 
      who participated in the Scleroderma Lung Study II, a randomized controlled trial 
      comparing MMF for 2 years with oral CYC for 1 year followed by 1 year of a 
      placebo. Joint models were created to evaluate the course of PROs over 2 years. 
      The difference in PRO scores from baseline to 24 months was measured, and the 
      percentage of patients meeting the minimum clinically important difference (MCID) 
      was calculated. Correlations between PROs and SSc-ILD disease severity measures 
      were also examined. RESULTS: Treatment with CYC and MMF led to improvements in 
      several PROs with no between-treatment differences. Scores for the Transitional 
      Dyspnea Index (TDI) and St. George's Respiratory Questionnaire (SGRQ) improved 
      significantly over 2 years, and 29%/24% and 28%/25% of participants in the 
      CYC/MMF groups met or exceeded the MCID estimates for TDI and SGRQ, respectively. 
      At baseline, the forced vital capacity (FVC) percentage predicted 
      (FVC%-predicted) did not correlate with the Baseline Dyspnea Index or SGRQ. 
      However, improvements in the FVC%-predicted were weakly associated with 
      improvements in dyspnea (assessed by the TDI) and SGRQ scores. CONCLUSION: 
      Treatment with CYC and MMF improved overall health-related quality of life in 
      patients with SSc-ILD. The relationship between PRO measures and the FVC was 
      relatively weak, suggesting that PROs provide complementary information about 
      treatment efficacy not captured by changes in the FVC alone in this patient 
      population.
CI  - (c) 2020 The Authors. ACR Open Rheumatology published by Wiley Periodicals, Inc. on 
      behalf of American College of Rheumatology.
FAU - Volkmann, Elizabeth R
AU  - Volkmann ER
AUID- ORCID: 0000-0003-3750-6569
AD  - University of California, Los Angeles.
FAU - Tashkin, Donald P
AU  - Tashkin DP
AD  - University of California, Los Angeles.
FAU - LeClair, Holly
AU  - LeClair H
AD  - University of California, Los Angeles.
FAU - Roth, Michael D
AU  - Roth MD
AD  - University of California, Los Angeles.
FAU - Kim, Grace
AU  - Kim G
AUID- ORCID: 0000-0003-1225-3489
AD  - University of California, Los Angeles.
FAU - Goldin, Jonathan
AU  - Goldin J
AD  - University of California, Los Angeles.
FAU - Clements, Philip J
AU  - Clements PJ
AD  - University of California, Los Angeles.
FAU - Furst, Daniel E
AU  - Furst DE
AD  - University of California, Los Angeles, University of Washington, Seattle, and 
      University of Florence, Florence, Italy.
FAU - Khanna, Dinesh
AU  - Khanna D
AUID- ORCID: 0000-0003-1412-4453
AD  - University of Michigan Medical School, Ann Arbor.
LA  - eng
GR  - Young Investigator Award/Scleroderma Foundation/
GR  - R01 AR-07047/AR/NIAMS NIH HHS/United States
GR  - K23 HL150237/HL/NHLBI NIH HHS/United States
GR  - Scientist Development Award/Rheumatology Research Foundation/
GR  - R01 HL089758/HL/NHLBI NIH HHS/United States
GR  - R01 HL089901/HL/NHLBI NIH HHS/United States
GR  - K24 AR063120/AR/NIAMS NIH HHS/United States
PT  - Journal Article
DEP - 20200520
PL  - United States
TA  - ACR Open Rheumatol
JT  - ACR open rheumatology
JID - 101740025
PMC - PMC7301868
EDAT- 2020/05/21 06:00
MHDA- 2020/05/21 06:01
PMCR- 2020/05/20
CRDT- 2020/05/21 06:00
PHST- 2019/12/07 00:00 [received]
PHST- 2020/02/14 00:00 [accepted]
PHST- 2020/05/21 06:00 [pubmed]
PHST- 2020/05/21 06:01 [medline]
PHST- 2020/05/21 06:00 [entrez]
PHST- 2020/05/20 00:00 [pmc-release]
AID - ACR211125 [pii]
AID - 10.1002/acr2.11125 [doi]
PST - ppublish
SO  - ACR Open Rheumatol. 2020 Jun;2(6):362-370. doi: 10.1002/acr2.11125. Epub 2020 May 
      20.