PMID- 23450327 OWN - NLM STAT- MEDLINE DCOM- 20140109 LR - 20211021 IS - 1931-3543 (Electronic) IS - 0012-3692 (Print) IS - 0012-3692 (Linking) VI - 144 IP - 2 DP - 2013 Aug TI - Influence of interstitial lung disease on outcome in systemic sclerosis: a population-based historical cohort study. PG - 571-577 LID - S0012-3692(13)60534-6 [pii] LID - 10.1378/chest.12-2768 [doi] AB - BACKGROUND: Interstitial lung disease (ILD) is a frequent complication of systemic sclerosis (SSc) and a major cause of SSc-related deaths. This study aimed to determine the influence of ILD on SSc in a population-based historical cohort study. The hypothesis was that patients with SSc who develop ILD have increased morbidity and mortality when compared with patients with SSc without ILD. METHODS: Using the record linkage system of the Rochester Epidemiology Project in Olmsted County, Minnesota, this study identified the incidence of SSc between 1980 and 2010 and point prevalence on December 31, 2010 and determined the progression of organ involvement and its influence on outcome. RESULTS: During the 30-year interval, we identified 64 incident cases of SSc: 57 women and seven men, median age 49.1 years (interquartile range [IQR], 39.8-67.6 years). There were 43 prevalent cases. ILD occurred in 19 cases, usually after the diagnosis of SSc (median, 2 years; IQR, 0-10 years), with only three cases occurring 6 to 24 months beforehand. Pulmonary arterial hypertension (PAH) was diagnosed in 14 cases, heart failure in 27 cases, and chronic kidney disease (CKD) in 21 cases. Seventeen patients died during the study period, with a median survival time after diagnosis of 22.9 years. ILD, PAH, and CKD were associated with an increased risk of death. CONCLUSIONS: The incidence of ILD associated with SSc was relatively low in this population-based cohort. ILD appeared to be a contributing factor to mortality. Other factors, including age, PAH, and CKD, were also associated with poor outcome. FAU - Bauer, Philippe R AU - Bauer PR AD - Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN. Electronic address: bauer.philippe@mayo.edu. FAU - Schiavo, Dante N AU - Schiavo DN AD - Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN. FAU - Osborn, Thomas G AU - Osborn TG AD - Rheumatology, Mayo Clinic, Rochester, MN. FAU - Levin, David L AU - Levin DL AD - Radiology-Diagnostic, Mayo Clinic, Rochester, MN. FAU - St Sauver, Jennifer AU - St Sauver J AD - Health Sciences Research-Epidemiology, Mayo Clinic, Rochester, MN. FAU - Hanson, Andrew C AU - Hanson AC AD - Health Sciences Research-Biomedical Statistics and Informatics, Mayo Clinic, Rochester, MN. FAU - Schroeder, Darrell R AU - Schroeder DR AD - Health Sciences Research-Biomedical Statistics and Informatics, Mayo Clinic, Rochester, MN. FAU - Ryu, Jay H AU - Ryu JH AD - Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN. LA - eng GR - R01 AG034676/AG/NIA NIH HHS/United States GR - UL1 TR000135/TR/NCATS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - United States TA - Chest JT - Chest JID - 0231335 SB - IM MH - Adult MH - Aged MH - Disease Progression MH - Female MH - Humans MH - Incidence MH - Lung Diseases, Interstitial/*etiology/mortality MH - Male MH - Middle Aged MH - Minnesota/epidemiology MH - Prevalence MH - Risk Factors MH - Scleroderma, Systemic/*complications/mortality PMC - PMC3734889 EDAT- 2013/03/02 06:00 MHDA- 2014/01/10 06:00 PMCR- 2014/08/01 CRDT- 2013/03/02 06:00 PHST- 2013/03/02 06:00 [entrez] PHST- 2013/03/02 06:00 [pubmed] PHST- 2014/01/10 06:00 [medline] PHST- 2014/08/01 00:00 [pmc-release] AID - S0012-3692(13)60534-6 [pii] AID - chest.12-2768 [pii] AID - 10.1378/chest.12-2768 [doi] PST - ppublish SO - Chest. 2013 Aug;144(2):571-577. doi: 10.1378/chest.12-2768.