title: Early stage lung cancer detection in systemic sclerosis does not portend survival benefit: a cross sectional study.
abstract: BACKGROUND
  Systemic Sclerosis (SSc) is a rare connective tissue disorder associated with an increased risk of malignancy including lung cancer.
  
  
  METHODS
  A single center review of all cases of lung cancer in patients with SSc was conducted. Clinical, radiographic, and detailed pathologic data was collected. Risk factors were compared with our center's SSc Registry. Cancer characteristics were compared with the National Cancer Institute SEER Cancer Statistics (NCI SEER) data.
  
  
  RESULTS
  17 cases were identified; the majority were females (82%) with the lung cancers diagnosed after the onset of SSc (88%). Tobacco use was identified in 65% of cases. Serologic testing showed 50% of cases were Scl-70 positive. Twelve cases had radiographic evidence of SSc lung involvement, however only 6 had restrictive physiology on pulmonary function testing. Thirteen cases had pulmonary nodules preceding lung cancer. Thirteen of the cancers were adenocarcinoma. Ten underwent molecular mutational profiling: 2/8 had KRAS mutation and 1/10 had EGFR mutation. More of the non-small cell lung cancers were diagnosed at localized disease (56%) than in the NCI SEER database. However, 5 years survival among the stage I cases was 25% versus an expected survival of 54%.
  
  
  CONCLUSIONS
  The high proportion of adenocarcinomas seen in our study is different from that reported in the literature. Lung cancers were diagnosed at an early stage, likely due to our center's practice of radiographic screening for SSc associated lung involvement, however this did not confer a survival advantage. A high proportion of patients who developed lung cancer had interstitial lung disease.
authors.affiliation: Department of Medicine, Division of Pulmonary and Critical Care Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois, United States of America.
authors.forename: Jeremy B
authors.initials: JB
authors.lastname: Katzen
authors.affiliation: Department of Pathology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, United States of America.
authors.forename: Kirtee
authors.initials: K
authors.lastname: Raparia
authors.affiliation: Department of Radiology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, United States of America.
authors.forename: Rishi
authors.initials: R
authors.lastname: Agrawal
authors.affiliation: Department of Medicine, Division of Hematology and Oncology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, United States of America.
authors.forename: Jyoti D
authors.initials: JD
authors.lastname: Patel
authors.affiliation: Department of Preventive Medicine, Division of Biostatistics, Northwestern University, Chicago, Illinois, United States of America.
authors.forename: Alfred
authors.initials: A
authors.lastname: Rademaker
authors.affiliation: Department of Medicine, Division of Rheumatology and Northwestern Scleroderma Program, Northwestern University Feinberg School of Medicine, Chicago, Illinois, United States of America.
authors.forename: John
authors.initials: J
authors.lastname: Varga
authors.affiliation: Department of Medicine, Division of Pulmonary and Critical Care Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois, United States of America.
authors.forename: Jane E
authors.initials: JE
authors.lastname: Dematte
mesh_terms: D000328:Adult; D000368:Aged; D002289:Carcinoma, Non-Small-Cell Lung / Q000150:complications / Q000175:diagnosis* / Q000401:mortality; D003430:Cross-Sectional Studies; D055088:Early Detection of Cancer; D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000150:complications* / Q000401:mortality; D008175:Lung Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000401:mortality; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012595:Scleroderma, Systemic / Q000150:complications* / Q000401:mortality; D015996:Survival Rate