Early Stage Lung Cancer Detection in Systemic Sclerosis Does Not Portend Survival Benefit: A Cross Sectional Study

Abstract

BackgroundSystemic Sclerosis (SSc) is a rare connective tissue disorder associated with an increased risk of malignancy including lung cancer.MethodsA 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.Results17 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%.ConclusionsThe 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.

Lung Cancer Survival in Patients With Autoimmune Disease

“…Some studies suggest that cancer is overall more aggressive in this group. 29 , 30 However, other studies did not observe an association between autoimmune disease and increased lung cancer mortality. 31 , 32 Why autoimmune disease could portend worse lung cancer prognosis is not clear.…”

Systemic Sclerosis Associated Interstitial Lung Disease: New Directions in Disease Management

“…Frequent HRCT images are not recommended and can be repeated when a change in clinical symptoms occur. (56) Most physicians seem to treat patients with extensive lung disease (presentation in HRCT and lung biopsy with UIP pattern, and evidence of ground glass opacities occupying more than 10% of lungs (Figures 1, 2). With the completion of more randomized clinical trials, newer treatments with or without the adopted immunosuppressive agents may demonstrate efficacy in SSc-ILD.…”

Clinicopathological characteristics of lung cancer in patients with systemic sclerosis

“…This is in consistent with the reported histological pattern of the SSc-associated lung cancer. [10][11][12] Noteworthy, of the nine patients with adenocarcinoma, all are female lifelong nonsmoker, and unexpectedly, did not harbor EGFR mutation or ALK-EML4 fusion gene. The reported prevalence of EGFR mutations is approximately 50% in Asian population 13,14 and even higher (75%) in female never-smokers.…”