SAT0280 ESOPHAGEAL DYSMOTILITY AND PULMONARY DISEASE IN PATIENTS WITH SCLERODERMA: A RETROSPECTIVE STUDY
Authors: Navya Kuchipudi, Shweta Kishore, Chia-Ling Kuo, Santhanan Lakshminarayanan, Ranadeep Mandhadi
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Systemic sclerosis (SSc) is a connective tissue disease with pulmonary involvement seen in 75% of patients and esophageal involvement in 90% of the patients. Pulmonary disease has overtaken renal disease as the leading cause of death (1). There is conflicting evidence about the association between esophageal dysmotility and lung involvement (Interstitial Lung Disease (ILD) and Pulmonary Artery Hypertension (PAH)). <h3>Objectives</h3> Our aim was to evaluate the relationship between esophageal dysmotility and lung disease by correlating the results of Echocardiogram and Pulmonary Function Test (PFT) with Esophageal Transit Study (ETT). <h3>Methods</h3> Charts of SSc patients fulfilling 2013 ACR/EULAR classification criteria seen in Rheumatology clinic from 2004 to 2015 were reviewed. Patient demographics, ETT result, FVC and DLCO data from PFT as well as pulmonary pressures from echocardiogram were collected at baseline, years 1, 3, 5 and 10. Patients were divided based on their initial ETT findings into normal and abnormal ETT groups. Using logistic regression models, we compared elevated PASP with ETT status at baseline, and years 3 and 5. Linear mixed effects model was used to adjust for covariates (disease subtype, smoking, obesity, use of immunomodulator) when analyzing PFT outcome with time period and ETT status <h3>Results</h3> 130 patients were identified with either Limited Cutaneous SSc (109) or Diffuse Cutaneous SSc (21) with a mean age of 52.65 years ± 12.59. ETT was normal in 67(52%) and abnormal in 63(48%) patients. Number of patients with abnormal PASP was not statistically different between the two groups (p values 0.104, 0.178, 0.653 at baseline, years 3 and 5 respectively). Likewise, the odds ratio for abnormal PASP was 2.75 in patients with abnormal ETT compared to normal ETT, but the results were not statistically significant (p: 0.428), and the odds ratio did not vary with time (p: 0.731). Sample size was too small to conduct separate longitudinal analysis for adjusted models. The mean DLCo was statistically worse in abnormal ETT patients [p value &lt;0.0001 and 0.0004 for unadjusted and adjusted models] as were the progression rates per year for DLCo at -1.95 (p-value: 0.023) and -2.25 (p-value: 0.019) for unadjusted and adjusted models respectively. The Mean FVC was not statistically different between the two groups, although it's progression rate per year was statistically worse only in the adjusted model (p value: 0.018). <h3>Conclusion</h3> Esophageal dysmotility was associated with increased pulmonary involvement in the form of abnormal DLCo with worsening progression rates per year. Interestingly, neither PASP nor FVC were statistically different even though progression rate for FVC was worse in adjusted models. We postulate that the small sample size for Echo and differences in the factors determining DLCo vs. FVC and PASP explains the disparity in the results. Larger prospective trials are needed to investigate if there is a causal relationship between esophageal dysfunction and pulmonary involvement. <h3>Reference</h3> [1] Mathai, S. C., Hummers, L. K. (2009), Survival in pulmonary hypertension associated with the scleroderma spectrum of diseases: Impact of interstitial lung disease. Arthritis &amp; Rheumatism,60: 569-577. doi:10.1002/art.24267 <h3>Disclosure of Interests</h3> None declared