Session Information
Date: Monday, November 9, 2015
Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's - Clinical Aspects and Therapeutics Poster II
Session Type: ACR Poster Session B
Session Time: 9:00AM-11:00AM
Background/Purpose: Several studies have shown that gastroesophageal reflux disease (GERD) is a risk factor in the progression of idiopathic pulmonary fibrosis. In systemic sclerosis (SSc), esophageal involvement is common; however, it does not generate symptoms in 50% of patients. Therefore, the presence of esophageal dilation and GERD may be a poor prognostic factor in patients with systemic sclerosis and diffuse interstitial lung disease (ILD-SSc) as a result of recurrent episodes of micro-aspiration. Our objective was to analyze whether the presence of esophageal dilation is a poor prognostic factor in ILD-SSc.
Methods: Thirty-one patients with SSc (ACR/EULAR 2013 criteria) and secondary symptomatic ILD, as confirmed by thoracic HRCT, were studied. HRCT images performed at the time of ILD diagnosis were reviewed with particular attention to: a)the presence of dilation in the lower two-thirds of the esophagus and b) the extent of ground glass areas and fibrotic changes (honeycombing areas, thickening of the interlobular septa and traction bronchiectasis with architectural distortion). The extent of HRCT lung abnormalities were scored in 3 categories: 1 = involvement of < 25% of total pulmonary parenchyma, 2 = 25-30%, and 3 = > 50%.
Results: Esophageal dilation was detected in the HRCT of 15 (48%) patients. The main clinical characteristics of the patients and the results from the comparative study between groups are shown in the following table:
|
Esophageal dilation in the HRCT |
No esophageal dilation in the HRCT N=16 |
p | |
|
Age (mean±SD), yrs |
54 ± 16 |
63 ± 15 |
0.120 |
|
SSc Types |
Limited :5 Diffuse: 9 SSc sine escleroderma 1 |
Limited: 12 Difuse: 2 SSc sine escleroderma:2 |
0.022 |
|
SSc disease duration (median ±SD), months |
102 ± 143 |
112 ± 95 |
0.812 |
|
ILD type |
NSIP: 12 UIP: 3 |
NSIP:12 UIP:4 |
0.095 |
|
ILD disease duration (median ±SD), months |
35 ± 27 |
70 ± 44 |
0.014 |
|
Baseline HRCT |
|||
|
Ground glass areas |
Score 1: 6 Score 2: 6 Score 3: 0 |
Score 1: 10 Score 2: 1 Score 3: 0 |
0.033 |
|
Fibrotic changes |
Score 1: 2 Score 2: 4 Score 3: 0 |
Score 1: 4 Score 2: 0 Score 3: 0 |
0.035 |
|
Baseline PFT |
|||
|
FVC% (mean ± SD) |
83.9 ± 22.1 |
103.4 ± 19.5 |
0.015 |
|
TLC% |
87.9 ± 25.7 |
110.7 ± 33.9 |
0.116 |
|
DLCO% |
57.4 ± 17.9 |
71.3 ± 24.3 |
0.095 |
|
PFT at the end of the follow-up period |
|||
|
FVC% |
79 ± 20.8 |
98.3 ± 26 |
0.031 |
|
TLC% |
79 ±19.2 |
98.5 ± 26.8 |
0.067 |
|
DLCO% |
48.8 ±15.7 |
62.2 ±18.3 |
0.046 |
|
Treatment with CYC and/or rituximab |
10 (67%)
|
5 (31%) |
0.049 |
|
Treatment with proton pump inhibitors |
14 (93%) | 11 (69%) | 0.172 |
At the time of ILD-SSc diagnosis, patients with esophageal dilation showed a greater extent of ground glass areas (P = 0.033) and fibrotic changes (P = 0.035) in the HRCT. A relatively common finding in these patients was an asymmetry in injury severity with greater involvement in one of the two lungs. In addition, patients with esophageal dilation showed greater deterioration of lung function parameters, although the differences were only statistically significant for baseline (P = 0.015) and final (P = 0.031) FVC measurements and for the final DLCO measurement (P= 0.046). These patients also received more frequent treatment with CYC or rituximab.
Conclusion: The presence of esophageal dilation appears to be a poor prognostic factor in ILD-SSc, which relates to a greater extent of HRCT lung abnormalities and further deterioration in baseline pulmonary function tests (PFT)
To cite this abstract in AMA style:
Narváez J, Heredia S, Borrell Paños H, Armengol E, De Lama E, Narvaez JA. Is the Presence of Esophageal Dilation a Poor Prognostic Factor in Dilated Interstitial Lung Disease Associated with Systemic Sclerosis? [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/is-the-presence-of-esophageal-dilation-a-poor-prognostic-factor-in-dilated-interstitial-lung-disease-associated-with-systemic-sclerosis/. Accessed .« Back to 2015 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/is-the-presence-of-esophageal-dilation-a-poor-prognostic-factor-in-dilated-interstitial-lung-disease-associated-with-systemic-sclerosis/