Session Information
Date: Tuesday, November 7, 2017
Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's – Clinical Aspects and Therapeutics Poster III
Session Type: ACR Poster Session C
Session Time: 9:00AM-11:00AM
Background/Purpose:
The evaluation of patients who develop interstitial lung disease (ILD) includes an assessment for underlying rheumatic disease or connective tissue disease (CTD). Delineating a CTD phenotype in patients with ILD is important as these patients may have a different prognosis compared to idiopathic pulmonary fibrosis (IPF) and other idiopathic interstitial pneumonias (IP). The subset of patients with ILD and predominantly fibrotic radiographic features are particularly challenging as they may be mislabeled as IPF. This study focused on the utility of a Rheumatologist’s clinical diagnosis and serologic testing in identifying those with CTD /ILD in a cohort of patients with fibrotic features.
Methods:
We retrospectively identified 100 new patients who presented to our multidisciplinary ILD clinic from 2013-2014. All were evaluated by a Pulmonologist and the same experienced Rheumatologist who stratified the likelihood of a CTD into 3 groups (no evidence, possible diagnosis or definite diagnosis). Age, sex and serologic data including ANA, RF, CCP, myositis panel, Scl-70, and muscle enzymes were recorded. Each patient was given an initial clinical diagnosis and a final diagnosis based on multidisciplinary consensus from chest CT, biopsy results, and serologic testing.
Results:
Of the 100 patients, 34% had a final diagnosis of IPF, 39% had CTD/ILD, and 27% other IP. (Table) In a subgroup of patients with fibrosis based on CT, a final diagnosis of IPF was made in 48% and final diagnosis of CTD/ILD was noted in 34%. Patients with CTD/ILD were younger and more likely to be female. However, there was no significant difference in age for females with fibrotic features between CTD/ILD and IPF. (Table) Amongst patients with no evidence of CTD on Rheumatologist evaluation, the final diagnosis was IPF in 79%. Serologic testing was positive in 9% but did not change the final diagnosis. In those with possible CTD a final diagnosis of CTD/ILD was made in 56%. Serologic testing was positive in 50% and resulted in a change in diagnosis for 3 patients. In those with a certain CTD all had CTD/ILD and positive serologic testing, but no changes in diagnoses. (Table)
Conclusion: In patients with fibrotic forms of ILD based on CT, CTD is present in a significant number of cases and when the Rheumatologist’s evaluation does not suspect a CTD diagnosis, serologic data is of no significant benefit. In patients where a rheumatologic diagnosis is suspected but not certain, serologic testing does offer benefit diagnostically. Assessment by a rheumatologist is a valuable aspect of the evaluation of the patient with ILD and may obviate the need for extensive serologic testing in a subset of patients.
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Table: Demographics and Diagnoses for Patients with Interstitial Lung Disease |
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Entire Cohort (N=100) |
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Idiopathic Pulmonary Fibrosis |
Connective Tissue Disease associated Interstitial Lung Disease |
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P value |
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N |
34 |
39 |
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Age (SD) |
72.0 (6.9) |
62.9 (13.3) |
P<0.01 |
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Female, N (%) |
16 (47) |
31 (79.5) |
P<0.01 |
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Fibrotic Lung Disease on CT scan (N=64) |
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N |
31 |
22 |
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Age (SD) |
71.5 (6.8) |
65.5 (13.0) |
P=0.03 |
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Female, N (%) |
15 (48) |
18 (82) |
P=0.02 |
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Females with Fibrotic Lung Disease on CT scan (N=33) |
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N |
15 |
18 |
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Age (SD) |
71.9 (7.7) |
66.4 (13.6) |
P=0.17 |
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Fibrotic Lung Disease on CT scan (N=64) |
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No Evidence of Rheumatologic Disease |
Possible Rheumatologic Disease |
Definite Rheumatologic Disease |
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N |
34 |
18 |
12 |
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Age (SD) |
71.1 (7.3) |
64.1 (12.9) |
67.3 (10.3) |
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Female, N (%) |
12 (35) |
16 (89) |
9 (75) |
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Patients with + serologies, N (%) |
3 (9) |
9 (50) |
12 (100) |
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Final diagnosis: IPF, N (%) |
27 (79) |
4 (22) |
0 (0) |
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Final diagnosis: CTD/ILD, N (%) |
0 (0) |
10 (56) |
12 (100) |
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Final diagnosis: other idiopathic IP |
7a (21)
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4b (22) |
0 (0) |
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Change in diagnosis due to serologies, N (%) |
0 (0) |
3c (17) |
0 (0) |
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SD: Standard deviation CTD/ILD: Connective tissue disease associated interstitial lung disease IPF: Idiopathic pulmonary fibrosis IP: interstitial pneumonia a. Hypersensitivity pneumonitis, emphysema/fibrosis, ILD NOS (2) b. Hypersensitivity pneumonitis (3), emphysema/fibrosis (1), ILD NOS (3) c. 1 patient with + Ro had change of diagnosis from RA ILD to interstitial pneumonia with autoimmune features. 1 patient with +myositis panel and +ANA and 1 patient with + myositis panel and + muscle enzymes had change of diagnosis from interstitial pneumonia with autoimmune features to antisynthetase syndrome. |
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To cite this abstract in AMA style:
MacFarlane LA, Dellaripa PF. Impact of Rheumatologic Evaluation and Serologic Testing on Patients with Fibrotic Interstitial Lung Disease: A Single Center Retrospective Experience [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/impact-of-rheumatologic-evaluation-and-serologic-testing-on-patients-with-fibrotic-interstitial-lung-disease-a-single-center-retrospective-experience/. Accessed .« Back to 2017 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/impact-of-rheumatologic-evaluation-and-serologic-testing-on-patients-with-fibrotic-interstitial-lung-disease-a-single-center-retrospective-experience/