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Treatment of primary sjögren's syndrome-related interstitial lung disease: a retrospective cohort study.
Sarcoidosis Vasc Diffuse Lung Dis. 2020; 37(2):136-147.SV

Abstract

Background

Interstitial lung disease (ILD) is a common complication of primary Sjögren's syndrome (pSS). Because there is a paucity of literature on the management of pSS-associated ILD (pSS-ILD), this retrospective cohort study assessed the efficacy of azathioprine and mycophenolate therapy in adult patients with pSS-ILD.

Methods

A retrospective cohort study was performed using electronic health records to identify adults meeting the 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for pSS. The presence of pSS-ILD was confirmed by characteristic high-resolution computed tomography and/or histopathology findings. Sociodemographic, clinical, and pulmonary function test (PFT) data were abstracted for patients meeting the criteria and followed longitudinally from the date of their ILD diagnosis. PFT values were anchored on time of treatment start, and linear mixed-effects modeling was used to analyze changes in diffusion capacity for carbon monoxide (DLCO) and forced vital capacity (FVC) before and after treatment initiation.

Results

We identified 19 subjects who had pSS-ILD, of whom seven were treated with azathioprine and seven were treated with mycophenolate. Within the azathioprine treated group, FVC% slope change trended toward improvement from a rate of -9.8% per month pre-treatment to 2.1% per month post-treatment (p = 0.13). Within the mycophenolate treated group, FVC% slope change improved from a rate of 1.5% per month pre-treatment to 4.3% per month post-treatment (p = 0.02) and DLCO% slope changed from a rate of -3.8% to -1.3% per month (p = 0.01) after therapy start.

Conclusions

Mycophenolate treatment was associated with significant improvement in PFTs of pSS-ILD patients over time, and azathioprine treatment followed a similar non-significanttrend. Additional prospective studies are needed to further evaluate these findings. (Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (2): 136-147).

Authors+Show Affiliations

Division of Rheumatology, Department of Internal Medicine Santa Clara Valley Medical Center, San Jose, CA.Rheumatology, Proclinic Center, Cairo, Egypt.Division of Pulmonary & Critical Care, Department of Internal Medicine, Santa Clara Valley Medical Center, San Jose, CA.Department of Radiology, University of Wisconsin School of Medicine and Public Health, Madison, WI.Division of Pulmonary & Critical Care, Department of Internal Medicine, University of Wisconsin School of Public Health, Madison, WI, 53705, U.S.A.Division of Pulmonary & Critical Care, Department of Internal Medicine, University of Wisconsin School of Public Health, Madison, WI, 53705, U.S.A.Department of Biostatistics, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin.Division of Rheumatology, Department of Internal Medicine, University of Wisconsin School of Public Health, Madison, WI.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

33093777

Citation

Amlani, Barkha, et al. "Treatment of Primary Sjögren's Syndrome-related Interstitial Lung Disease: a Retrospective Cohort Study." Sarcoidosis, Vasculitis, and Diffuse Lung Diseases : Official Journal of WASOG, vol. 37, no. 2, 2020, pp. 136-147.
Amlani B, Elsayed G, Barvalia U, et al. Treatment of primary sjögren's syndrome-related interstitial lung disease: a retrospective cohort study. Sarcoidosis Vasc Diffuse Lung Dis. 2020;37(2):136-147.
Amlani, B., Elsayed, G., Barvalia, U., Kanne, J. P., Meyer, K. C., Sandbo, N., Li, Z., & McCoy, S. S. (2020). Treatment of primary sjögren's syndrome-related interstitial lung disease: a retrospective cohort study. Sarcoidosis, Vasculitis, and Diffuse Lung Diseases : Official Journal of WASOG, 37(2), 136-147. https://doi.org/10.36141/svdld.v37i2.8461
Amlani B, et al. Treatment of Primary Sjögren's Syndrome-related Interstitial Lung Disease: a Retrospective Cohort Study. Sarcoidosis Vasc Diffuse Lung Dis. 2020;37(2):136-147. PubMed PMID: 33093777.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Treatment of primary sjögren's syndrome-related interstitial lung disease: a retrospective cohort study. AU - Amlani,Barkha, AU - Elsayed,Ghada, AU - Barvalia,Umang, AU - Kanne,Jeffrey P, AU - Meyer,Keith C, AU - Sandbo,Nathan, AU - Li,Zhanhai, AU - McCoy,Sara S, Y1 - 2020/06/30/ PY - 2019/05/10/received PY - 2020/05/12/accepted PY - 2020/10/23/entrez PY - 2020/10/24/pubmed PY - 2020/11/4/medline KW - Interstitial Lung Disease KW - Sjögren’s Syndrome KW - Treatment SP - 136 EP - 147 JF - Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG JO - Sarcoidosis Vasc Diffuse Lung Dis VL - 37 IS - 2 N2 - Background: Interstitial lung disease (ILD) is a common complication of primary Sjögren's syndrome (pSS). Because there is a paucity of literature on the management of pSS-associated ILD (pSS-ILD), this retrospective cohort study assessed the efficacy of azathioprine and mycophenolate therapy in adult patients with pSS-ILD. Methods: A retrospective cohort study was performed using electronic health records to identify adults meeting the 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for pSS. The presence of pSS-ILD was confirmed by characteristic high-resolution computed tomography and/or histopathology findings. Sociodemographic, clinical, and pulmonary function test (PFT) data were abstracted for patients meeting the criteria and followed longitudinally from the date of their ILD diagnosis. PFT values were anchored on time of treatment start, and linear mixed-effects modeling was used to analyze changes in diffusion capacity for carbon monoxide (DLCO) and forced vital capacity (FVC) before and after treatment initiation. Results: We identified 19 subjects who had pSS-ILD, of whom seven were treated with azathioprine and seven were treated with mycophenolate. Within the azathioprine treated group, FVC% slope change trended toward improvement from a rate of -9.8% per month pre-treatment to 2.1% per month post-treatment (p = 0.13). Within the mycophenolate treated group, FVC% slope change improved from a rate of 1.5% per month pre-treatment to 4.3% per month post-treatment (p = 0.02) and DLCO% slope changed from a rate of -3.8% to -1.3% per month (p = 0.01) after therapy start. Conclusions: Mycophenolate treatment was associated with significant improvement in PFTs of pSS-ILD patients over time, and azathioprine treatment followed a similar non-significanttrend. Additional prospective studies are needed to further evaluate these findings. (Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (2): 136-147). SN - 2532-179X UR - https://www.unboundmedicine.com/medline/citation/33093777/Treatment_of_primary_sjögren's_syndrome_related_interstitial_lung_disease:_a_retrospective_cohort_study_ L2 - https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/33093777/ DB - PRIME DP - Unbound Medicine ER -