4. Clinical and subclinical characteristics of interstitial lung disease in connective tissue disease

Bui Van Dan, Tran Minh Long Trieu, Nguyen Anh Minh, Tran Van Luong, Nguyen Hoang Phuong, Le Dinh Tung

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Abstract

Our study aimed to evaluate the frequency and phenotype of interstitial lung disease (ILD) in patients diagnosed with connective tissue disease (CTD). Cross-sectional descriptive study on 49 patients with a confirmed diagnosis of CTD including systemic sclerosis (SSc), systemic lupus erythematosus (SLE), polymyositis/dermatomyositis (PM/DM) and mixed connective tissue disease (MCTD), treated at the Center for Allergy - Clinical Immunology, Bach Mai Hospital from August 2023 to February 2024. The average age of the study subjects was 57.8 ± 12.1 (years), mainly women (∼80%), ILD was definitely diagnosis after CTD at around 18.1 months. SSc is most common in 21/49 cases (42.9%), SLE in 16/49 patients (32.7%), and PM/DM is 9/49 cases (18.4%) and the lowest MCTD group with 3/49 patients (6.1%). About 67.3% of cases present with cough; 87.5% of patients had difficulty breathing on exertion, including 29/49 cases of mMRC = 2; and over 75.5% of cases found ILD signs when auscultating the lungs. Abnormalities on regular chest X-ray can only be detected in 50 - 60% of cases. On HRCT, ground glass opacities, honeycombing, and traction bronchiectasis are the most common types of interstitial lesions. NSIP and UIP are two common patterns in the ILD-CTD group. We found that ILD can be seen in most CTD diseases with different morphology patterns. Therefore, a thorough clinical examination is needed and an early HRCT scan may be taken to avoid missed or delayed ILD diagnosis in the CTD group.

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References

1. Wijsenbeek M, Cottin V. Spectrum of Fibrotic Lung Diseases. N Engl J Med. 2020;383(10):958-968.
2. Ye Y, Sing CW, Hubbard R, et al. Prevalence, incidence, and survival analysis of interstitial lung diseases in Hong Kong: a 16-year population-based cohort study. Lancet Reg Health West Pac. 2024;42:100871.
3. Duchemann B, Annesi-Maesano I, Jacobe de Naurois C, et al. Prevalence and incidence of interstitial lung diseases in a multi-ethnic county of Greater Paris. Eur Respir J. 2017;50(2).
4. Jeganathan N, Sathananthan M. The prevalence and burden of interstitial lung diseases in the USA. ERJ Open Res. 2022;8(1).
5. Gotway MB, Freemer MM, King TE Jr. Challenges in pulmonary fibrosis. 1: Use of high resolution CT scanning of the lung for the evaluation of patients with idiopathic interstitial pneumonias. Thorax. 2007;62(6):546-553.
6. Dai Y, Wang W, Yu Y, et al. Rheumatoid arthritis-associated interstitial lung disease: an overview of epidemiology, pathogenesis and management. Clin Rheumatol. 2021;40(4):1211-1220.
7. Perelas A, Silver RM, Arrossi AV, et al. Systemic sclerosis-associated interstitial lung disease. Lancet Respir Med. 2020;8(3):304-320.
8. Fathi M, Vikgren J, Boijsen M, et al. Interstitial lung disease in polymyositis and dermatomyositis: longitudinal evaluation by pulmonary function and radiology. Arthritis Rheum. 2008;59(5):677-685.
9. Pego-Reigosa JM, Medeiros DA, Isenberg DA. Respiratory manifestations of systemic lupus erythematosus: old and new concepts. Best Pract Res Clin Rheumatol. 2009;23(4):469-480.
10. Gunnarsson R, Aalokken TM, Molberg O, et al. Prevalence and severity of interstitial lung disease in mixed connective tissue disease: a nationwide, cross-sectional study. Ann Rheum Dis. 2012;71(12):1966-1972.
11. Deneuville L, Mageau A, Debray MP, et al. Chronic interstitial lung disease associated with systemic lupus erythematosus: A multicentric study of 89 cases. Respirology. 2024.
12. Yayla ME, Balci G, Torgutalp M, et al. Interstitial Lung Disease in Systemic Sclerosis: A Single-center Retrospective Analysis. Curr Rheumatol Rev. 2022;18(2):150-156.
13. Li Y, Gao X, Li Y, et al. Predictors and Mortality of Rapidly Progressive Interstitial Lung Disease in Patients With Idiopathic Inflammatory Myopathy: A Series of 474 Patients. Front Med (Lausanne). 2020;7:363.
14. Lưu Phương Lan. Tổn thương phổi trên bệnh nhân xơ cứng bì hệ thống. Tạp chí Nghiên cứu Y học. 2018;112:7.
15. Nguyễn Anh Minh, Hoàng Thị Lâm. Đặc điểm lâm sàng và cận lâm sàng của bệnh nhân viêm da cơ tự miễn có viêm phổi kẽ tại Bệnh viện Bạch Mai. Tạp chí Y học dự phòng. 2020;30(6):7.
16. Lu M, Gong L, Huang C, et al. Analysis of Clinical Characteristics of Connective Tissue Disease-Associated Interstitial Lung Disease in 161 Patients: A Retrospective Study. Int J Gen Med. 2022;15:8617-8625.
17. Hyldgaard C, Bendstrup E, Pedersen AB, et al. Interstitial Lung Disease in Connective Tissue Diseases: Survival Patterns in a Population-Based Cohort. J Clin Med. 2021;10(21).