EN | TR
E-ISSN: 2651-2661 ISSN: 2651-2653
The main differences between connective tissue diseases-associated interstitial lung disease and idiopathic pulmonary fibrosis
PDF
Cite
Share
Request
Review
VOLUME: 13 ISSUE: 1
P: 22-28
April 2021

The main differences between connective tissue diseases-associated interstitial lung disease and idiopathic pulmonary fibrosis

J Turk Soc Rheumatol 2021;13(1):22-28
DOI: 10.4274/raed.galenos.2021.39306
Ahmet Karataş
Burak Öz
Rabia Pişkin Sağır
Mustafa Gür
Süleyman Serdar Koca
1. Fırat Üniversitesi Tıp Fakültesi, İç Hastalıkları Anabilim Dalı, Romatoloji Bilim Dalı, Elazığ, Türkiye
No information available.
No information available
Received Date: 02.04.2020
Accepted Date: 15.02.2021
Publish Date: 29.04.2021
PDF
Cite
Share
Request

ABSTRACT

Connective tissue diseases (CTD) can often cause respiratory system involvement. Interstitial lung disease (ILD) is an example of respiratory system involvement, which is the most frequent cause of mortality and morbidity, which challenges patients and their physicians in routine practice. Idiopathic pulmonary fibrosis (IPF) is another common cause of ILD. Similarities between CTD-ILD and IPF in terms of pathogenic, clinical and radiological findings complicate the distinction of the two diseases. However, the treatment of CTD-ILD and IPF is very different. Therefore, it is very important to distinguish both. ILD is grouped histopathologically and radiologically as usual interstitial pneumonia (UIP), non-specific interstitial pneumonia (NSIP), lymphocytic interstitial pneumonia (LIP) and organized pneumonia. Patients with CTD-ILD in the NSIP pattern have a better response to immunosuppressive therapy than patients with the UIP pattern. The effectiveness of antifibrotic therapy is more prominent in patients with the UIP pattern. While IPF is in the UIP pattern, CTD-ILD is often in the NSIP pattern. However, rheumatoid arthritis may be in the UIP pattern and Sjögren’s syndrome in the LIP pattern.

References

1
Rosas IO, Dellaripa PF, Lederer DJ, Khanna D, Young LR, Martinez FJ. Interstitial lung disease: NHLBI workshop on the primary prevention of chronic lung diseases. Ann Am Thorac Soc 2014;11(Suppl 3):S169-77.
2
Travis WD, Costabel U, Hansell DM, et al. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2013;188:733-48.
3
Mira-Avendano I, Abril A, Burger CD, et al. Interstitial lung disease and other pulmonary manifestations in connective tissue diseases. Mayo Clin Proc 2019;94:309-25.
4
Fischer A, Strek ME, Cottin V, et al. Proceedings of the American College of Rheumatology/Association of Physicians of Great Britain and Ireland connective tissue disease-associated interstitial lung disease summit: a multidisciplinary approach to address challenges and opportunities. Arthritis Rheumatol 2019;71:182-95.
5
Perelas A, Silver RM, Arrossi AV, Highland KB. Systemic sclerosis-associated interstitial lung disease. Lancet Respir Med 2020;8:304-20.
6
Denton CP, Wells AU, Coghlan JG. Major lung complications of systemic sclerosis. Nat Rev Rheumatol 2018;14:511-27.
7
Perelas A, Arrossi AV, Highland KB. Pulmonary manifestations of systemic sclerosis and mixed connective tissue disease. Clin Chest Med 2019;40:501-18.
8
Farquhar H, Vassallo R, Edwards AL, Matteson EL. Pulmonary complications of rheumatoid arthritis. Semin Respir Crit Care Med 2019;40:194-207.
9
Flament T, Bigot A, Chaigne B, Henique H, Diot E, Marchand-Adam S. Pulmonary manifestations of Sjögren’s syndrome. Eur Respir Rev 2016;25:110-23.
10
Lopez Velazquez M, Highland KB. Pulmonary manifestations of systemic lupus erythematosus and Sjögren’s syndrome. Curr Opin Rheumatol 2018;30:449-64.
11
Hervier B, Uzunhan Y. Inflammatory myopathy-related interstitial lung disease: from pathophysiology to treatment. Front Med (Lausanne) 2020;6:326.
12
Lederer DJ, Martinez FJ. Idiopathic pulmonary fibrosis. N Engl J Med 2018;378:1811-23.
13
Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011;183:788-824.
14
Fischer A, Antoniou KM, Brown KK, et al. An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J 2015;46:976-87.
15
Herzog EL, Mathur A, Tager AM, Feghali-Bostwick C, Schneider F, Varga J. Review: interstitial lung disease associated with systemic sclerosis and idiopathic pulmonary fibrosis: how similar and distinct? Arthritis Rheumatol 2014;66:1967-78.
16
Adegunsoye A, Vij R, Noth I. Integrating genomics into management of fibrotic interstitial lung disease. Chest 2019;155:1026-40.
17
Arish N, Petukhov D, Wallach-Dayan SB. The role of telomerase and telomeres in interstitial lung diseases: from molecules to clinical implications. Int J Mol Sci 2019;20:2996.
18
Hsu E, Shi H, Jordan RM, Lyons-Weiler J, Pilewski JM, Feghali-Bostwick CA. Lung tissues in patients with systemic sclerosis have gene expression patterns unique to pulmonary fibrosis and pulmonary hypertension. Arthritis Rheum 2011;63:783-94.
19
Bouros D, Wells AU, Nicholson AG, et al. Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med 2002;165:1581-6.
20
Toyoda Y, Hanibuchi M, Kishi J, et al. Clinical features and outcome of acute exacerbation of interstitial pneumonia associated with connective tissue disease. J Med Invest 2016;63:294-9.
21
Collard HR, Ryerson CJ, Corte TJ, et al. Acute exacerbation of idiopathic pulmonary fibrosis. An international working group report. Am J Respir Crit Care Med 2016;194:265-75.
22
Enomoto N, Oyama Y, Enomoto Y, et al. Differences in clinical features of acute exacerbation between connective tissue disease-associated interstitial pneumonia and idiopathic pulmonary fibrosis. Chron Respir Dis 2019;16:1479972318809476. doi: 10.1177/1479972318809476.
23
Suda T, Kaida Y, Nakamura Y, et al. Acute exacerbation of interstitial pneumonia associated with collagen vascular diseases. Respir Med 2009;103:846-53.
24
Tachikawa R, Tomii K, Ueda H, et al. Clinical features and outcome of acute exacerbation of interstitial pneumonia: collagen vascular diseases-related versus idiopathic. Respiration 2012;83:20-7.