Conditions with pulmonary fibrosis
Many conditions can be associated with pulmonary fibrosis. Here we discuss some of the more common ones.
Rheumatoid arthritis-associated interstitial lung disease
Rheumatoid arthritis (RA) is a systemic autoimmune disease. Besides the joints, it can also affect other parts of the body, including lungs. Interstitial lung disease (ILD) refers to a group of disorders that cause inflammation and scarring of the lung tissue, or pulmonary fibrosis.1,2
When someone has both RA and ILD, that is known as rheumatoid arthritis-associated interstitial lung disease (RA-ILD).
Watch the video below to learn more about pulmonary fibrosis in RA:
Symptoms of RA-ILD
Most people don’t notice the changes to their lungs at first, as they may already be feeling fatigued due to their RA.3 However, this can also be one of the symptoms of ILD, so it’s important to be aware of your body’s capabilities and notice any changes.
Symptoms that might indicate ILD4:
The earlier lung involvement is spotted, the sooner you can be tested and get support as your doctor can decide the best way forward for your situation. Symptoms can vary from one person to another, so your symptoms may not be the same as somebody else's who has ILD. Some people might have ILD but don’t experience any respiratory symptoms, like cough or difficulty breathing.
These symptoms may arise from other causes, and do not necessarily mean you have ILD. They are also present in other lung diseases and heart problems, and so can be difficult to tell apart from other lung conditions that can exist alongside RA, including inflammation of the lung lining, airways, or of the network of blood vessels that supply the lungs.5
Quick tip
Keeping a diary of your symptoms may help in a conversation with your doctor if you're concerned. Download our symptom tracker.
Risk factors for ILD
If you have RA, you may be wondering if you could go on to develop ILD. Below are certain risk factors that may elevate the risk for RA-ILD.
RA-ILD stats
- Approximately 10% of people with RA develop ILD8
- An additional 30% of people might have subclinical ILD (an ILD that does not show noticeable symptoms)8
- Most cases of ILD occur within the first 5 years of RA diagnosis5
Research shows that a person is more likely to develop ILD if they have RA and6:
As ILD with pulmonary fibrosis can be progressive, recognising it early is key to getting the support you need. If you have RA with one or more of the above risk factors, speak with your doctor. Depending on the risk, your doctor might decide to check your lungs using high-resolution computed tomography (HRCT), an imaging method to look at your lung tissue and/or pulmonary function tests, including a spirometry test.7
Symptoms of RA Stats
Respiratory symptoms can appear before development of the joint symptoms of RA.5
- In 10% to 20% of cases, respiratory symptoms appeared first5
- 14% of people with RA-ILD were diagnosed with ILD 1 to 5 years before their RA diagnosis9
- However, RA is usually diagnosed before detection of ILD10
The main symptoms of RA are:
Some people can experience more general symptoms, such as11:
If you are concerned that you may have, or be at risk for, RA speak with your doctor.
Monitoring for progression of RA-ILD
RA-ILD can become progressive, which means that over time, your symptoms and the overall impact on your lungs may get worse. A high-resolution computed tomography (HRCT) scan may also show signs of pulmonary fibrosis spreading throughout your lungs. Currently, there is no way of predicting if, or how fast, this will happen. Some patients might not have any changes in their symptoms, while other patients might experience a faster worsening of their symptoms.7
Respiratory symptoms are mostly nonspecific and not all symptoms you experience will be due to ILD.12 It may be difficult to tell whether the symptoms you experience, such as fatigue, are due to ILD or RA.13
Signs of progression
These are signs that can mean the ILD has progressed but your doctor, supported by a wider team of specialists, will work closely with you to monitor your symptoms. Your doctor will then discuss your treatment options and tailor the best treatment plan for you.
Managing life with RA-ILD
Receiving a diagnosis of RA-ILD can feel lonely and uncertain; at these times it can feel very difficult to stay positive. Daily life can be challenging, both practically and emotionally. However, there are many strategies to help people living with pulmonary fibrosis.
Watch the video below to see how Liz manages both rheumatoid arthritis and pulmonary fibrosis.
Abbreviations: CT, computed tomography; GERD, Gastro-oesophageal reflux disease; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis; PF, pulmonary fibrosis; RA, Rheumatoid arthritis; RA-ILD, rheumatoid arthritis-associated interstitial lung disease.
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Olson AL, Gifford AH, Inase N, Fernández Pérez ER, Suda T. The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype. Eur Respir Rev. 2018;27(50):180077.
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Arthritis Foundation. What you need to know about RA and lung disease. Accessed October 2, 2024. https://www.arthritis.org/diseases/more-about/what-you-need-to-know-about-ra-and-lung-disease
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National Institutes of Health. InformedHealth.org. Rheumatoid arthritis: learn more – fatigue related to rheumatoid arthritis. Accessed October 2, 2024. https://www.ncbi.nlm.nih.gov/books/NBK384467/
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Asthma + Lung UK. What are the symptoms of pulmonary fibrosis? Updated June 2, 2022. Accessed October 2, 2024. https://www.blf.org.uk/support-for-you/pulmonary-fibrosis/symptoms
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Shaw M, Collins BF, Ho LA, Raghu G. Rheumatoid arthritis-associated lung disease. Eur Respir Rev. 2015;24(135):1-16.
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Bendstrup E, Møller J, Kronborg-White, Prior TS, Hyldgaard C. Interstitial lung disease in rheumatoid arthritis remains a challenge for clinicians. J Clin Med. 2019;8(12):2038.
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American Lung Association. Stages of pulmonary fibrosis. Updated June 7, 2024. Accessed October 2, 2024. https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/introduction/stages-of-pulmonary-fibrosis
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Geerts S, Wuyts W, Langhe E, Lenaerts J, Yserbyt J. Connective tissue disease associated interstitial pneumonia: a challenge for both rheumatologists and pulmonologists. Sarcoidosis Vasc Diffuse Lung Dis. 2017;34(4):326-335.
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Hyldgaard C, Hilberg O, Pedersen AB, et al. A population-based cohort study of rheumatoid arthritis-associated interstitial lung disease: comorbidity and mortality. Ann Rheum Dis. 2017;76(10):1700-1706.
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Kim EJ, Collard HR, King TE Jr. Rheumatoid arthritis-associated interstitial lung disease: the relevance of histopathologic and radiographic pattern. Chest. 2009;136(5):1397-1405.
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National Health Service. Rheumatoid arthritis - Symptoms. Updated March 8, 2023. Accessed October 2, 2024. https://www.nhs.uk/conditions/rheumatoid-arthritis/symptoms/
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Zibrak JD, Price D. Interstitial lung disease: raising the index of suspicion in primary care. NPJ Prim Care Respir Med. 2014;24:14054.
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Mittoo S, Frankel S, LeSage D, et al. Patient perspectives in OMERACT provide an anchor for future metric development and improved approaches to healthcare delivery in connective tissue disease related interstitial lung disease (CTD-ILD). Curr Respir Med Rev. 2015;11(2):175-183.
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Iqbal K, Kelly C. Treatment of rheumatoid arthritis-associated interstitial lung disease: a perspective review. Ther Adv Musculoskel Dis. 2015;7(6):247-267.
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Raghu G, Remy-Jardin M, Richeldi L, et al. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults. Am J Respir Crit Care Med. 2022;205(9):18-47.
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Swigris JJ, Brown KK, Abdulqawi R, et al. Patients' perceptions and patient-reported outcomes in progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):180075.