When managing scleroderma, it’s important to know about interstitial lung disease.
Interstitial lung disease (ILD) is a complication of systemic sclerosis that may affect breathing. Here’s a big picture overview of what this complication is, what signs to watch out for, common management and treatment strategies, and a look to the horizon for future potential treatments.
An Overview of Interstitial Lung Disease in Scleroderma
Scleroderma-related ILD, or scleroderma-associated interstitial lung disease (SSc-ILD), occurs when there is inflammation and scarring to the lung tissue that supports the alveoli—the air sacs within the lungs. Over time, this scarring, called pulmonary fibrosis, makes it difficult to breathe in and out. This reduces the amount of oxygen that can reach the bloodstream.
ILD is characterized by the extent of lung fibrosis and decreased lung function. When a doctor listens to the lungs of a person with this complication, they may hear what sounds like “velcro” crackles.
Approximately 40% of individuals with systemic sclerosis have clinically significant ILD.1 30% of individuals with SSc develop progressive ILD.5 As with many scleroderma complications, disease progression is variable. Some people may remain stable, others may experience slow progression, and others may experience rapid progression.
Symptoms may include:
- Shortness of breath during normal activities
- Exercise intolerance
- Difficulty taking deep breaths
- Increased fatigue
- Dry cough that you can’t get rid of
- Feeling dizzy
- Widening of the fingertips
- Muscle and joint pain
That said, some ILD is subclinical, which means the only evidence of this complication is through imaging showing fibrosis in the lungs. In these cases, the person with ILD has normal lung function and capacity and no clinical symptoms.
It is not known why some SSc patients develop ILD, while others do not. Development of ILD appears to be associated with previous lung infections, occupational exposures (such as asbestos, coal or grain dust, mold, and silica dust), and certain medications (such as chemotherapy, immunomodulating drugs, heart medications, and some antibiotics).
Screening for ILD in Scleroderma
People with scleroderma are at the highest risk of developing SSc-ILD within the first few years after their SSc diagnosis. The scarring of lung tissue caused by ILD cannot be reversed. As such, early detection of ILD in scleroderma is key.
A baseline evaluation gives you a starting point for understanding your disease. It shows your current status, so you can track if, and how much, things change over time. There are several ways that clinicians may develop a baseline evaluation of SSc-ILD.4
High-resolution computed tomography (HRCT)
This is a specialized type of CT scan that generates highly accurate imagery of your lungs. This can confirm the presence, extent, and pattern of pulmonary fibrosis.
Pulmonary function tests (PFTs)
This is a category of tests that assesses lung function and capacity via the following metrics:
- Spirometry (FVC): This test measures how much air goes into and out of your lungs as you breathe.
- Lung volume: This test measures how much air your lungs can hold.
- Diffusion capacity (DLCO): This test measures how gases, like oxygen, move between the air in your lungs and red blood cells.
Your doctor may want to evaluate symptoms and PFTs every 3-6 months to monitor disease severity and progression. If PFTs or clinical symptoms detect changes or worsening fibrosis, they may consider repeat HRCT scans to learn more.
It’s worth noting that there is an autoantibody biomarker that is related to ILD. Anti-top 1 (SCL-70) can be a highly specific biomarker for SSc-ILD and ILD progression if assessed using ELISA and immunodiffusion.3
Patients may be referred to a pulmonologist for further assessment and treatment.
Treatments for ILD in Scleroderma
There are treatments to improve scleroderma interstitial lung disease symptoms, slow disease progression, and improve the quality of life for people with ILD.
Possible Non-pharmacologic Recommendations
The following measures are often recommended as part of comprehensive care.7
- No smoking
- Acid reflux prevention (elevate the head of the bed, avoid late eating, and specific foods)
- Cardiopulmonary rehabilitation and resistance training
- Palliative care, including symptom-based treatment (cough, pain, air hunger)
- Doctors will measure blood oxygen levels at rest and with activity. If they are low, supplementary oxygen may be prescribed.
Possible Pharmacologic Recommendations
Identifying the best course of treatment is a highly individual process, and your doctor will review many factors when making drug recommendations. This includes considerations like side effects, other medications you currently use, and the state of your disease progression.
Therapies approved by the FDA specifically for SSc-ILD
The FDA has approved two therapies, Nintedanib and Tocilizumab.6
Professional Society Recommendations
Several leading rheumatology and thoracic societies have also made recommendations on additional therapies that may be of use in treating ILD. It’s important to note that these societies make most recommendations conditionally, which means that not every treatment option is appropriate for every SSc-ILD patient.
The European Alliance of Associations for Rheumatology (EULAR), the American College of Rheumatology (ACR), the American College of Chest Physicians (CHEST), and the American Thoracic Society (ATS) all recommend, to some degree, the following medications: mycophenolate, rituximab, and cyclophosphamide.2 4 8
Possible Surgical Recommendations
Lung transplantation may be suggested to patients who have severe SSc-ILD with significant scarring. This is a major procedure and may have its own complications, but it may also significantly improve the patient’s quality of life.
Looking to the Future of Scleroderma-Associated ILD Treatment
There is a need for more treatment options, and as such, there are currently several clinical trials specifically looking at potential SSc-ILD treatment. This means that down the road, new options may be available to you.
Or, if you’re eligible, you could consider participating in a clinical trial where you may have access to try a potential new therapy. As always, consult your doctor when deciding if a clinical trial may be a good option for you.
Watch Now: SRF Webinars about ILD and Participating in SSc-ILD Research
ENROLLMENT INFORMATION
Join the quest to advance systemic sclerosis research through CONQUEST.
Consider enrolling in the CONQUEST study, a scleroderma clinical trial for people with systemic sclerosis-associated interstitial lung disease (SSc-ILD). Participants help progress research to potentially uncover new scleroderma-associated ILD treatment options.
References
1. Allanore Y, Simms R, Distler O, et al., Nat Rev Dis Primers. 2015;1. doi:10.1038/nrdp.2015.2
2. Del Galdo F, Lescoat A, Conaghan PG, et al. Ann Rheum Dis. 2025;84(1):29-40. doi:10.1136/ard-2024-226430 https://pubmed.ncbi.nlm.nih.gov/39874231/
3. Homer KL, Warren J, Karayev D, et al. J Clin Rheumatol. 2020;26(3):115-118. doi: 10.1097/RHU.0000000000000971
4. Johnson SR, Bernstein EJ, Bolster MB, et al. Arthritis and Rheumatology. 2024;76(8):1201-1213. doi:10.1002/art.42860 https://www.the-rheumatologist.org/article/the-acr-and-chest-release-2-new-ild-guidelines/
5. Khanna D, Tashkin DP, Denton CP, Renzoni EA, Desai SR, Varga J. Am J Respir Crit Care Med. 2020;201(6):650-660. doi:10.1164/rccm.201903-0563CI
6. Khanna SA, Nance JW, Suliman SA. Curr Rheumatol Rep. 2022;24(5):166-173. doi:10.1007/s11926-022-01067-5 https://pubmed.ncbi.nlm.nih.gov/35499699/
7. Parodis I, Girard-Guyonvarc’h C, Arnaud L, et al. Ann Rheum Dis. 2024;83(6):720-729. Published 2024 May 15.doi:10.1136/ard-2023-224416
8. Raghu G, Montesi SB, Silver RM, et al. Am J Respir Crit Care Med. 2024;209(2):137-152. doi:10.1164/rccm.202306-1113ST https://pubmed.ncbi.nlm.nih.gov/37772985/
