Scleroderma can affect the lungs in a variety of ways and the following information was sourced from Patients Engage; an enterprise with social impact, a patient / caregiver focused healthcare platform for supporting the management of chronic diseases.
In June 2023 Dr. Sujeet Rajan, a highly reputed Pulmonologist spoke on Interstitial Lung Disease (ILD) and its management in Scleroderma at the Sclerocon e-conference.
Dr. Rajan is a practising respiratory physician in Mumbai, India. He graduated from the Grant Medical College in Mumbai in 1989.
Following his post-graduation in Respiratory Medicine from the Seth, Dr. Rajan spent a brief time as a Lecturer at the GS Medical College following which he joined the Bhatia Hospital in Mumbai in January of 1996. He is also a post-graduate teacher at the Bombay Hospital Institute of Medical Sciences.
“There are three parts in the lung that we must know to understand what is happening in because sometimes you have an airway problem, sometimes you have a structure problem, and sometimes you have a problem in the lung vessels which is the pulmonary arteries.
1. The Airways are the simplest part of the lung. They are balanced, bilateral and branched out into the lungs and look like trees turned upside down. They are rarely involved in Scleroderma. In airway diseases such as COPD and Asthma, these branches become narrow and the lungs distend. Such diseases are pretty easy to manage with inhalers.
2. Structural or Parenchymal diseases are more difficult to treat. This is what gets affected in Scleroderma. The interstitium is the thin lining inside our lung which transfers oxygen into the blood. The airways just bring the oxygen to the line, so they are the conducting mechanism – but the transfer into the blood occurs via the interstitium. Therefore, one of the tests to understand the interstitium is called the DLCO, which measures how well this interstitium is functioning.
If you stretch out the interstitium it is as big as a tennis court. It can get affected in two ways, one is that it can get swollen, and the other is it can get fibrosed or scarred. Both of these situations cause a problem in getting the oxygen into the blood.
Inflammation can be potentially reversed, but once fibrosis occurs there’s no drug available to reverse it.
For inflammation you have steroids, azathioprine, mycophenolate and cyclophosphamide that have been used to reverse the inflammation with decent results. For fibrosis you have anti-fibrotic drugs available to help slow the progress.”
In the full article you can read a bit more about fibrosis and the prognostic factors that you need to know to get fibrosis treated or prevent the progression. Along with an overview of the studies that have been carried out to date (June 2023).
But as with all information check with your doctor / specialist what is relevant for you and your symptoms, as Scleroderma manifests itself in a myriad of ways.
I was further diagnosed with Interstitial Lung Disease only a few years after initially being diagnosed with Scleroderma in 2018, and have been on a bit of a journey ever since. Trying to work out how this incurable chronic autoimmune disease is affecting my body, mind and spirit.
This has involved a number of scans and tests, and as the disease slowly progresses making adjustments to my daily life so that I can continue thriving instead of letting it defeat me and those around me.
Recent scans and tests have revealed additional things / concerns that we will need to discuss with our specialists in due course, as we continue navigating this journey – one being the involvement of the third area that Dr. Sujeet touches on next…
“3. The third part of our lung is the vasculature and all the pulmonary arteries. About 15 years ago we had a blank slide on treating pulmonary artery hypertension, but we now have multiple treatments.
The right ventricle in our heart pumps through the pulmonary artery to the lungs. The lungs oxygenate this blood and returns it to the left atrium and down to the left ventricle and then it’s pumped to our entire body.
When pulmonary hypertension develops, the right ventricle pressure goes up.”
The thing is, if this pressure goes higher than the left ventricle you may experience bouts of dizziness and potentially blackout.
So it is far better to be diagnosed at an early stage through having an echocardiogram on a regular basis to highlight any early markers / changes.
“The treatments in pulmonary hypertension are those that are exclusive to the disease and patient needs.” They may include; PDE5 Inhibitors (Sildenafil, Tadalafil), Endothelin Receptor Antagonists (Bosentan, Ambrisentan), Prostacyclin Receptor Agonists (Inhaled Iloprost, IV Epoprostenol, Inhaled Treprostinil, Oral Selexipag), Soluble Guanyl Cyclate Inhibitors (Riociguat).
Unfortunately, “a lot of ILD patients get pulmonary hypertension” therefore it is important to explore preventative and rehabilitative therapy for those with SSc-ILD.
- Vaccinations are critical as one bad attack of the flu or Covid can really set you back.
- Management of Reflux because of esophageal dilation.
- Pulmonary Rehabilitation under a physiotherapist who understands cardiopulmonary rehabilitation is extremely important as symptoms progress.
- The use of Oxygen may be required at rest, when active, or potentially all the time. It’ll pay to know your oxygen saturation rate at rest, as well as when exercising to get an accurate picture.
- Explore Palliative Care – Unfortunately, “palliative care is often misunderstood as hospice and end-of-life care and is perceived as giving up on the patient.” This is not the case it often needs to go hand in hand with regular treatment and rehabilitation.
- Regular check-up with specialists and doctors, and explore referrals (as required) to other specialists as and when symptoms progress.
Hopefully, you have found this information beneficial.
Check out the full article, or listen to the presentation to the Sclerocon e-conference held in June 2023 on the Patients Engage site, to find out even more information about Interstitial Lung Disease and its management in Scleroderma.
Blessings ’til next time 🙂
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