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Who is at risk of developing pulmonary hypertension?

This Premium Q&A, reviewed and published, features a real conversation between an iCliniq user and a physician.

Patient's Query

Hello doctor,

Six years ago, I was diagnosed with dermatomyositis, which caused ILD with very mild pulmonary fibrosis on the base of the lung. I am currently in remission. What are my chances of getting pulmonary hypertension as a result of this? I recently read on the internet that it could occur. I am currently taking 2 grams per day of Cellcept.

Thank you.

Hello,

Welcome to icliniq.com.

I read your query and can understand your concern.

Pulmonary hypertension(high blood pressure in the lung's vessels, making the heart's right side work harder) usually develops in two situations, especially in interstitial lung disease (ILD, explain) patients.

1. If the patient develops hypoxia(low-level of oxygen in the body).

2. If underlying Fibrosis(formation of fibrous tissue) is worsening.

Could you please provide the following information:

1. Your baseline oxygen saturation?

2. Any respiratory symptoms like breathing difficulty, coughing, or sputum production?

3. When did you undergo the last CT (Computed tomography) thorax?

Kindly respond with the answers to the above-asked questions so that I can guide you better about pulmonary hypertension in your case.

I hope I have answered your question.

Let me know if I can assist you further.

Regards.

Patient's Query

Hello doctor,

Thank you for your reply.

My oxygen Saturation is between 97 percent and 100 percent, so fine. I underwent a CT(computed tomography) scan last year and there has been no significant change in the small amount of fibrosis. I have no cough. I had an echocardiogram last year which was fine. I plan to undergo another echocardiogram this year as well.

My biggest fear is about the future. What is the likelihood of getting pulmonary hypertension in the future? Any information you could share, I would be grateful.

Hello,

Welcome back to icliniq

It is good that you are not having hypoxia or any other respiratory symptoms. Generally, the development of pulmonary hypertension in dermatomyositis is very very rare. You can say less than one percent. So, in your case, it is extremely unlikely that you develop pulmonary hypertension in the future.

Try to be in remission. Consider undergoing annual echocardiography and CT scan in two years (if you stay without respiratory symptoms). The medicine that you are taking, Mycophenolate mofetil is anti-fibrotic. So it will also help in managing fibrosis.

I hope I have answered your question.

Let me know if I can assist you further.

Regards.

Medically reviewed byiCliniq medical review team

Published At July 13, 2024
Reviewed AtMay 11, 2026

Same symptoms don't mean you have the same problem. Consult a doctor now!

Dr. Kaushal Bhavsar
Dr. Kaushal Bhavsar

Pulmonology (Asthma Doctors)

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