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Pulmonary Fibrosis

Mixed connective tissue disease/lupus

02/09/2009

Question:

It is my understanding that there is a better prognosis for patients with Pulmonary Fibrosis that is secondary to MCTD/Lupus vs IPF. Also I have developed moderate(PAP 55mmHg) Pulmonary Hypertension in the last few months. How might this affect the prognosis? I am also considered a high functioning patient but on oxygen 24 hrs a day.

Answer:

The course of interstitial lung disease from Mixed Connective Tissue Disease (MCTD) or Systemic Lupus Erythematosus (SLE) tends to be slower than the course of patients with Idiopathic Pulmonary Fibrosis (IPF). There are several forms of interstitial lung disease in MCTD and SLE including

Also, some of the drugs used to treat MCTD and SLE can occasionally cause interstitial lung disease. Each of these forms of interstitial lung disease respond differently to treatment and have different prognoses.

The first step is to determine which of these forms you have. Although this often requires a lung biopsy, it is risky to do a biopsy in patients with pulmonary hypertension so your doctors may need to rely on other tests (such as high resolution chest CT) to establish a presumptive diagnosis.

In general, pulmonary hypertension will make the prognosis worse but it is important to determine what is causing the pulmonary hypertension. Although pulmonary hypertension can arise from the MCTD or the SLE, pulmonary hypertension can also arise from treatable conditions such as blood clots and sleep apnea.

The pulmonary hypertension of MCTD and SLE can be treated with medications that can help but only after other causes of pulmonary hypertension have been ruled out.

For more information:

Go to the Pulmonary Fibrosis health topic, where you can:

Response by:

James N Allen, Jr, MD James N Allen, Jr, MD
Clinical Professor of Pulmonary, Allergy, Critical Care & Sleep Medicine
College of Medicine
The Ohio State University