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Pulmonary Fibrosis

ILD and Lupus

09/11/2011

Question:

I am a 46 yr old female recently dx`ed with lupus. I have had several pulmonary issues going on as well- several nodules ( some ground glass,AV malformations LUL,enlarged PA). I have dyspnea. A couple weeks ago, I began having more dyspnea, waking up at night difficulty breathing, edema in LE, severe joint pain. I saw my rheumatologist who did a CR. It showed fluid in my lungs. I also am having pain in upper back with deep breaths. I had a 6 lb wt gain. He put me on prednisone taper for 6 days and lasix. I saw my pulmo. dr yesterday.He says he feels I have ILD due to the lupus.He said there was crackles in my bases. He also feels there is pleural issues going on as well. He said my HR was high yesterday when listening to my heart. I am having another CT chest next week along with repeating PFT`s. I have a restrictive pattern with decreased DLCO and high CO2 and low O2 on ABG`s. Also have severe resp.muscle weakness with low MIP and MEP ( 18% and 21%). IS ILD common in lupus and what is the usual treatment? I felt so much better on the prednisone. But my sxs are now back.I am scared but hopeful that maybe the progression can be slowed. I am only on plaquenil at this time. Thanks.

Answer:

Systemic lupus erythematosus ("lupus") can have many different types of lung disease associated with it. Some of the more common ones include interstitial lung disease, pleural disease, pulmonary emboli (blood clots), infection, the so-called disappearing lung syndrome (caused by weakness of the diaphragms), and side effects of the various medications used to treat the lupus.

It can often be difficult to sort out the cause of different respiratory symptoms and x-ray abnormalities. Sometimes pulmonary function tests or a chest CT scan are required to determine the specific cause of the respiratory problems. Each of these conditions are treated differently and so arriving at a confident diagnosis is the first step. If the lung disease does prove to be interstitial lung disease, then it can be one of several different types including usual interstitial pneumonitis (UIP) and non-specific interstitial pneumonitis (NSIP).

The NSIP variety is more common and often responds favorably to medications designed to interfere with the immune system such as mycophenylate or cyclophosphamide.

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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