Non Specific Interstitial Pnuemonitis

[carefreesf]

I'm not sure if there is a question in here. My mother, who recently turned 58, was diagnosed with Non Specific Interstitial Pnuemonia (pneumonia) after an open lung biopsy.  I know NSIP means no known cause.  Tests showed no connective tissue disease and we've also tested her for her parrots. Her PFT's were marginal at best and she has been on oxygen therapy basically 24 hours a day since being hospitalized back in February of 2001. Her Pulmonoligist put her on 60 mgs of Prednisone to start and she stayed at that for about two months and then was lowered to 50 and eventually 40 mgs a day where she has been at since around late August. Her PFT's have not shown marked improvement since beginning therapy and all the predinisone is beginning to takes its toll not only physically but emotionally as well.  (i.e.  weight gain, hair growth, body changes)  She recently suffered a compression fracture in her back that has caused a setback in her physical therapy. She lives in New Mexico at an altitude of 4000 ft.   I live in San Francisco and she has been staying with us for a couple of weeks and the improvement is dramatic.  I am able to take her to the pool for exercise and her 02 sats remain above 90 for the most part on about a flow rate of 4.
I want to be doing all I can to help her but wonder if her Dr is taking a proactive stance.  Staying at 40 mg of Prednisone for about 5 months now seems a bit excessive.  He provides no real plan of action in hopes of getting her off oxygen.  I want to be realistic about her future but guess I need advice.

Thanks,   Cary

[National Jewish]

Non-Specific Interstitial Pneumonitis is a form of Interstitial Lung Disease (ILD). With ILD swelling leads to scarring of the lungs in the area between the air sacs and the blood vessels. Prednisone® is an oral steroid that helps to decrease this swelling for some people, but not for everyone. For some people it can take at least 6 months of daily Prednisone® to see symptoms decrease.
As you have seen in your mother, side effects such as osteoporosis are possible. Osteoporosis can lead to fractures of any weakened bones including those in the back. Bone density is useful in watching for osteoporosis and checking for changes in osteoporosis. Please check with her doctor about watching for side effects and steps to take to lessen the side effects. Sometimes adding other medications makes it possible to decrease the Prednisone® dose and still decrease symptoms.
With ILD the oxygen in the blood may be low. When this happens supplemental oxygen is needed. Being at sea level may improve the oxygen level so that the supplemental oxygen is not needed.
Pulmonary rehabilitation conditions the entire body so that the muscles are working as efficiently as possible. This allows the breathing muscles to use the least amount of energy to move air in and out of the lungs.
Please contact Lung Line® to see if there is a lung specialist on our referral list in your area if you would like to pursue a second opinion. For further information, please read our Interstitial Lung Disease MedFact at http://www.nationaljewish.org/medfacts/interstitial.html. You are welcome to share this information with your mother’s doctor.

[carefreesf]

just a follow up to my previous.   The histological diagnosis was NSIP but the doctors here at SF General feel as though its Hypersensitivity Pnuemonitis.  We had tests for her parrots done against her blood and found no connection.   Might there be another external environmental possibility?  She lives in Eastern New Mexico that does have a lot of wind/dirt as well as dairies that are sprouting up left and right all over the place.  She also has a swamp cooler (evaporative).  We have had that cleaned as well as pulled up all the carpets in the house and did a thourough cleaning of all fabrics, etc.

[band]

What tests for autoimmune diseases did they do? What was her ANA titer?

[band]

What tests for autoimmune disease were done. What was the ANA titer?