Monday, May 2, 2016

What could have caused my Pulmonary Fibrosis?

I have noticed that many people living with PF are frustrated by the scientific and medical community's lack of understanding about the causes of PF. I thought I would take a moment to let you know what we DO and DON'T know about the causes of PF. I will also help you try to figure out the cause of your PF (if a cause is known for your kind of PF).

Idiopathic forms of PF

First, let's address the frustrating "idiopathic" types of PF. We use the term "idiopathic" to describe disease without a known cause. As many of you know, "idiopathic pulmonary fibrosis" (also known as IPF) is only one of the many types of PF for which we don't know the cause. Doctors group these diseases of unknown cause into a category called "idiopathic interstitial pneumonias." There are many of them. I am not going to go into detail about each one. But, I will tell you two pieces of (hopefully) useful information.

(1) Many of these conditions are named after the appearance of your lung tissue under the microscope. Importantly, many of these lung biopsy appearances (which we call "patterns") can be seen in other types of PF of known cause (see later on about the "interstitial lung diseases of known cause or association.") So, just because your biopsy shows "NSIP" doesn't necessarily mean that you have idiopathic NSIP. You might have an "interstitial disease of known cause or association" with an NSIP pattern on your biopsy. Read on and then talk to your doctor for more information.

(2) All of the "idiopathic interstitial pneumonias" require a surgical biopsy of the lung to diagnose, with two exceptions: idiopathic pulmonary fibrosis and unclassifiable idiopathic interstitial pneumonia can in some cases be diagnosed without a biopsy.

Here is a list of the idiopathic interstitial pneumonias (and their abbreviations):  

Major types:
  • Idiopathic Pulmonary Fibrosis (IPF)
  • Idiopathic Non-specific Interstitial Pneumonia (idiopathic NSIP)
  • Respiratory Bronchiolitis-interstitial lung disease (RB-ILD)
  • Desquamative Interstitial Pneumonia (DIP)
  • Cryptogenic Organizing Pneumonia (COP)
  • Acute Interstitial Pneumonia (AIP)

Rare types:
  • Idiopathic Pleuroparenchymal Fibroelastosis (IPPFE)
  • Idiopathic Lymphoid Interstitial Pneumonia (Idiopathic LIP)

Unclassifiable Idiopathic Interstitial Pneumonia (Unclassifiable IIP)

There are other types of PF of unknown cause too. For example, pulmonary sarcoidosis and a number of more rare types of lung disease would also be called "idiopathic", even if they are not included in the "idiopathic interstitial pneumonia" category.

Interstitial Lung Disease of Known Cause of Association
There are a handful of types of PF for which we do know the cause – or at least we understand what might have triggered the disease. We call these diseases "interstitial lung diseases of known cause or association" (I know, ridiculously long).  Here are five major categories of "interstitial lung diseases of known cause or association"
  • Medication-induced
  • Radiation therapy-induced
  • Autoimmune disease (also called Connective Tissue Disease-ILD)
  • Hypersensitivity pneumonitis (Environmental causes)
  • Pneumoconioses (Occupational causes)

If you have been diagnosed with PF (or ILD), you and your doctor can work together to see if you have one of these "interstitial lung diseases of known cause or association." It's not too difficult, and you can start the process by asking yourself a few questions. I'll share with you the questions I ask my patients to sort out possible causes of their PF:


"Have you ever taken a medication to slow a fast heartbeat? Have you ever had atrial fibrillation ("A Fib") or had your heart shocked to stop an abnormal heart rhythm? Have you ever taken a medication called Amiodarone or Cordarone?"
            Amiodarone (Cordarone) can cause ILD.

"Have you ever had urinary or prostate infections? Have you ever taken an antibiotic called nitrofurantoin, Macrobid, or Macrodantin?"
            Nitrofurantoin can cause ILD.

"Have you ever taken methotrexate or received chemotherapy?"
            Methotrexate and chemotherapy can cause ILD.

"Are there other medications you've taken for long periods of time in the past?"
            Lung disease due to medications that you either take currently or have taken in the past can be looked up at Be warned: Many of the cases of lung disease on are NOT caused by medications – but when people develop a lung disease after taking a medication, doctors scratch their heads and wonder if the drug could have been responsible for the lung disease – but they can't really prove the link. Be sure to discuss your medications with your health care provider before you make any changes to your medical regimen – seriously, you could be harmed by stopping your medications.


This one is usually pretty obvious. Any history of radiation treatment to the chest for breast cancer, lymphoma, or another type of cancer can cause PF.

Autoimmune disease

There are a variety of autoimmune diseases that can cause "Connective Tissue Disease-associated Interstitial Lung Disease" (also known as CTD-ILD). "Auto" means "self" – so autoimmune diseases are conditions in which your immune system attacks tissues and organs in your body. This can lead to inflammation, injury, and scarring in different organs. The lungs are often involved.

In my experience, the most common autoimmune diseases that cause PF are:
  • Rheumatoid Arthritis (this is different and less common that Osteoarthritis)
  • Scleroderma (now called Systemic Sclerosis)
  • Polymyositis
  • Dermatomyositis
  • Anti-synthetase syndrome
  • Sjögren's Syndrome.

Occasionally other types of autoimmune disease (such as Systemic Lupus Erythematosis, or "lupus") can cause PF. Certain types of blood vessel inflammation diseases (called "vasculitis") can also cause PF. Some people even develop an "idiopathic interstitial pneumonia" due to "autoimmunity" but without developing one of these specific autoimmune diseases. We now use the term "interstitial pneumonia with autoimmune features" (or "IPAF") to describe this condition. Many of these folks have "NSIP" on their biopsy (see above section on "idiopathic interstitial pneumonias") and can also (correctly) be diagnosed with idiopathic NSIP.

In addition to asking my patients if they have ever had one of the autoimmune diseases listed above, I ask my patients the following questions to sort out if they might have an autoimmune disease:

"Do you have pain, swelling, or stiffness in any of your joints?"
"Are your hands stiff when you wake up in the morning? How long does it last?" (an hour suggests autoimmune disease)
"Have you noticed any thickening or tightening on your fingers, hands, or neck?"
"Have you noticed any rash on your face, chest, or arms? Does it occur in areas exposed to the sun?"
"Do you suffer from heartburn? Do you occasionally have food come all the way up to your throat?
"Do you suffer from dryness of the eyes?"
"Do you suffer from dryness of the mouth?"
"Do your fingers sometimes turn white or red? Does cold weather make this worse?"
"Do you suffer from pain in your muscles in your arms or legs? How about weakness?"

Now, many of us have had one or more of these symptoms – so don't start to worry that you have an autoimmune disease because you've had some joint pains or a rash. But definitely talk to your health care provider about any symptoms you are experiencing --- your doctors can help you determine if you have an autoimmune disease.

Hypersensitivity Pneumonitis

If you break down the term Hypersensitivity Pneumonitis, you can see that it means your lungs are "too sensitive" to something. Usually the "somethings" are proteins from a living thing that you are breathing in – the most common culprits are proteins from mold spores, bacteria, and animals (particularly birds living indoors). Breathing these proteins triggers inflammation in the tiniest little airways in your lungs, followed by inflammation in the walls of the air sacs, and in some cases this leads to scarring.

Hypersensitivity Pneumonitis is a long medical term – let's call it "HP" for short. In my practice, I see a LOT of HP – about 25% of my patients who have any kind of PF have HP (another 25% have autoimmune diseases, and 25% have idiopathic pulmonary fibrosis). There are 3 critical tests that help doctors figure out if you have HP as the cause of your PF:
  • Asking the right questions to identify a possible exposure to proteins you are breathing in (see below)
  • Listening carefully to the lungs for a special kind of lung sounds (called an "inspiratory squeak") – Squeaks sounds just like you'd expect. Squeaky. Ask your doctor.
  • Getting the right kind of CAT scan – in addition to the usual CAT scan performed when you take a deep breath and hold it, a 2nd CAT scan performed after you breath out is also important. With this kind of CAT scan, your doctor can look for "gas trapping," or pockets of air that get trapped in different parts of your lungs when you breathe out.

Here are the questions I ask my patients to figure out if they have HP:

"Do you have a forced air heating system in your home? In a prior home? In a second or vacation home? In your workplace? If so, how long have you been exposed to it? Does it have a humidification system? Has it ever been contaminated with mold?"
"Have you ever had any water in your basement or another part of your home? Have you ever had water damage in your home?"
"Are you aware of any mold in your home (outside of your shower)?"
"Do you use a hot tub on a regular basis?"
"Do you use a humidifier in your home?"
"Do you have any birds that live in your home currently or in the past? Do you spend time in someone else's home where birds live? Do you take care of birds in cages or coops currently or in the past?"
"Have you been exposed to renovation or construction?"
"Have you been exposed to farming or an agricultural setting?"
"What hobbies do you have?"

Remember – just because you might have been exposed to one of the above does not mean it was the cause of your PF. Lots of us have had water in our basements at some point. But be sure to bring this up with your doctor.

Pneumoconioses (Occupational causes)

Pneumoconioses are types of PF that result from exposures in the workplace. Here are just a few examples:
  • Asbestosis (due to asbestos exposure)
  • Coal Worker's Pneumoconiosis
  • Silicosis
  • Berylliosis
  • Hard Metal Lung Disease
  • (there are many others)

I usually ask my patients about their current job and all prior jobs. I also ask whether you ever worked in the military (particularly with exposure to Navy shipyards or dusts, fumes, gases, vapors, or chemicals).

I also ask if you've ever had a job where you were exposed to dusts, fumes, vapors, or gases that you might have breathed in. I also ask about the following exposures:
  • Asbestos
  • Mining
  • Excavating
  • Coal Work
  • Foundry Work
  • Sand Blasting
  • Stonework
  • Ceramics
  • Abrasives
  • Heavy metals
  • Factory work

Please talk to your doctor if you've had a job where you might have been exposed to one or more of these things.

If you do have an "interstitial lung disease of known cause or association," it is important to figure this out. We can sometimes treat you based on the factors that are triggering your disease:
  • Remove exposure to harmful medications
  • Treat autoimmune diseases (usually with a Rheumatologist)
  • Remove exposure to mold, bacteria, birds, etc.
  • Remove exposure to occupational triggers

If you have PF of unknown cause, please talk to your doctor to figure out if you might have an "interstitial lung disease of known cause or association."

You can always get more information about PF and where to get medical care from the Pulmonary Fibrosis Foundation:


  1. Coincidental to my being DXed with cHP I stopped taking Hydrochlorothiazide, which I had taken for several years. After my VATS BX, I mentioned this to my local pulmonologist and my MN Mayo pulmonologist. They just shrugged their shoulders, taking no stand. I understand that it cannot be proven. But I am convinced of Hydrochlorothiazide being my trigger because since I stopped taking it and was DXed within a month's time, I have remained stable. (4 years) I indicate Hydrochlorothiazide on all medical histories as a 'drug sensitivity'. Your thoughts?

    1. Always hard to know whether a particular drug might have contributed to ILD (or any other disease). On occasion, hydrochlorothiazide has been suggested as a trigger of lung disease, but these reports cannot confirm that the drug was the culprit. Possible.

    2. After a couple years and talk of a lung transplant, I came to Portland, OR and the pulmonologist said it was most likely from a medication for UTI, macrodantin, and put me on high dose prednisone, got it reversed and I have been good since 2003.

    3. I have pf and pbc with sjogrens. After over a decade of hctz use i developed pf and when i got really sick and started coughing up clear fluid i quit everything. my pf became stable like old scarring. Then i restarted one by one with hctz last. it was the hctz. recently i was given maxide and didnt know it hctz in it and became really ill. i started coughing up fluid and realized what was goinv on and quit. now my xray shows that my pf progressed and i have to get ct scans and all. for me there is no doubt hctz causes it as could any sulfa drug is you are sensitive. Also I got or have worsened myasthenia gravis from plaquenil. i have many drug sensitivities due to my liver. now i have these. I believe pbc is underdiagnosed and its triggered from chemicals in almost every health and beauty item.

  2. This is an outstanding article. You might also note if someone has a biological family member (e.g., parent, sibling, aunt/uncle) who has had the disease, which could have predisposed them to it, and then the disease might have been triggered by one of the influences noted above.

    1. Excellent point, Linda. I always ask my patients if they have ever had a family member with scarring in the lungs, or someone who was on oxygen at home.

    2. Very informative article. My husband died December 2014 of IPF, his Father died in 1990 aged 76 he had been diagnosed with Sarcoidosis and was on oxygen at time of his death. Would there be any connection between Father and Son having these 2 types of lung diseases.

    3. Hi Theresa. I am sorry to hear about your husband's passing in 2014. There are a number of possibilities here. Your father-in-law might have had IPF and was labeled as "sarcoidosis" in 1990. Or it is possible that your husband and your father-in-law shared one or more genes that predisposed to scarring in the lungs -- we really don't know enough about these diseases to be certain.

  3. Well done David. One of the best laid out discussions on a very complicated disease. I shall refer people to this constantly as I do many of your blog posts.

  4. Great explanation of a super complex topic. Thank you.

    1. Thanks, Noah. And thanks for your help with my patients.

  5. Many thanks for clarifying this mysterious disease! My sister passed in 2014 from it; my brother was diagnosed with the disease in his early 60's and passed in 2011 from complications related with it. There is a history of autoimmune related diseases in my family, so I want a more thorough check of my lungs to rule out any chance I may have it.

  6. Thank you for a very clear and concise article about IPF. My husband was diagnosed in 2007 with IPF and HP. Our insurance at the time required the biopsy to be able to participate in pulmonary rehab and to use oxygen - so that is how the final diagnosis was made. In looking back, we feel that his exposures to benzene, trichlorethylene, and JP-4 jet fuel - all of which were mixed together in a big barrel and used to wash aircraft - are the cause for his illness. My husband was assigned to the wash rack at the base he was assigned was before OSHA and there was no protective gear (no suiting up, no respirators to protect the lungs) he'd spend upwards of 12 hours a day washing aircraft, and being soaked in this stuff and inhaling it. We know there is no way to prove that is the cause...but gut instinct is that we are right. And we found some some articles out there tying those specific chemicals to diseases that come under the interstitial lung disease umbrella. My husband remains relatively stable thus far - we are thankful for that.

  7. David, I assume that PF related to Saroidosis falls under Autoimmune Diseases?

    1. Sarcoidosis seems to be a unique disease. No one really understands the causes of sarcoidosis beyond a few genetic and environmental risk factors that do not seem to account for the vast majority of cases of sarcoidosis. While autoimmunity has been implicated, it is generally not considered an autoimmune disease in the same sense as the diseases I have outlined above. Some theories point toward infectious causes. Others to environmental exposures. Really unclear despite some high quality research in the past 2 decades.

  8. My wife got both diagnosis of Systemic Scleroderma and Dermatomyositis and thanks to the Antibiotic Treatment her IPF cleared up 100% and no longer on oxygen. There is definitely hope when the medical establishment gives up.

  9. Wow, this is the most informative article I've read yet on ILD. I live in Minnesota. I was diagnosed a year ago with ILD secondary to Crohn's Disease. I've had Crohn's since the mid-1990's and have had 4 surgeries for it. I also have psoriatic arthritis, psoriasis, and Sjogren's Syndrome. My pulmonologist ruled out methotrexate as a cause for my ILD after I was off it for two months. She also ruled out the Sjogren's because of the lung biopsy results that revealed Crohn's granuloma cells in my lungs. Now, when my lungs act up, I also have GI symptoms! For the ILD, I'm taking Cellcept. Cinda

  10. What about Systemic lupus erythematosus (SLE)?

    1. SLE is definitely an autoimmune disease. SLE does not frequently cause ILD. The vast majority of people that I've seen who have both an autoimmune disease and ILD have either rheumatoid arthritis, scleroderma, Sjögren's syndrome, or an inflammatory muscle disease (such as dermatomyositis). Very uncommonly SLE.

  11. Hi David,
    What about formaldehyde...cadaver dissection 1954; histoplasmosis...1940-50; significant GERD Rx w Prilosec...2000-2010; droperidol/Diprivan/mother's milk ...1996, 2010; Radon?,,,as potential causative agents?

    Accuracy of current state of the art radiologic to call it IPF?

    "Fun" stuff (not).
    Gardner Rhea

    1. Hi Gardner. Pulmonary histoplasmosis can cause some lung scarring. GERD has been linked to pulmonary fibrosis (although far from a proven link). The other items you mention have not been linked to pulmonary fibrosis to my knowledge. As to the accuracy of our current state of the art of diagnosis -- we have some rules we follow, but we are only in the infancy of our understanding of these diseases. Sorry to hear you are dealing with this disease. Best regards.

  12. I am so grateful for you, Dr. Lederer and all your priceless work. This is the best snapshot of the whole pie relating to PF. After attending the 2015 summit, my husband went to center of excellence and found his original diagnosis was incorrect after 5 yrs diagnosis with mild sob on exertion. Latest scan showed slight progression with one smallhoneycomb area? Tests show PF with high antigens of mold. If the mold types that came back are above normal, could they be the cause. Drs. Want a biopsy and think that will give conclusive or at least strong percentage. Do you agree and does it matter what part of the lung is biopsied. I suppose for hp the only treatment would be steroids and are they effective in staving off disease? Thank you so much!!

    1. Hi there. It sounds like your husband is getting excellent care. I wish I had answers for your questions, but since I don't have all of the medical information about your husband, I cannot answer accurately. These are good questions for your doctor (I know you know that). All the best.

    2. We did biopsy-no IPF, one time scarring and a little Emphasema with no progression! Can't believe the good news!!

    3. Thanks to the 2015 summit and COE we are now living a miracle compared to 5 years of dread which was a misdiagnosis! Hope others find positive news with COE!!!

  13. Dr. Lederer, the forced air heating issue is huge for my mom, who has IPF and now has developed lung cancer as well. Every year once the forced air heating system went on in their small home, she came down sick (and this was years before the IPF diagnosis). Now she lives in a large building and it too has forced air heating, and she has gotten sick there as well once the heating is used. These are large panels in each room where the heat comes out. My dad is trying to figure out if some kind of padding or filtering could be installed. I was wondering about hypersensitivity pneumonitis. She has so much else going on right now, but your post did a service by addressing forced air heating. It's summer but this is already something we are worried about for this winter. However long my mom can survive the IPF and lung cancer, the forced air heating is definitely an issue. With everything else a patient in my mom's situation has to deal with, forced air heating is a practical issue that is also significant. Debra

    1. Hi Debra. I am sorry to hear about your mom's situation. I strongly recommend that you and your mom talk to your mom's pulmonologist about the possibility of hypersensitivity pneumonitis and whether it would make sense to have your mom's home inspected for mold. If mold is contributing to her lung disease, then it is possible that removing the mold (through "remediation") could have a positive health effect for your mom. But -- please don't do anything until you receive guidance from your mom's doctors. You could also seek another opinion at a Pulmonary Fibrosis Foundation Care Center:

  14. Dr Lederer, I am 68 yrs old and until March /2015 was in excellent health. Got very bad bilateral pneumonia, hospitalized 2 weeks, treated with anti bacterial intravenous and tablet,lost 20 lbs and at release could not walk more than 10 feet. Told I would be better in 4 weeks I suffered for 3 months without any treatment from family doctor. Luckily I discovered that ibuprofen (2400g/day) allowed me to breathe and I slowly improved.i was finally seen by specialist after 3 months who did cat scan and diagnosed with bird lung. We had a cockatiel for 17 years! Got rid of bird , started prednisone at 50 mg, regained strength and went back to work in Sept. By Feb this year I was off prednisone but still suffer occasions of shortness of breath. I did a lot of wood and stone carving during the last 30 years.
    I exercise regularly , eat healthy and have never smoked although my wife smoked until I got sick. Although I have never been told I have anything other than "bird lung" or HP, it would appear that I have ILD or IPF and will experience slow or fast deterioration of lung function. What are your thoughts given this limited background information? I attended a meeting at St Pauls hospital in April and met wonderful people there and plan on returning.

    1. Hi Larry. Thanks for sharing your experience. If you doctor's diagnosed you with "bird lung", there is a chance that you do have hypersensitivity pneumonitis -- you should definitely ask your doctors about this possibility. You should know that there are different forms of "HP" -- including reversible and treatable forms, and some that do not have any fibrosis. please consider seeing a pulmonologist who specializes in interstitial lung disease, who can help in a more substantial fashion. All the best.

  15. I have been dx'ed with scleroderma, Sjogrens, PF amongst other problems. I haven't been told whether or not my PF is due to my autoimmune diseases or it is idiopathic. I'm assuming it is sclero and Sjogrens related. My question is, do the medications available for IPF work for those of us that have an autoimmune fibrosis? If not, why? Thank you so much. This was an awesome article.

    1. Hi Mary. Thanks for your question. We really don't know whether the new drugs for IPF (pirfenidone and nintedanib) help people with other kinds of PF. There was one study that was just published (link below) in which the investigators have pirfenidone to people who had both scleroderma and PF. There was no "placebo" group, so they couldn't common on whether or not the drug worked --- but pirfenidone seemed to be tolerated reasonably well. I should point out that in the countries where pifenidone and nintedanib are approved, they are only approved for IPF, not for other kinds of lung disease. In my practice, I do not prescribe either medication for other kinds of PF (only for IPF). As more studies are carried out, hopefully we will find out that these drugs (and perhaps others) do help people with other forms of PF. Here is the link:

  16. Doctor Lederer, how is the blood test for HP panel called? We made one but cant see any alergens in it , only abtibodies for artrites, birds and antibodies for connective tissue disease. I thought there should be a lot more

    1. The blood test is called a "hypersensitivity pneumonitis panel." There are a number of different tests available at different labs. While this test can sometimes be helpful, we see a lot of people with "false positive" results (positive test result when they don't have HP) and "false negative" results (negative test result when they do have HP). Any test result has to be interpreted individually for each person.

  17. I commented but looks like it didn't take. This is a good post thanks Dr. Lederer. Every since I was diagnosed with IPF I always wanted to know what the "I" was. I thought that the biopsy tissue was tested for various possible causes based on the answers I gave the doctor to the environmental questions. Now years into my disease and researching I believe that is not the case, that the slides are looked at for a common pattern and that pattern showed no known cause. I still wonder what the "I" is. I think that if the slides, tissue was tested and a cause was given that maybe there would be a treatment more appropriate to what Fibrosis I do have. I spoke to a post-transplant patient who said he requested and paid to have his own lung(s) sent out to determine the cause, and he was given that cause. Is that the only time we would be able to determine the nature of our disease? While in some of us the outcome won't change or treatment need but I would think, upon transplant or biopsy that more could be done to determine and maybe help and possibly assist with a cure for some of us

    1. Hi Laura. Often the major test performed on lung tissue from a biopsy or from a transplant is a visual one. A pathologist uses various stains (dyes) to color different cells and tissues with different colors. Then the pathologist looks at the "pattern" of disease under a microscope. Additional tests are usually not performed, other than looking for bacterial and other infections. Doctors use the information from a biopsy to help determine the nature of the disease, but doctors also use much more -- that's why we ask so many questions, examine you carefully, and perform a CT scan and blood work. It's the combination of all of this information that helps us determine the nature of your disease. I hope that is helpful.

  18. Thank you for your informative article. My dad was diagnosed in July 2013 with PF and passed away March 2015. Initially he it was moderate and stable, then developed pneumonia (which he had a few times in the passed) and was found to have the nasty bacteria pseudomonas. Because he was on immunosuppressant drugs for a kidney transplant 19years previously, he couldn't fight it. He was also on warfrin for a heart valve replacement. He was in ICU for 2 months on a roller coaster ride. There were times it looked like he was improving, then would spike a fever, white blood cells would sky rocket.
    He was a light smoker for many years also.
    Do you have any thoughts as to what may have caused his PF?
    Medication? Smoking? History of pneumonia? Combination of all?
    Just a little concerned if hereditary.
    Thank you
    Sia (Melbourne)

    1. Hi Sia. I am sorry to hear about your dad. It sounds like he went through a lot - kidney transplant for 20 years plus PF in the last few years. I hope he didn't suffer in his last days. The ICU can be a difficult place. It is really hard for me to answer your question -- there are many many causes of PF that I discussed in this blog post, and any one or more (or none!) of these may have contributed to your dad's condition. But there's no way for me to know. I can also understand your concern that the risk of developing PF could be passed down from your dad's genes. Most people with PF in the family will never develop PF. Always a good idea to make sure your doctor knows that your dad had PF. your doctor might listen to your lungs to see if there are any abnormal lung sounds that suggest PF. There are no recommendations by any medical society to do genetic testing or any other tests (like CAT scans) in people whose parent had PF. I hope that information is helpful.

    2. Thank you so much, there isn't much information on PF here in Melbourne, and enjoy reading your posts.

  19. One thing not mentioned is that a small number of people may have multiple causes of fibrosing lung disease.

  20. One thing not mentioned is that a small number of people may have multiple causes of fibrosing lung disease.

  21. Dr. Lederer,

    If there is a way to identify what caused PF why are patients that know how they got the disease excluded from clinical trials? There are many clinical trials for IPF and not any for patients with PF.

    1. Great question. A lot of time has passed since you posted your question (sorry!). Since then, a number of clinical trials have begun that are enrolling people with other forms of PF. Here is one example:

  22. Im 39 year old and i had a cough for about 4 months, xray was performed. X ray revealed mild pulmunary fibrosis and hyperaetion of lungs. Is x ray accurate to determine a pulmunary fibrosis? Im very frighthen at this moment , i havent slept since i saw the x ray report. I havent seen a pulmunologist up to this date. Please advice. Thank you

    1. Hi. Thanks for reaching out. An x-ray can suggest PF, but it cannot confirm PF. A CAT scan is the test that pulmonologists use to confirm PF. Sometimes a biopsy of the lung is needed. If you live in the US, consider finding a medical center that specializes in PF. A team there can help sort things out.

      All best wishes to you.

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