Understanding the Stages of Pulmonary Fibrosis

When a person is diagnosed with pulmonary fibrosis (PF), they may also go through additional tests so doctors can determine the stage of the disease. Staging of pulmonary fibrosis is important to establish the optimal treatment plan and to offer the patient an accurate prognosis.

MORE: Eight of the most common lung diseases in women. 

The treatment and prognosis will depend on how advanced the patient’s pulmonary fibrosis is. This is usually determined by a forced vital capacity (FVC) test using a spirometry device. Patients are instructed to breathe in and then exhale into the device for as long as possible. The spirometry measures how much air a person can expel from their lungs.

An FVC test result of more than 75 percent is regarded as mild PF, 50 percent to 75 percent is moderate PF, 25 percent to 49 percent is severe PF, and less than 25 percent is considered very severe PF.

The current system is not without its limitations. Researchers are working on newer techniques to better stage pulmonary fibrosis so patients can receive the best care possible.

MORE: Using a spirometry device to determine the severity of pulmonary fibrosis.

Pulmonary Fibrosis News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or another qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.

9 comments

  1. I wish we had this to read much soon. My husband’s doctor never really told us what stage he was in, but he must have been close to severe when he was first diagnosed. When we first saw sympthoms we sought medical attemtion and was first told pneumonia, then 2 weeks later bronchitus, and another 2 weeks later pneumonia, but was advised to get a lung scan, which we did. Then he was scheduled for a bronchoscopy & diagnosed with IPF. He took Esbriet, but had trouble breathing from the first. No breathing rehab in our area that we could find and little advice from doctors. Mike died on Valentine’s day 2017 after a miserable year. I miss him terribly, but I am glad he’s finally at peace.

    • Dianne Roncal, DMD says:

      So sorry to hear that, Alice. It is a terrible feeling to lose someone you love and feeling helpless about the whole situation. Know that you are not in this alone and yes, he is definitely at peace, free from all pain. 🙂

    • Betty Lintern says:

      Very sorry to hear of your loss Alice – I was diagnosed in April 2016 – my specialist is “keeping me under observation ” and my GP knows “basically nothing ” about my illness – feeling so alone with this insidious disease !!!

    • Sara Everett says:

      Alice, I am so sorry for the loss of your husband. If it’s possible to ever realize how someone else feels, then I think I come close. I lost my dear husband Bo, on August 10 of this year. It’s almost unbearable sometimes. I cried the way through Walmart yesterday watching people prepare for their Thanksgiving dinners and remembering happy times I the past. My husband had almost the same experience as yours. He was using the VA health system and at the time he and I thought his symptoms might be caused by his heart. His GP sent him to a VA hospital out of town. We thought he was going for cardiac testing but the sent us straight to home oxygen therapy We were shocked! A technician ( the pulmonologist was out sick) used a oximeter on him and said he has intestinal lung disease) . He coudn’t pronounce Interstitial lung disrase. He prescribed oxygen therapy before he ever saw a pulmonologist. He saw the doctor about two weeks later and he said he thought it was IPF, but gave us very little information about it. He said that he just had to get used to the fact that it was what would eventually “ get “ him. He went to two other VA doctors who told us very little. They wouldn’t even talk about a transplant because he was over 65. He was 67 at the time. We decided to find a civilian doctor closer to home and again were given little info. I asked two doctors if there was any respiratory therapy that he could take and was told no, so he never had any. Now I know that it is highly recommended. We never knew how advanced his condition was. He got a lot worse in June and was rushed to the hospital on July23. He spent 17 days in ICU on a ventilator and died on it. They were17 horrible days of suffering. He didn’t deserve that and I wish I could have spared him. His doctors were nice, but I wish they would have put themselves in his shoes a little. Even though it’s an incurable disease. doctors could give patients a little advice on living better with this horrible condition.

  2. Joe Bailey says:

    I used to blow a 14 into the tube when I was working and a fire fighter in the plant where I worked. It changed suddenly after a year. I couldn’t get it to 7. Thus no SCBA for me…Now I’m slowly losing me capacity to breath. At idle ,I’m fine but as soon as I eat; it drops to very little capacity…The say, after a pulmonary test that I have moderate illiactic(sp?) lung disease…I’ve breathed in a lot of junk while working.Nitric/sulphuric fumes; car exhaust; chlorine gas; triclorethyolene solvent…you name it; I’ve breathed it in…quit smoking 30 years ago…Going for more tests. Why just after I eat, even small amounts, does it get so bad…I’m a little overweight but not a lot. It’s the ability to walk after I eat that’s bad…can’t walk 50 feet…without gasping…

  3. Patricia A Harkins says:

    My husband worked as a printer since 1982. He ran the printing press and was subjected to the fumes from the ink used in printing the advertisements. For 16 years prior to retiring he worked at the local newspaper printing the inserts. His press was in the basement, He was instructed to close his door because the fumes were irritating the employees upstairs. He was diagnosed with IPF in May of 2015. I told the doctors about his experience with the fumes, however, they do not connect it to the IPF. Has anyone experienced this situation or had exposure to printing fumes and diagnosed with IPF> Thank you for any advice you can offer.

    • Julie Miller says:

      Hi Patricia
      My Father was diagnosed 6 yrs and is currently at home under Palliative care have took bad in Feb with Heart failure and since his IPF has now worsened and he is basically just waiting. and needs 24/7 care he worked in the print industry as a printers assistant for over 30 years. Not sure I can give any advice but the dust and fibres must have contributed to his condition.

      julie

    • Dianne Bond says:

      I think the union and Workers Compensation Board needs to know. I have reason to fear that this may be an occupational disease because when I visited my husband one night where he worked in pre-press toxic smelling air was obvious as soon as I entered the building, His clothing always had that same smell when he came home from working in that building, and now he is facing premature death.

      When the printing plant he worked in was built we took a tour and got some little plastic plate samples for water based printing, but when the water and plastic process did not work out the plant was used for traditional printing, and then direct to plate. My husband worked in that plant for 13 years, until 2015, when he retired at age 62 as they shut the plant down. The smell of the air and his clothing and the condition of his health may be evidence of improper ventilation or other health and safety reasons for why they had to shut down that particular plant, and I now suspect that this injury to his lungs arose out of his employment.

  4. penny upchurch says:

    i have been told rheumatoid arthritis could have caused mp i.p.f. i had pheumonia in 2014 and was septic with it and in 2017 i was diagnosed with pulmonary fibrosis. my doctor never told me the dangers and out come of this disease,or disorder. after finding this is fatal i went for a second opinion.i now have a good dr. who listened and after proper tests was given oxygen. i now read all i can on this and i appreciate the information and experience of others. i can now prepare for the future with this.i was told when diagnosed with rheumatoid arthritis that it effects other body organs but was not aware that this is a auto immune disorder. this does not mean every one with rheumatoid arthritis will develop pulmonary fibrosis. i was not given details and had i not pushed to find the right dr. i would not have found out i had i.p.f.until it [probably] was in the last stages.i want to say to those who have lost loved ones to this disease,as others have said they are not suffering now and i understand how it hurts to lose those in any death. but they are a future when we will see them again.i am speaking in a biblical promise at John 5;28 revelation 21;4 this gives me hope while dealing with this disease. that life will be given back with no suffering and no sickness. the former things will have passed away.

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