IPF and Ossification: Who Knew?

Kevin Olson avatar

by Kevin Olson |

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awareness, Fight for Air Climb, ossification, diagnosis

After my idiopathic pulmonary fibrosis (IPF) diagnosis, I was in denial about having the disease. So, I had a video-assisted thoracoscopic lung biopsy in July 2014.

The pathologist said my diagnosis was “fibrosing and interstitial pneumonia with foci of organizing pneumonia with both hyalinization and extensive, somewhat dendriform ossification.”

Yes, reread that. It said bone/calcium deposits were in my lungs — another sharp turn and dive on the roller coaster.

2014 biopsy findings

The pathologist sent the biopsy slides to the Mayo Clinic for confirmation. Following are some comments from Mayo’s biopsy summary:

  • “Thank you very much for the opportunity to review this challenging case.” This comment did not give me a warm feeling about the biopsy.
  • “The sections show an unusual combination of findings.” This was an indication that my IPF was unique to me.
  • “Finally, given the unusual nature of the case, I’d like to keep these slides for our teaching files.” Self-explanatory.

Additionally, the report mentioned stellate and linear opacities in my lungs that were unchanged since 2012. This information was an indirect indication that I had IPF in 2012 but was not diagnosed at the time. 

The summary went into a discussion about usual interstitial pneumonia with dendriform ossification. It also discussed the possibility of dendriform ossification by itself. The consensus was that I had usual interstitial pneumonia with dendriform ossification.

Second opinion

After my biopsy, my pulmonologist referred me to an interstitial lung disease (ILD) pulmonologist. The ILD pulmonologist said he had at most three IPF patients with ossification out of 500 patients. He mentioned that my scarring was in the lower lung lobes, which is nontypical. Also, he barely heard any crackles. 

As I learned about IPF and biopsies, I discovered that the primary method of IPF diagnosis is a high-resolution computed tomography scan. A biopsy was secondary. My thoughts were that I made a mistake having the biopsy. My ILD pulmonologist confirmed I needed the biopsy.

I emailed a former classmate at the U.S. Military Academy at West Point and told him about my ossification. He found that there have been 100 ossification cases since about 1880. When researching for ossification, I found the instances in autopsies. The bad news just kept coming.

Dendriform pulmonary ossification is another rare disease that is most likely diagnosed by surgery or autopsies. There are 1.63 cases per 1,000 autopsies.

Approximately 200,000 people in the U.S. have PF. If you multiple 200,000 by 1.63, and divide by 1,000, you get 326 people who might have IPF with dendriform ossification. This was another indication that I was different from most other people with IPF.

Having ossification excluded me from clinical trials because of my atypical IPF scarring. Research companies didn’t want a stand-alone variant IPF case. Over time, the criteria changed and allowed me to be in a trial, but by that time, I had IPF for too long, which disqualified me for studies. You win some and lose some.

2017 biopsy review

In 2017, a pathologist at the Barnes-Jewish Hospital Lung Transplant Center reviewed my biopsy slides. Some conclusions were:

  • The findings mentioned the appearance of extensive bone within the lung. Additionally, the bone appeared to be present within essentially normal lungs.
  • “There is little or no honeycombing fibrosis present, which would be very unusual in UIP [usual interstitial pneumonia], where honeycombing is typically the dominant feature that is seen.”
  • “The biopsy does not demonstrate the uniform and diffuse fibrosis that one would expect to see in typical cases of nonspecific interstitial pneumonitis (NSIP).”
  • “Represents patchy nonspecific fibrosis associated with dendriform ossification of the lung, as perhaps a stand-alone variant of IPF.”

My conclusion was that I had a slow-progressing ILD. It started at least in the early 2000s. The biopsy was inconclusive about which disease was primary or secondary. Was IPF primary and ossification secondary, or vice versa? Knowing my health history, I believed the ossification was primary, then the IPF developed.

An August 2020 surgery pathology report from Barnes-Jewish Hospital confirmed everything I stated above. It added that secondary pulmonary arterial hypertension had developed.

                                                         General comparison
Symptom Me with ossification IPF patient without ossification
Scarring Little to no honeycombing Honeycombing
Cough None Cough
Crackles in lungs Little to no crackles Crackles
Scarring location Extensively in the lower lobes of the lungs Throughout lungs
Eligibility for clinical trials Unlikely Likely
Treatment Same Same
Prognosis Same Same
ILD classification Does not fit into usual interstitial pneumonia (UIP) or nonspecific UIP (NUIP). In unclassifiable category. It can be classified into an ILD category.

 

I was confident that I have two rare and unique diseases: IPF and dendriform ossification. Now, I was unclassifiable and called Abby Normal.

It bothered me that the cause of my PF was unknown. Previously, the causes of my blood clots and pulmonary embolisms were undetermined. I needed some closure. Finally, my biopsy provided some clues. It helped me to understand my IPF. If you had a biopsy, reread your findings and see if you can find an indicator.

***

Note: 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 other 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. The opinions expressed in this column are not those of Pulmonary Fibrosis News or its parent company, BioNews, and are intended to spark discussion about issues pertaining to pulmonary fibrosis.

Comments

Melissa Kazee avatar

Melissa Kazee

I had a chest x-ray because of shortness of breath on exertion.
I was told I had scarring and collapsed alveoli.
I googled. It gave me pul.onary fibrosis. What I read scarede.

Reply
Jane France avatar

Jane France

My husband died a month ago of pulmonary fibrosis at age 79. He was diagnosed with IPF in December 2019, although minor scarring was observed by his pulmonologist in 2010, unchanged in 2015. He and I had many questions about his 2019 diagnosis, then Covid effectively cut off his access to his pulmonologist until March of 2021. At age 65, he was diagnosed with hemochromatosis, also a rare disease, and was told that, if untreated, the iron could build up in all the organs of his body, especially his liver. He had therapeutic phlebotomy to control it. But he was told by his pulmonologist that it could not have caused or contributed to the IPF. As this article lists, he did not have a cough, and rarely could medical providers hear crackles. He wanted to advance knowledge of IPF, so at his death his body was donated to Northeast Ohio Medical University for medical research. I absolutely agree that more research into IPF is needed!

Reply
Kevin Olson avatar

Kevin Olson

Jane, condolences on the loss of your husband. Praying for you and your family. Yes, more research needs to continue and more awareness. Thank you for sharing.

Reply
Clive Lugmayer avatar

Clive Lugmayer

I like the Young Frankenstein blurb.... I might have my doctor check into this... Having IPF is bad enough.

Reply
Carol avatar

Carol

I have started Ofev on June 15. So far I have taken 3 pills & 2 hrs after I take the pill O feel nauseous & then I throw up. Does anyone else have this problem & if so can u give me some tips on how to avoid this?
Thank you
Carol

Reply
Susan Ferreira Atencio avatar

Susan Ferreira Atencio

I started Ofev in Jan 2021 and had the usual side effects including nausea. I asked my pulmonologist to prescribe
an anti nausea prescription. I take it with my morning pill and has worked very well. I should try stopping the anti
nausea pill to see if I do in fact still get sick. My body may have adjusted to the Ofev. I was very hesitant to start with
Ofev because of all the side effects and risks. I have been pleasantly surprised with my adjustment. ....potential risk factors are still a worry, but that comes with the territory.

Reply
Catherine avatar

Catherine

Dear Kevin, AKA: Abby Normal,
What an interesting journey you've traveled. Thank you for posting such an informative and succinct description of the process you've gone through to get a clear diagnosis. Next, the part you left out: whether ossification continues to grow within lungs, and if so, is there any treatment that can minimize it's progress. In your comparison chart you state that treatment and prognosis are the same for both cases: IPF with and without ossification.
Again. Thank you for sharing your story!
Catherine - Also Abby-Normal - and another ongoing story...
IPF + Irritation (organizing pneumonia) which evolved into non-Hodgkins DLBC Lymphoma - and now with cancer treated and in remission, the scar tissue remains to irritate my lungs. City of Hope in CA and expecting referral to Cedars-Sinai soon.... Wish me Luck.

Reply
Kevin Olson avatar

Kevin Olson

Catherine,

You are welcome.

It is likely my sleep apnea caused aspirations (stomach acid reflux) into my lungs. This caused the ossification deposits in my lungs.

The ossification would continue to develop. Aspirations into my lungs are probably the cause of my ossification. Before and after the transplant, I take pantoprazole and use a CPAP to reduce any aspiration into my lungs.

Also, I was taking Esbriet and also on supplemental oxygen.

I know several persons using a CPAC, taking some GERDS medicine, oxygen, and Esbriet or Ofev.

Praying for you!

Reply
denise anton avatar

denise anton

Dear Carol, I too had the same response to Ofev. I switched to 100mg from 150mg. That did nothing. I was told that my violent retching was unusual. It lasted for 6 mos., and then the side effect abated. I don't take a pill if the timing is off, or if my diet isn't right when taking the pill. I have a 1-1 1/2 hr cushion in the 12 hour spread, and it is very much trial & error on what, and how much, to eat - esp. at night. Best of luck with this crumby illness.

Reply
Diane Beck avatar

Diane Beck

My husband takes Ofev and does not have any issues with it..or maybe minor ones. He has always made sure to eat lots of protein in the a.m. when he takes it. He was going to a Chinese buffet and getting lots of meat, and filling up on it . He has to be up by around 4 a.m. and has to sometimes choke it down that early but he says it is about the only thing he feels like eating since he was told to eat a lot of protein.

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