IPF and Ossification: Who Knew?
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.
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.
|Symptom||Me with ossification||IPF patient without ossification|
|Scarring||Little to no honeycombing||Honeycombing|
|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|
|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.
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