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    • #29500
      Catherine Leonard
      Participant

      Fibrotic hypersensitivity pneumonitis is a complex interstitial lung disease that is not entirely understood. In its chronic and fibrotic form, hypersensitivity pneumonitis is one of the main mimickers of idiopathic pulmonary fibrosis (IPF). Distinguishing between these two conditions is challenging but is of particular clinical relevance. Two approved therapies are available for IPF, and a considerable number of clinical trials are now exploring newer pharmacological options. This impressive research effort is a consequence of new pathogenetic understanding, updated diagnostic criteria and a long history of pharmacological trials. Conversely, current knowledge gaps on pathogenesis of chronic hypersensitivity pneumonitis, coupled with lack of validated diagnostic criteria, make the management of this disease an unsolved clinical challenge. This also reflects the paucity of therapeutic clinical trials in this field. In this review, we describe the current evidence and the possible future options to approach this complex disease.

      This abstract comes from: https://pubmed.ncbi.nlm.nih.gov/32415523/

      About Me:  So… After over two years of looking for answers and thinking I had a clear diagnosis of IPF and six chemo treatments for Non-Hodgkins DLBCL lung cancer:   the team of doctors at Cedars Sinai in Los Angeles provided me this diagnosis.  They will be treating me with Esbriet, and I am ever so thankful for the topic page on this medication.  It gives me hope, and I feel comfortable with the idea of starting it.  I have so many questions about this ‘immune disorder’ lung disease, which I won’t go into here, but more to the point:  I wonder if anyone else has heard of it, been diagnosed or treated for same?  Lacking any other source for information I will continue to read through information that comes through this PF news source.  It has been a gold mine for me.  Many Thanks.

      • This topic was modified 5 months, 2 weeks ago by Charlene Marshall.
      • This topic was modified 5 months, 2 weeks ago by Charlene Marshall. Reason: formatting to remove code
    • #29504

      Hi @catherine,

      Let me know if you don’t hear from anyone regarding HP — I know a few forum members who have it, and would likely be happy to correspond with you about their experience. I’ll let them speak up first, but if they don’t see this note, I’ll ask to make introductions 🙂
      Char.

      • #29509
        Catherine Leonard
        Participant

        Charlene,

        Thank you.

        For now, I continue to benefit from information available in PF news, and appreciate the way you manage and respond with care and kindness to posts.  I know you are a blessing to many.  I look forward to hearing from folk diagnosed with the enigmatic HP.

        [email protected]

    • #29518
      Donald Salzberg MD
      Participant

      Good Afternoon

      Fascinating stuff. When I turned 63 for the first time in my life I felt sick. I ran my own Ophthalmology practice and NEVER missed a day. All of a sudden I started to have an array of symptoms including palpitations, brain fog, intense fatigue, myalgias (muscle pains), minimal shortness of breath (SOB), rapid heart rate. I was convinced I was like every other MD out there— burnt out and depressed. Four months later i had a sudden onset of SOB and wheezing. I was then told i had pulmonary fibrosis (XRay) and likely have HP (hypersensitivity pneumonitis)!  I was “textbook for HP” but with normal blood work. As i was cancelling 20-25 patients a day and sometimes cancelling surgery i wanted to treat this. HP and IPF are often  fairly indistinguishable on X-Ray and HDCT and PFTs. My pulmonologist was ready to start Prednisone. Not OFEV or Esbriet. I pushed hard for a VATS (open lung) biopsy and guess what—I was found to have IPF. That’s when i started OFEV 36 months ago and my PFTs (FEV and DLCO) have not changed. I still have a lot of the systemic aspects of IPF such as fatigue, altered/distorted sense of smell, cough, mucous/hoarseness. With HP there is often an environmental exposure thing and avoidance of whatever it is (molds, livestock, silicosis, etc) plus a period of prednisone would arrest things. I don’t think they are using anti-fibrotic meds for HP but I could be wrong on that. GERD is a big player in this so treatment for it is important. Treat sleep apnea too.
      Don Salzberg MD

    • #29530
      Marilyn Cellucci
      Participant

      Dear Catherine,

      Did you have a blood test called hypersensitivity pneumonitis profile done? Did parts of it come positive? I’m just curious because mine have just come negative and the pulmonologist originally thought that maybe that is what I have.

      Kindly,
      Marilyn Cellucci

    • #29532
      Donald Salzberg MD
      Participant

      Catherine:

      You state you have a diagnosis of IPF. I am interested in hearing what tests you have had done. Curious as to your age. We’re you tested for lupus? RA? scleroderma?
      don

      • #29544
        Catherine Leonard
        Participant

        Marilyn and Donald,

        Marilyn – re: hypersensitivity profile blood test – Yes. The test was positive for a reaction to Aspergillus fumigates.

        Donald – Since my ‘short of breath’ symptoms were first noted in 2013, with subsequent (numerous) PFT tests, CTs, 4 Bronchoscopies, 2 lung biopsies, and one robotic bronchoscopy.  A nodule, or cyst was spotted in lower right lung, which finally (over one year later) was diagnosed as a Non-Hodgkins DLBC Lymphoma.  This took center stage over general lung disease.  I finished chemotherapy 4/8/21, and believe I am in remission; overall lung condition has gotten worse: fatigue and cough are my main complaints.

        Care providers: I switched from Kaiser to City of Hope for Cancer treatment at the end of 2020, and have sought and received care from Cedars-Sinai for their expertise in Advanced Lung Disease.  As to lab work and blood tests I’ve undergone?  The list would fill pages, and yes: Lupus, RA and scleroderma were ruled out.  Cedars is careful in stating my diagnosis of Hypersensitive pneumonitis is still preliminary, and they  plan to discuss my case with a panel in the next week.  I am thankful for their thoughtful consideration even though I find the diagnosis frustrating since even less is known about the condition and effective treatment of same than is known about PF or IPF.

        This is why I am interested in hearing from others diagnosed with HP regarding recommended care and responses to same.  I have not started recommended treatment Esbriet/Pirfenidone yet, and already I have questions about the ‘consent form,’ something I have never seen before.  I have benefited from posts from folk here who have been taking it.

        Age: 70 – Retired Educator (High School English Teacher).

        Donald: re: prednisone – It worked well for me in reducing cough and shrinking the cyst in early days, and is a part of the chemo treatment I received.  Not sure which is worse or better: Esbriet or prednisone for this condition, although the antifibrotic nature of the former seems to be more beneficial.

        Thank you both for responding.

    • #29545
      Christie Patient
      Moderator

      @catherine My mom’s initial diagnosis was HP. She was suffering from symptoms for 3-4 years before that diagnosis. A year or two later, she was formally diagnosed with IPF. Six months after that, she was in the ICU waiting for a lung transplant. Her IPF progressed somewhat like a ball rolling toward the edge of a cliff. 10-year-old chest Xrays show early signs of ILD (they weren’t looking at her lungs in that case, so they failed to list it as an incidental finding). Each case is different of course, I am not saying this to scare you. Just keep on it and keep saying yes to diagnostic options. The sooner you can get a correct diagnosis, the better you will do with treatment. Wishing you the best,
      Christie

    • #29546
      Catherine Leonard
      Participant

      Christie,

      Sorry to hear that early x-ray ILD symptoms were missed!  I am hoping the lung transplant has and will provide her with many more years.  Thank you for your encouragement and description of your mother’s case, as you say each case is different.  I may not know what the future holds, but I trust in a God who does, and am blessed to be able to receive what I consider high quality care, even if they’re practicing!

      [email protected] / Catherine

      • #29611
        Christie Patient
        Moderator

        Thanks @catherine 🙂 She is doing really well after her transplant. I am glad to hear that you have high quality care and faith to get you through. We’re always here if you need more support.

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