Q. I wonder if you could help me a bit with an unusual case of pneumonia. Our patient is a 56-year-old male with who woke up one morning with dyspnea, fever and chest pain. He came to the emergency department and was diagnosed with pneumonia, put on antibiotics and left to his merry ways. He seemed to be improving over a week until one day the ambulance brought him in with severe dyspnea. Our respiratory team eventually came to the diagnosis of cryptogenic organizing pneumonia/idiopathic bronchiolitis obliterans organizing pneumonia. I’ve tried reading about this but I still don’t quite understand what an “organizing pneumonia” is. Maybe I’m overthinking it and you might be able to shed some light on this.
A. The disease your patient has – bronchiolitis obliterans organizing pneumonia (or BOOP, as it’s more commonly called) – is an uncommon inflammatory disease of the bronchioles and surrounding tissue. It’s sometimes called cryptogenic organizing pneumonia in order to avoid the term bronchiolitis obliterans.
“Bronchiolitis obliterans” is kind of confusing. It’s a term which pathologists use to describe plugging of bronchioles (a fairly nonspecific finding), but which pulmonologists use to imply a chronic, irreversible scarring process resulting in obliteration of small airways (a specific disease often occurring in lung allografts). “Organizing,” by the way, is another fairly non-specific term (meaning that it can be used in several different diseases) used to describe the fibrosis that can develop if a pneumonia persists for a long time.
Back to BOOP. BOOP is a pneumonia, but not an infectious one. By xray, it looks a lot like a severe infectious pneumonia, with extensive, widespread involvement of both lungs. Your patient presents in a typical way for this disease: it looked for all the world like he had pneumonia, but after sending him home on antibiotics, he didn’t get better, and presented again with worsening disease.
The main thing you see under the microscope in BOOP is plugs: little masses of connective tissue rich in fibroblasts that fill the bronchioles and extend into alveolar spaces. Check out the image above: there’s a nice connective tissue plug filling an airway at 7 o’clock. In some plugs, there may be inflammatory cells and capillaries present, giving the lesions a granulation-tissue-like appearance. There is very little, if any, interstitial fibrosis present (a feature commonly seen in other lung diseases, such as usual interstitial pneumonitis).
Although BOOP is not technically an infectious disease, it can be triggered by infectious agents. It can also be triggered by toxic exposures, and it’s more common in patients with autoimmune diseases, like lupus. It can also be idiopathic. The treatment usually involves steroids, and more often that not, patients recover following treatment.
Why the patient wasn’t hospitalized with pneumonia? Is it treated at home ?
@Serge, a patient presenting with bacterial pneumonia without any significant past medical history can be treated outpatient with oral antibiotics, and seek a follow-up appointment if he doesn’t improve after completion of the antibiotics. If he doesn’t get better, or if he gets worse, the patient and physician can work on further workups and treatment.
I have being dealing with Boop for almost 3 years. Since my initial bout,(Jan. 2011,) I am currently experiencing my 6th re-occurrence. My lung specialist says he has 2 patients with Boop(me being one,) that the Boop keeps coming back once weaning off the Prednisone. His other Boop patients recovered completely after the steroid treatment finished. This disease is so rare there is no research or much knowledge of it. My question is “Is it an auto-immune disease? Why do I keep getting it? I want off the Prednisone as it is making my life miserable. Anyone out there with a similar story?
I was diagnosed with BOOP in February 1993. Still have residual damage and am now on Oxygen 24/7. I have been on prednisone of varying doses since 1993. I am currently on 20mg/day and will most likely stay on that. I am looking into my only option which is Transplant. I was only 30yrs old when I contracted this confusing mess.
I had BOOP for 2012. I was on prednisone for 8 months and I made a complete recovery. I know proper weaning of steroids is important. I went to 5 doctors and 9 doctor appointments in 9 weeks before I saw a pulmonologist and got a dx. Just keep trying. I had a good doctor but I know there are some good lung hospitals out there one being the The Jewish Lung hospital in Denver.
Coming off and/or weaning is not the problem. I have been weaned to 5mg/day before, what happens is that I usually start having issues and they have to increase the dosage. I was merely pointing out that while there are people out there such as yourself that recover within a year or so – there are others out there that will not recover fully – I again I have had this since 1993 – 21 YEARS!! My only option is a Lung Transplant. I have had a Pulmonologist in my case since day 1. I worked in a hospital (Oklahoma City) when I became ill, so the medical is not in question. I received the best care available at the time.
Forgot to mention – My diagnosis was via an OPEN LUNG BIOPSY. So there were absolutely no errors in diagnosis.
This is in response to Sharon T and Mary. I think people respond to this monster in varying degrees. I have it now and I am on 20mg of prednisone, down from 50mg. This disease is rare and hard to diagnose, but the prednisone is the killer for me. I am a Type 1 diabetic and my blood sugar is through the roof. Plus all the other side effects – weight gain, personality changes, possible necrosis of the hip, cataracts, thinning of the skin, hair, et cetera. I am thinking of trying macrolide therapy like erythomycian, an antibiotic which has inflammatory properties. I want to get off steroids. It’s killing me, but I am scared this monster will come back to kill me.
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Sorry, I am on 10mg of prednisone and I got the book, BOOP: You’re The Boss. Excellent read by a doctor, researcher, and author who founded this disease in 1985. It’s sometimes called Epler’s Pneumonia. I found out about macrolide therapy or erythromycin from Dr. Epler’s book. It my Bible of Health.
I was diagnosed with BOOP September 2014.
It was very difficult to diagnose at first. I have gone through so much misery being on steroids since it is The only medication that will help.I am presently on 20 mg now.I have been much better but I wish I didn’t have to take prednisone. I worry about weight gain and possible diabetes.
I was diagnosed in 2009 and had a few relapses so far. I just had another a few days ago and are back on 10mg prednisone a day. I have read a lot and my guess is maybe the reason for recurring relapses have a lot to do with initial diagnoses. I nearly died before they diagnosed me because it is such a rare disease and now with scarring of the lungs I think it makes it easier to relaps. Good luck to all out there this is a hateful disease.
Ten years ago I was exposed to some toxic vapors on a construction job site. The next week I was admitted to the hospital where I stayed for 21 days. Biopsy was done. Diagnosed with BOOP. I`ve been hospitalized 8 times since for lung related illness. BOOP left a lot of scar damage in lungs.I`m still under doctors care with 3 month follow-ups and will remain so. Severe BOOP will change your life forever.
My mother was diagnosed in April 2013 with BOOP, she was in and out of the hospital due to reoccurrence. She passed away April 2015 from BOOP.