Pleuroparenchymal Fibroelastosis (PPFE)

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    Andrew Griffiths

    Following on from Helen Davies’ case presentation at the pleural seminar today, I thought that I would share my experience of dealing with a case of┬áPleuroparenchymal Fibroelastosis (PPFE).

    A middle-aged lady was seen in Chest Clinic complaining of malaise and breathlessness. This was her chest radiograph:

    Initially, it was felt that this was in keeping with “burnt out” sarcoid, with upper lobe fibrosis and volume loss pulling up the hila. At this point the FEV1 was 2.27L (88% pred.), FVC 2.54L (84% pred.) and TLCO 65% pred. Follow-up was arranged in the ILD clinic. The serum ACE was mildly elevated on a number of occasions over the following year with broadly stable lung function. A CT wasn’t pursued at this point, as there was a “normal” CTPA a couple of years earlier.

    There was a significant functional decline some fifteen months into follow-up. Lung function had deteriorated with an FEV1 of 1.45L (56% pred.) and FVC 1.64L (55% pred.). Transfer factor was not obtained due to coughing. The patient was offered a trial of steroids, but declined this owing to fears of side effects. Further imaging revealed a left lower lobe collapse, prompting bronchoscopy and a CT. The bronchoscopy demonstrated only mild inflammation. No biopsy or lavage was obtained. The CT revealed subpleural fibrosis and cyst formation which was upper lobe predominant. This was a significant change in comparison to the CTPA three years earlier. Significant mediastinal lymphadenopathy was also noted.

    The patient continued to deteriorate over the coming months. High-dose oral corticosteroids were accepted at this point and brought about a transient improvement in lung function, but her clinical and radiological deterioration was relentless.

    At this point, referrals were made to the Brompton and to our local Pulmonary Hypertension service. The patient was admitted prior to attending these appointments due to hypoxia.

    An inpatient transfer to the Brompton was arranged. They deemed her to be too unfit for a surgical lung biopsy or consideration of transplant. A radiological diagnosis of Pleuroparenchymal Fibroelastosis (PPFE) was made. She was sent back to us for palliation, surviving only a matter of days after transfer. It was quite striking how the long appeared to almost melt away on serial imaging over the course of around ten weeks.

    PPFE is a rare entity. It was included as a rre idiopathic interstitial pneumonia in the latest ATS/ERS Classification. Overlap with sarcoid, or at least diagnostic uncertainty, is observed in the literature. Our local ILD expert was sceptical of the diagnosis of PPFE, favouring aggressive fibrosing sarcoidosis. Certainly, the clinical presentation, mediastinal lymphadenopathy, elevated sACE and partial response to steroids would be in keeping with this. Many features of this case differ to those presented at the pleural seminar today.

    As Dr. Davies mentioned, it’s likely that PPFE is underdiagnosed and awareness of its diagnostic criteria is poor. Such cases highlight the importance of an experienced and up-to-date multidisciplinary team.

    Do others have experiences of managing PPFE that they could share?



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