Pleuroparenchymal fibroelastosis

Pleuroparenchymal fibroelastosis (PPFE) is a recently described rare, benign entity. About half of cases are felt to be idiopathic, with the other half secondary to transplantation. Idiopathic cases belong to the group of idiopathic interstitial pneumonias

It is a very rare disorder. Roughly 70 patients have been described in the English-language literature 8, however it is very likely both under-recognised and misdiagnosed 5. Median age at presentation in reported cases approximates the fifth decade of life, with age distribution likely bimodal 8:

  • early peak in the third decade
  • late peak in the sixth decade

​No gender predilection has been reported, and patients are most often non-smokers 2,4,8

Most often significant chronic respiratory symptoms, which may include:

  • non-productive cough
  • dyspnoea, either on exertion or in a worsening course
  • angina
  • frequent airway infections

Signs may include:

  • pneumothorax
  • positive autoimmune titers
  • reduced lung function of mixed (obstructive and restrictive) pattern (reduced forced expiratory in 1st second FEV1, reduced forced vital capacity FVC, reduced diffusing capacity for carbon monoxide DLCO)

The pathophysiology is unknown. PPFE is characterised by predominantly upper lobe pleural and subjacent parenchymal fibrosis (the latter being intra-alveolar with accompanying elastosis of the alveolar walls). 

Aetiology

The aetiology remains unknown. Roughly half of cases occur as sequel of lung transplantation 6,8 or bone marrow transplantation 8,10. About one tenth might even be drug-induced by chemotherapy 8. Familial cases have been reported, especially in young women 8.

Histology

Typical features include abundance of short, curled and randomly oriented elastic fibers, resulting in elastic fibrosis of the visceral pleura. These may be identifiable on standard hematoxylin and eosin (H&E) stains, however elastic fiber stains (e.g. Elastica van Gieson EVG) may be useful in cases of doubt.

Plain radiograph

Chest x-rays may be normal or present non-specific findings comprising:

CT

Optimally performed as high-resolution computed tomography (HRCT) of the lung, it may depict:

Nuclear medicine

Hitherto one case has been described, presenting as hypermetabolic pulmonary nodule on 18F-FDG-PET with associated hypermetabolic axillary lymphadenopathy 7.

PPFE usually shows a progressive clinical course and carries a poor prognosis. There is currently no specific therapy. Medical options with steroids, cyclophosphamide, azathioprine, N-acetylcysteine, azithromycin, sulfamethoxazole and trimethoprim may all be tried. In progressive cases lung transplantation may be required.

Complications

Postprocedural pneumothorax and/or bronchopleural fistula BPF are often seen and may even raise clinical suspicion of this underlying entity 5,8.

For the relatively rare combination of both pleural and interstitial fibrosis may include 5,8:

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Article information

rID: 25734
System: Chest
Section: Pathology
Tag: cases
Synonyms or Alternate Spellings:
  • Pleuroparenchymal fibroelastosis (PPFE)
  • PPFE
  • IPPFE
  • Idiopathic pleuroparenchymal fibroelastosis (IPPFE)
  • Idiopathic pleuroparenchymal fibroelastosis
  • secondary pleuroparenchymal fibroelastosis

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