What is pleuroparenchymal fibroelastosis?
Pleuroparenchymal fibroelastosis (PPFE) is a type of pulmonary fibrosis in which dense scar tissue rich in elastic fibres builds up in the pleura (the lung lining) and in the lung tissue just beneath it. It mainly affects the upper lobes, which gradually shrink and pull upwards.
PPFE is listed among the rare idiopathic interstitial pneumonias in the ATS/ERS classification. It is uncommon, although it is increasingly recognised on CT scans, and it can occur alone or alongside other patterns of interstitial lung disease, including idiopathic pulmonary fibrosis in the lower lobes.
Symptoms
- Breathlessness on exertion that worsens slowly over months or years.
- Dry cough.
- Weight loss and a slender build: many patients are thin, and a flattened chest front-to-back (platythorax) is typical.
- Sudden chest pain and breathlessness from a collapsed lung (pneumothorax), which is a recognised complication.
- Repeated chest infections.
Causes and associations
Many cases are idiopathic, meaning no cause is found. Recognised associations include:
- Bone marrow (stem cell) and lung transplantation, where PPFE can develop years later.
- Previous chemotherapy, particularly alkylating agents such as cyclophosphamide.
- Recurrent lung infections, including non-tuberculous mycobacteria and aspergillus.
- Autoimmune and connective tissue diseases.
- Occupational dust exposure, such as asbestos or aluminium.
- Genetic changes in telomere-related genes in some familial cases.
How PPFE is diagnosed
The diagnosis is usually made from high-resolution CT, reviewed in a multidisciplinary discussion. Lung biopsy is rarely needed and carries a higher risk of air leak in PPFE.
| Test | What it shows |
|---|---|
| High-resolution CT chest | Dense pleural thickening and subpleural scarring at the lung apices, with upward pulling of the hila and loss of upper-lobe volume |
| Lung function tests | A restrictive pattern. The residual volume may be relatively high compared with total lung capacity |
| Gas transfer (DLCO) | The degree of impairment in oxygen uptake |
| Sputum and bronchoscopy samples | Infection such as tuberculosis, non-tuberculous mycobacteria or aspergillus |
| Autoimmune blood tests | Evidence of an associated connective tissue disease |
| Six-minute walk test | Exercise capacity and oxygen levels on exertion |
In India, upper-lobe scarring is often assumed to be healed tuberculosis. Previous TB should be confirmed rather than presumed, and active infection excluded with sputum or bronchoscopy samples before PPFE is diagnosed.
Treatment
Antifibrotic therapy
There is no treatment proven to reverse PPFE. Where the disease is progressing, the antifibrotic drug nintedanib may be considered, as it slows decline in progressive pulmonary fibrosis of various types. Steroids and immunosuppressants are generally of little benefit unless a coexisting autoimmune process is active.
Nutrition and general care
Weight loss is common and is linked with worse outcomes, so dietitian support is important. Pulmonary rehabilitation, vaccinations against influenza and pneumococcus, and prompt treatment of chest infections help maintain function.
Managing pneumothorax
Collapsed lung is a recurring problem and may be slow to heal. Management ranges from observation to chest drain insertion and, in selected cases, surgical or medical pleurodesis, planned with thoracic surgery. See pleural disease for more on pneumothorax care.
Oxygen and transplantation
Oxygen is prescribed when levels fall. Suitable patients with progressive disease are referred for lung transplant assessment, although the chest wall changes and low weight can make transplantation technically challenging.
Outlook and follow-up
The course of PPFE varies. Some people remain stable for years, while others decline more quickly, particularly after transplantation or when lower-lobe fibrosis coexists. Follow-up every few months with lung function tests, weight checks and periodic CT helps identify progression. Patients in Hyderabad are reviewed at Apollo Hospitals, Financial District, with input from thoracic surgery and transplant teams when needed.
When to seek urgent care
Seek emergency care for sudden one-sided chest pain with breathlessness, which may indicate a pneumothorax, as well as for rapidly worsening breathlessness, high fever, coughing up blood, or confusion or bluish lips.
Frequently asked questions
Is PPFE the same as old tuberculosis scarring?
No. Both cause upper-lobe scarring and are often confused, especially in India. PPFE has a characteristic CT appearance and a different course. Active infection should be excluded with proper tests rather than assumed.
Why have I lost weight?
Weight loss is a common feature of PPFE, partly from the extra effort of breathing. Maintaining nutrition with dietitian support is an important part of treatment.
Why do I keep getting a collapsed lung?
The scarring at the top of the lungs makes air leaks more likely and slower to heal. Some patients need a chest drain or a procedure to prevent recurrence.
Are there medicines for PPFE?
No drug has been proven to reverse PPFE. Nintedanib may be considered when the fibrosis is progressing, and supportive care with rehabilitation, nutrition and oxygen is important.
Can PPFE follow a bone marrow transplant?
Yes. PPFE is a recognised late complication of stem cell transplantation and of some chemotherapy drugs, and can appear years later.
Is a lung biopsy needed?
Usually not. CT findings reviewed by a multidisciplinary team are generally enough, and biopsy carries a higher risk of air leak in PPFE.
Related conditions and procedures
Consult Dr. Gogineni Ratnakara Rao
Apollo Hospitals, Financial District: Monday to Saturday, 8:30 AM to 5:00 PM. Anything in Lung Clinic, Chanda Nagar: Monday to Saturday (Thursday closed), 6:00 PM to 9:00 PM.