The information on this website is not intended for diagnosis or treatment. Please consult your physician for health concerns.

+90 850 321 50 00

Idiopathic Pleuroparenchymal Fibroelastosis (iPPFE)

Rare upper-lobe predominant fibrotic interstitial lung disease with pleural and subpleural elastic fibrosis.

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Göğüs Hastalıkları department. Book Appointment →

What is Idiopathic Pleuroparenchymal Fibroelastosis (iPPFE)?

Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is a rare idiopathic interstitial pneumonia recognized by the 2013 ATS/ERS classification, defined by intense fibroelastic thickening of the visceral pleura and immediately subjacent parenchyma, predominantly in the upper lobes.

Patients typically present with progressive dyspnea, dry cough, weight loss and a flattened thoracic cage; pulmonary function shows severe restriction with reduced FVC, increased RV/TLC ratio and decreased DLCO.

High-resolution CT shows upper-lobe pleural and subpleural reticulation with traction bronchiectasis and volume loss; histology demonstrates dense elastic-rich fibrosis confirmed by elastic stains and is differentiated from connective tissue disease, post-radiation, post-transplant and chemotherapy-induced PPFE.

Symptoms

Progressive exertional dyspnea
Dry persistent cough
Unintentional weight loss
Flattened anteroposterior chest diameter
Recurrent pneumothorax
Reduced exercise tolerance
Inspiratory crackles in upper zones

Risk Factors

Familial PPFE clusters
Telomere-related gene mutations (TERT, TERC, RTEL1)
Prior bone marrow or lung transplantation
Chemotherapy with alkylating agents
Radiation therapy to the chest
Connective tissue diseases (rheumatoid arthritis, scleroderma)
Coexisting usual interstitial pneumonia

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Persistent dyspnea with weight loss
  • Upper-lobe fibrosis on imaging
  • Recurrent pneumothorax of unclear cause
  • Family history of pulmonary fibrosis
  • Worsening pulmonary function despite anti-fibrotic therapy
  • Need for lung transplantation evaluation
  • Suspected connective tissue disease overlap

Treatment Methods

01
Multidisciplinary discussion with thoracic radiologist and pathologist
02
Smoking cessation and pulmonary rehabilitation
03
Nutritional support to counter cachexia
04
Antifibrotic therapy (pirfenidone, nintedanib) in selected cases
05
Treatment of secondary pneumothorax
06
Long-term oxygen therapy when indicated
07
Early lung transplantation referral in eligible patients

Which Department to Visit?

You can visit our Göğüs Hastalıkları department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Göğüs Hastalıkları Department

Let us help you

You can make an appointment with our specialists or contact us for your concerns.

Book AppointmentContact Us

Related Health Topics

Other articles from the same department you may want to explore.

Asthma

Göğüs Hastalıkları

Asthma is characterized by wheezing, coughing and shortness of breath attacks; with proper treatment it can be kept under control.

COPD (Chronic Obstructive Pulmonary Disease)

Göğüs Hastalıkları

COPD is an irreversible lung disease characterized by shortness of breath and chronic cough; quitting smoking slows its progression.

Pneumonia

Göğüs Hastalıkları

Pneumonia presents with high fever, cough and shortness of breath; the vast majority recover with appropriate antibiotic treatment.

Tuberculosis (TB)

Göğüs Hastalıkları

Tuberculosis presents with weeks-to-months of cough, fever, and night sweats; early diagnosis and treatment lead to full recovery.

Pleural Effusion

Göğüs Hastalıkları

Pleural effusion is the accumulation of excess fluid in the pleural space, resulting from imbalances in fluid production and removal, and represents a manifestation of diverse cardiopulmonary, infectious, and malignant disorders.

Pneumothorax

Göğüs Hastalıkları

Pneumothorax is the presence of air in the pleural space resulting in partial or complete lung collapse, classified as spontaneous (primary/secondary), traumatic, or iatrogenic, with tension pneumothorax representing a life-threatening emergency.

Bronchitis (Acute and Chronic)

Göğüs Hastalıkları

Acute bronchitis is mostly viral and resolves spontaneously, while chronic bronchitis is a smoking-related component of COPD.

Bronchiectasis

Göğüs Hastalıkları

Bronchiectasis is a chronic respiratory disease characterized by permanent, abnormal dilation of bronchi with associated destruction of muscular and elastic components of airway walls, resulting in impaired mucociliary clearance and recurrent infection.

Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.