A Case Study of Pleuroparenchymal Fibroelastosis Exhibiting No Radiological Evidence of Pleural Involvement - Scorecard - MDSpire

A Case Study of Pleuroparenchymal Fibroelastosis Exhibiting No Radiological Evidence of Pleural Involvement

  • By

  • Iris A. Simons

  • Daniel A. Korevaar

  • Teodora Radonic

  • Carmen Ariño-Palao

  • Ralf W. Sprengers

  • Martijn van Dorp

  • Marjolein E. M. Lacor

  • JanWillem Duitman

  • Esther J. Nossent

  • January 19, 2026

  • 0 min

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Clinical Scorecard: A Case Study of Pleuroparenchymal Fibroelastosis Exhibiting No Radiological Evidence of Pleural Involvement

At a Glance

CategoryDetail
ConditionPleuroparenchymal Fibroelastosis (PPFE)
Key MechanismsExcessive elastosis and fibrosis affecting pleura and adjacent lung parenchyma, potentially due to recurrent pulmonary injury and mechanical stress.
Target PopulationPatients with interstitial lung disease, including those with idiopathic and secondary PPFE.
Care SettingMultidisciplinary team (MDT) in an interstitial lung disease clinic.

Key Highlights

  • PPFE can present atypically without overt pleural involvement.
  • Histopathological confirmation is essential for diagnosis.
  • Poor prognosis with progressive decline in lung function.
  • Immunosuppressive therapy may be necessary for management.
  • Clinical features may include weight loss, platythorax, and recurrent infections.

Guideline-Based Recommendations

Diagnosis

  • Histopathological examination is crucial for confirming PPFE.
  • Consider multidisciplinary evaluation for atypical presentations.

Management

  • Initiate immunosuppressive therapy in confirmed cases.
  • Consider antifibrotic therapy for progressive disease.

Monitoring & Follow-up

  • Regular pulmonary function testing to assess disease progression.
  • Follow-up imaging to monitor changes in lung patterns.

Risks

  • Increased morbidity and mortality due to respiratory failure.
  • Risk of recurrent pneumothorax and pulmonary infections.

Patient & Prescribing Data

Patients diagnosed with PPFE, including those with idiopathic and secondary forms.

Combination therapy with immunosuppressants and antifibrotics may be beneficial in managing disease progression.

Clinical Best Practices

  • Conduct thorough physical examinations to identify clinical signs like deepened suprasternal notch.
  • Utilize high-resolution computed tomography (HRCT) for detailed imaging of lung patterns.
  • Engage in a multidisciplinary approach for diagnosis and management.

References

Original Source(s)

A Case Study of Pleuroparenchymal Fibroelastosis Exhibiting No Radiological Evidence of Pleural Involvement

  • by Iris A. Simons, Daniel A. Korevaar, Teodora Radonic, Carmen Ariño-Palao, Ralf W. Sprengers, Martijn van Dorp, Marjolein E. M. Lacor, JanWillem Duitman, Esther J. Nossent

  • January 19, 2026

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