A Case Study of Pleuroparenchymal Fibroelastosis Exhibiting No Radiological Evidence of Pleural Involvement - Takeaways - 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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  • 1

    Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease characterized by excessive elastosis and fibrosis, primarily affecting the upper lobes.

  • 2

    Atypical presentations of PPFE can occur without overt pleural involvement, leading to intra-alveolar airway-centered fibrotic patterns.

  • 3

    The case study describes a 66-year-old female with PPFE, initially presenting with intra-alveolar fibroelastosis and later progressing to classical pleuroparenchymal phenotype.

  • 4

    The patient experienced a decline in lung function and was treated with immunosuppressive therapy and antifibrotic therapy after diagnosis.

  • 5

    Clinical findings such as a deepened suprasternal notch may indicate upper lobe volume loss in PPFE, highlighting the need for thorough physical examination.

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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