To illustrate an atypical presentation of pleuroparenchymal fibroelastosis (PPFE) without overt pleural involvement, emphasizing the disease's variability in presentation.
Approach:
Key Findings:
Histopathology confirmed NSIP in lower lobes and intra-alveolar fibroelastosis in upper lobes consistent with PPFE.
Initial presentation showed no radiological evidence of pleural involvement, which later progressed to classical PPFE features with dense pleural consolidations.
A deepened suprasternal notch was noted, potentially indicating upper lobe volume loss.
Interpretation:
This case highlights the atypical presentation of PPFE, emphasizing the critical need for thorough clinical and radiological evaluation in diagnosing interstitial lung diseases.
Limitations:
The case is a single patient study, limiting generalizability to broader populations.
Long-term outcomes and responses to treatment were not fully explored, which may affect the understanding of disease progression.
Conclusion:
The study underscores the importance of recognizing atypical presentations of PPFE and the potential for disease progression, warranting ongoing monitoring and timely treatment adjustments.
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
