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Idiopathic pleuroparenchymal fibroelastosis, a new idiopathic interstitial pneumonia: A case report
Enrique Javier Soto Hurtado1, Maria Luisa Amaya González2, Maria Del Mar Elena Soto3
1Department of Respiratory Medicine. La Serranía Hospital, Ronda (Málaga), Spain enriquesoto40@hotmail.com.
Abstract:
Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disease, idiopathic in most of the cases described in the literature. We report the case of a 55-year-old patient, non-smoker with tuberculosis treated in his youth, starting with progressive dyspnoea and cough, with radiographic abnormalities consisting of pleural thickening, bronchiectasis and structural distortion predominantly in the upper lobes. Due to functional impairment and persistent radiographic abnormalities, biopsy by video-assisted thoracoscopic surgical was decided. The presence of striking elastosis, absence of interstitial damage and abrupt boundary of the fibrous lesion with healthy lung allowed the diagnosis of IPPFE. Currently, the patient has no specific treatment and is in follow-up in the Transplant Unit.
Insights
Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare lung disease. This case highlights its distinct elastosis and upper lobe findings, emphasizing diagnostic challenges.
Area of Science:
- Pulmonology
- Pathology
Background:
- Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare interstitial lung disease.
- Most cases are idiopathic, posing diagnostic challenges.
Observation:
- A 55-year-old non-smoker with a history of tuberculosis presented with progressive dyspnea and cough.
- Radiographic findings included upper lobe predominant pleural thickening, bronchiectasis, and structural distortion.
Findings:
- Video-assisted thoracoscopic surgery biopsy revealed striking elastosis.
- The absence of interstitial damage and a sharp lesion boundary supported the IPPFE diagnosis.
Implications:
- This case underscores the importance of considering IPPFE in specific clinical presentations.
- Accurate diagnosis is crucial for patient management, even in the absence of specific treatments.
