A Case of Pleuroparenchymal Fibroelastosis

Area of Science:

• Pulmonology
• Rare Diseases
• Fibrotic Lung Diseases

Background:

• Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia.
• PPFE often presents with upper lobe predominant fibrotic changes.
• Some PPFE cases share radiological and clinical similarities with unilateral upper-field pulmonary fibrosis (unilateral upper-PF).

Purpose of the Study:

• To present a case of Pleuroparenchymal fibroelastosis (PPFE).
• To discuss the clinical course and similarities to unilateral upper-field pulmonary fibrosis.
• To highlight the poor prognosis associated with this condition.

Main Methods:

• Case report of a 55-year-old male patient with PPFE.
• Review of patient's medical history, including prior renal cancer resection.
• Documentation of disease progression and treatment response.

Main Results:

• The patient presented with dyspnea and was diagnosed with PPFE.
• He had a history of renal cancer resection.
• Despite treatment with nintedanib, the patient succumbed to respiratory failure, demonstrating a poor prognosis.

Conclusions:

• This PPFE case exhibited features overlapping with unilateral upper-PF.
• The patient's medical history and disease trajectory underscore the aggressive nature of PPFE.
• Insights from this case may inform management strategies for similar PPFE patients.