Formerly healthy young woman. Several episodes of productive cough for the past two months, treated each time with antibiotics. Gradually worsening shortness of breath (SOB). When she arrived at the ER she complained of SOB after climbing one flight of stairs or walking several hundred meters. Echocardiogram normal. At the ER, bloodwork was all within normal ranges, including troponin, C-reactive protein, D-dimer, and CBC.

Fibrosing interstitial lung disease with a pleural component was diagnosed based on the CT findings, compatible with pleuroparenchymal fibroelastosis (PPFE). There is progression of pathologic findings on subsequent CT studies.

Transbronchial biopsy obtained between the second and third CT scan:
“Fragments of bronchial mucosa with scanty peribronchial lung tissue with very mild fibrosis on mason trichrome stains, and mild chronic inflammation. Gram, Silver and PAS histochemical strains are negative.”

Pleuroparenchymal fibroelastosis was officially diagnosed, for which as yet there is no definitive treatment except for lung transplantation.

She went on to receive a bilateral lung transplantation.

Special thanks go out to Dr. Osnat Moreh Rahav, chest radiologist, and to Dr. Tatiana Boikaner, pulmonologist.