Presentation
Shortness of breath on exertion with normal echocardiogram. Bouts of productive cough.
Patient Data
Bilateral irregular apical pleural thickening.
Symmetrically accentuated bilateral bronchovascular pattern.
Horizontal fissure higher than expected - pulled upwards.
Elevated right hemidiaphragm.
Unchanged compared with previous x-ray from 2 months ago (not shown).
Upper lobe-predominant fibrosis, pleural tags, interlobular thickening, and impression of mild traction bronchiectasis. Thickened pleura prominent in the right hemithorax.
RLL intrabronchial secretions with patchy consolidations and tree-in-bud opacities, compensatory hyperinflated surrounding lung.
Deep suprasternal notch.
RML pneumonia - consolidations with air bronchogram, patchy consolidations, and tubular bronchiectasis. Bronchial secretions in RML and in lower lobes. Posterobasal tree-in-bud opacities in lower lobes, more prominent in the LLL. Bronchial wall thickening.
Trachea pulled to the right. Worsening of apical fibrotic changes. Pleural, including fissural, thickening. Interlobular thickening more extensive now. New fibrotic changes in the RML. Small loculated pneumothorax peripheral to the RML.
Case Discussion
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 metres. 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.
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