Our case series describes two siblings with complex fibrosing lung diseases. The first patient was initially given a diagnosis of sarcoidosis based on imaging and exclusion of alternative diagnoses. A number of years after diagnosis, he had rapid deterioration of his disease and following surgical lung biopsy, his lung fibrosis was re-classified as chronic hypersensitivity pneumonitis (cHP) with a usual interstitial pneumonia pattern. He subsequently underwent successful lung transplantation. The second patient presented with rapidly progressing exertional dyspnoea. His bloods, imaging, bronchoalveolar lavage and histology were discussed at our multidisciplinary team meeting. His histology was most in keeping with subacute on cHP with overlapping imaging features between the two siblings. He was treated accordingly but unfortunately succumbed to his illness shortly after diagnosis. These cases highlight the difficulties differentiating between the various interstitial lung disease (ILD) subtypes and the challenges in management while also increasing awareness of familial ILD.
- medical management
- respiratory medicine
- interstitial lung disease
- drugs: respiratory system
- cardiothoracic surgery
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Patient consent for publication Obtained.
Contributors Category 1: identification of case for write-up: MK, TJM. Planning for data collection and write-up: MOC, TJM. Acquisition of patient consent: TJM, MK. Acquisition of patient information to write-up case report: MOC, AF, MK, TJM. Interpretation and reporting of histopathological slides: AF. Interpretation and reporting of radiological images: MK, TJM. Analysis and interpretation of literature relating to case: MOC. Category 2: drafting the case report: MOC. Critical revision of the case report manuscript: MK, TJM. Category 3: approval of the version of the manuscript to be submitted for publication: MOC, MK, AF, TJM. Correction of manuscript submission: MOC, TJM.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
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