PURPOSE: Making an accurate and confident diagnosis of specific forms of ILD is often difficult, and incorrect diagnoses are often made. We examined (1) the correlation of pre-transplant diagnoses with diagnoses reached on the basis of explanted lung pathology, and (2) the correlation of explant pathology with the pre-transplant UNOS diagnosis.
METHODS: We reviewed the charts of 121 patients with ILD to ascertain the pre-transplant diagnosis and UNOS listing diagnosis, and we also examined the histopathology of explanted lungs to correlate the consistency of pre- and post-transplant diagnoses. In cases where the pathology report was inconclusive, we reviewed the slides directly with a pulmonary pathologist.
RESULTS: A total of 121 patients have been transplanted for interstitial lung disease at the University of Wisconsin between October 1988 and November 2009. The pre-transplant diagnoses include idiopathic pulmonary fibrosis (94 cases), sarcoidosis (14 cases), nonspecific interstitial pneumonia (2 cases), post-ARDS pulmonary fibrosis (2 cases), hypersensitivity pneumonitis (2 cases) and one each of the following: Langerhans cell histiocytosis, silicosis, collagen vascular disease-associated ILD, berylliosis, eosinophilic pneumonia, medication-induced ILD and Hermansky-Pudlak syndrome. The post-explant biopsy was confirmatory of the pre-transplant diagnosis in 91%. Of the 11 cases where the pre-transplant diagnosis was incorrect, 8 had undergone lung biopsy prior to transplant listing. The most commonly mistaken diagnosis was IPF (10 cases).
CONCLUSIONS: The pre-transplant diagnosis was confirmed in 91 % of the patients undergoing either single lung or bilateral lung transplant for interstitial lung disease. Pre-transplant lung biopsy did not always correlate with explant pathology.
CLINICAL IMPLICATIONS: Accurate diagnosis of specific forms of ILD remains elusive, and has implications for listing priority with the advent of the lung allocation score as well as maintaining accurate lung transplant databases. Clinical and radiologic diagnosis of IPF is not infallible, nor is pre-transplant lung biopsy.
DISCLOSURE: The following authors have nothing to disclose: Jeremy Siegrist, Keith Meyer, Nilto DeOliveira, Jose Torrealba
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