SESSION TITLE: Topics in ILD and Lung Transplantation
SESSION TYPE: Original Investigation Slide
PRESENTED ON: Wednesday, October 29, 2014 at 02:45 PM - 04:15 PM
PURPOSE: Sarcoidosis is a multi-system disorder of unknown etiology that generally has a favorable prognosis and frequently spontaneously resolves. However, in a subset of patients is a chronically progressive disease with mortality rates reported of up to 6%.
METHODS: The medical records and autopsy findings of 35 consecutive patients with sarcoidosis undergoing autopsy at Mayo Clinic (Rochester, MN, USA) over a 10-yr period, from January 1 2004 to December 31 2013 were reviewed.
RESULTS: The median age at death was 58 years (range, 33-94 years) and included 17 (49%) females. Of the patients with a listed ethnic group, 91% (29 out of 32 patients) were Caucasian. Sarcoidosis was clinically diagnosed in 19 (56%) of the 35 patients while being incidentally found at the time of the autopsy in the additional 15 (44%) patients. In one additional patient it was indeterminate if sarcoidosis was pre-diagnosed before autopsy. Sarcoidosis-related causes accounted for 7 deaths (20%) and included 1 from invasive aspergillosis related to immunosuppressive therapy and 6 deaths resulting from cardiac sarcoidosis. Cardiac involvement was diagnosed in only 2 (11%) of the 19 patients with clinically detected sarcoidosis in contrast to 6 (40%) of the 15 cases diagnosed t at autopsy. Five (83%) of the 6 cardiac-sarcoidosis related deaths were due to sudden death and sarcoidosis had never been clinically diagnosed in 4 (80%) of these 5 cases. Coexisting cancer was found in 18 patients (51%) and included 5 patients in whom it was newly found at autopsy. The most common cancer was skin cancer (6 cases) followed by lymphoma (3 cases). In our series none of the deaths were related to pulmonary fibrosis.
CONCLUSIONS: We conclude that the majority of sarcoidosis-affected patients die from causes unrelated to sarcoidosis. Cardiac involvement is the main cause in those who suffer a sarcoidosis-related death which is likely to remain undiagnosed during life.
CLINICAL IMPLICATIONS: Sarcoidosis patients likely die of sarcoidosis-unrelated causes. Almost half of the number of sarcoidosis-affected patients will remain undiagnosed in their lifetimes. Cardiac involvement of sarcoidosis is likely be unrecognized and is still the most frequent cause of sarcoidosis-related deaths.
DISCLOSURE: The following authors have nothing to disclose: Xiaowen Hu, Eva Carmona Porquera, Joanne Yi, Jay Ryu
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