Complications and mortality of Cushing's disease: report on data collected over a 20-year period at a referral centre

ContextCushing's disease (CD) is rare condition burdened by several systemic complications correlated to higher mortality rates. The primary goal of clinicians is to achieve remission, but it is unclear if treatment can also increase life expectancy.AimTo assess the prevalence of cortisol-related complications and mortality in a large cohort of CD patients attending a single referral centre.Materials and methodsThe clinical charts of CD patients attending a referral hospital between 2001 and 2021 were reviewed.Results126 CD patients (median age at diagnosis 39 years) were included. At the last examination, 78/126 (61.9%) of the patients were in remission regardless of previous treatment strategies. Patients in remission showed a significant improvement in all the cardiovascular (CV) comorbidities (p < 0.05). The CV events were more frequent in older patients (p = 0.003), smokers and persistent CD groups (p < 0.05). Most of the thromboembolic (TE) and infective events occurred during active stages of the disease. The CV events were the most frequent cause of death. The standardized mortality ratio (SMR) resulted increased in persistent cases at the last follow-up (SMR 4.99, 95%CI [2.15; 9.83], p < 0.001) whilst it was not higher in those in remission (SMR 1.66, 95%CI [0.34; 4.85], p = 0.543) regardless of the timing or number of treatments carried out. A younger age at diagnosis (p = 0.005), a microadenoma (p = 0.002), and remission status at the last follow-up (p = 0.027) all increased survival. Furthermore, an elevated number of comorbidities, in particular arterial hypertension, increased mortality rates.ConclusionsPatients with active CD presented a poor survival outcome. Remission restored the patients' life expectancy regardless of the timing or the types of treatments used to achieve it. Persistent CD-related comorbidities remained major risk factors.

Automated summary

This study evaluated the prevalence of cortisol-related complications and mortality rates in a large cohort of Cushing’s disease (CD) patients referred to a single center. It was found that patients in remission showed a significant improvement in cardiovascular comorbidities, while those with active CD had poor survival outcomes.