Mortality and Morbidity in Cushing's Disease over 50 Years in Stoke-on-Trent, UK: Audit and Meta-Analysis of Literature

Abstract

Overall mortality in CD is double that of the general population. However, patients with CD in remission fare much better than those with persistence of hypercortisolism, and they appear not to have an increased mortality rate. Hypertension and diabetes mellitus are risk factors for worse outcome. Because diagnosis and treatment of patients are at a young age, much longer follow-up of patients in remission is required before one can be confident that their mortality outcome is no different from that of the general population, especially because cardiovascular risk factors may persist after successful biochemical control of the disease.

Recommendations of the Neuroendocrinology Department of the Brazilian Society of Endocrinology and Metabolism for the diagnosis of Cushing’s disease in Brazil

“…However, when analyzing the mortality rate in relation to the remission of Cushing's syndrome, there was a higher mortality rate in patients with persistent disease compared to those in clinical remission: 5.50 (2.69-11.26) vs. 1.20 (0.45-3.18), respectively (15).…”

“…In children, a short stature, abnormal virilization, pubertal delay or pseudo precocious puberty may be observed. Moreover, there are common comorbidities in clinical prac- Patients with Cushing's syndrome have a higher mortality than the general population (9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21), mainly due to the development of cardiovascular disease (ischemic heart disease and cerebrovascular disease), diabetes mellitus (secondary to hypercortisolism) and infections (due to the state of immunosuppression).…”

“…A meta-analysis showed a standardized mortality ratio (SMR) of 2.22 (1.45-3.41; confidence interval 95%) (15), which varied between studies from 0.98 (0.44-2.18) (10) to 4.80 (2.79 to 8.27) (15). However, when analyzing the mortality rate in relation to the remission of Cushing's syndrome, there was a higher mortality rate in patients with persistent disease compared to those in clinical remission: 5.50 (2.69-11.26) vs. 1.20 (0.45-3.18), respectively (15).…”

Cardiac hypertrophy in Cushing’s syndrome: if not hypertension then what?

“…Currently, it is acknowledged that hypertension is a prominent feature of CS affecting around 75% of the patients [1]. Cardiovascular disease is a major cause of morbidity and is largely responsible for the increased mortality of CS [2]. Importantly, the increased cardiovascular mortality risk in patients with Cushing's disease persists even after longterm remission [3,4], suggesting that not only functional but also structural abnormalities develop that may not be reversible.…”

Budget impact of pasireotide for the treatment of Cushing’s disease, a rare endocrine disorder associated with considerable comorbidities

“…Mortality studies have been conducted among only a limited set of patients. However, one meta-analysis shows that the overall CD-related mortality among CD patients that are treated primarily with transsphenoidal surgery (TSS) is roughly twice that of the general population 7 . Epidemiological studies of CD are few and provide only very limited information regarding estimates of the incidence and prevalence of this rare disorder in the general population [8][9][10] .…”