ENDOCRINOLOGY IN THE TIME OF COVID-19: Management of Cushing's syndrome

Abstract

Clinical evaluation should guide those needing immediate investigation. Strict adherence to COVID-19 protection measures is necessary. Alternative ways of consultations (telephone, video) should be used. Early discussion with regional/national experts about investigation and management of potential and existing patients is strongly encouraged. Patients with moderate or severe clinical features need urgent investigation and management. Patients with active Cushing's syndrome, especially when severe, are immunocompromised and vigorous adherence to the principles of social isolation is recommended. In patients with mild features or in whom a diagnosis is less likely, clinical re-evaluation should be repeated at 3 and 6 months or deferred until the prevalence of SARS-CoV-2 has significantly decreased; however, those individuals should be encouraged to maintain social distancing. Diagnostic pathways may need to be very different from usual recommendations in order to reduce possible exposure to SARS-CoV-2. When extensive differential diagnostic testing and/or surgery is not feasible, it should be deferred and medical treatment should be initiated. Transsphenoidal pituitary surgery should be delayed during high SARS-CoV-2 viral prevalence. Medical management rather than surgery will be the used for most patients, since the short- to mid-term prognosis depends in most cases on hypercortisolism rather than its cause; it should be initiated promptly to minimize the risk of infection in these immunosuppressed patients. The risk/benefit ratio of these recommendations will need re-evaluation every 2-3 months from April 2020 in each country (and possibly local areas) and will depend on the local health care structure and phase of pandemic.

Figure 1

Suggested algorithm for investigation and management of suspected Cushing's syndrome during COVID-19 pandemic. ONDST: overnight dexamethasone suppression test; UFC: urinary free cortisol; ACC: adrenocortical cancer. ¹The higher the UFC or post-dexamethasone serum cortisol the greater the confidence that the patient has Cushing's syndrome; ²MRI or CT pituitary if clear visual field defects to identify sight-threatening macroadenoma – CT head with coronal reconstructions can be included in the body CT to minimise need for extra imaging.

Figure 2

Medical management by ‘Block and Replace’. These recommendations for block and replace regime are ‘expert advice’ rather than being evidence based. Telephone consultations should evaluate symptomatology, weight, BP and capillary blood glucose to assist judging the effectiveness of the replacement glucocorticoid dose. Patients should be instructed to follow ‘sick day rules’ for glucocorticoid replacement (see text). Note: when using metyrapone any assay that has cross-reactivity with 11-deoxycortisol will read falsely high – where possible – LC-MS/MS assays are recommended; *doses may be increased further if needed and ketoconazole and metyrapone may be used in combination; with monitoring, it may also be possible to reduce the dose while maintaining blockade – collaboration with expert in Cushing's is recommended.