内分泌学とメタボリックシンドローム
オープンアクセス

概要

Endogenous Cushing Syndrome- A Spectrum of Cases

Santosh B, Raman Boddula, Ashok Venkatanarasu, Aditya Hegde, Chimutai Chinte, Vidya Tickoo

Introduction

Cushing’s syndrome (CS) is a rare disorder characterised by symptoms and signs of chronic excessive tissue exposure to glucocorticoids, and is associated with high morbidity and mortality if untreated. The excess cortisol state can result from a number of etiologies- both exogenous and endogenous. It can present with varied and non-specific clinical features. Hence having a high clinical suspicion is important and it can be challenging to arrive at an exact diagnosis, which is very important to initiate appropriate treatment. Here, we present a spectrum of endogenous Cushing’s syndrome of different etiologies, who presented to us at different age groups and different clinical features.

Description of Cases

The main aim of this article is to understand the different symptoms and signs with which the patients with Endogenous Cushing’s syndrome present, the knowledge required to suspect it even in a non-typical presentation, the extensive and laborious work-up done at arriving the diagnosis and localizing the cause, and then finally initiating appropriate treatment. We randomly chose 8 cases of Endogenous Cushing’s syndrome of different presentation and etiology, who presented to our Hospital. We had patients whose age ranged from 13 years to 60 years; patients presenting with typical features of Cushing’s syndrome to non-specific features and even being asymptomatic. All the cases underwent appropriate and elaborate work-up to arrive at a diagnosis and also to localize the exact lesion. They then underwent appropriate treatment either in the form of surgery (excision of the lesion responsible for the cortisol excess) or medical therapy. The outcome of the patients was good in 6 of these patients, they achieved remission during follow-up. However, 2 patients, one with Adrenocortical carcinoma and another with Ectopic adreno-cortico tropic hormone (ACTH) syndrome due to Poorly differentiated adenocarcinoma of left lung, died due to the severity of underlying diseases.

Conclusion

Endogenous Cushing’s syndrome, though rare, is associated with high morbidity and mortality if untreated. A high degree of suspicion and a good clinical examination is required to diagnose Cushing’s syndrome especially in nonflorid cases and those with nonspecific features. A systematic, orderly evaluation is required to establish the correct diagnosis and to localize the cause, which will help in planning of appropriate treatment.