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. A few common features of CS include truncal obesity, moon facies,
increased fat in dorsocervical area, ecchymoses, plethora, striae, proximal
muscle weakness, thin skin, pigmentation of the skin, osteopenia/ osteoporosis
with fractures, avascular necrosis of bone, mood changes, hypertension,
impairment of glucose tolerance or worsening of glycemic control, susceptibility
to infections, edema, deep vein thrombosis/ pulmonary thromboembolism. 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, which are presented to us at different age
groups and with 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 ages 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-cortical tropic hormone (ACTH) syndrome due to Poorly
differentiated adenocarcinoma of the 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 are required to diagnose Cushing’s
syndrome, especially in non-florid 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 the planning of appropriate
treatment.
Author(s) Details:
Santosh B,
Department of Endocrinology, Yashoda Hospital, Secunderabad,
Telangana, India.
Raman
Boddula,
Department
of Endocrinology, Yashoda Hospital, Secunderabad, Telangana, India.
Ashok Venkatanarasu,
Department of Endocrinology, Yashoda Hospital, Secunderabad,
Telangana, India.
Aditya Hegde,
Department of Endocrinology, Yashoda Hospital, Secunderabad,
Telangana, India.
Chimutai Chinte,
Department
of Endocrinology, Yashoda Hospital, Secunderabad, Telangana, India.
Vidya
Tickoo,
Department
of Endocrinology, Yashoda Hospital, Secunderabad, Telangana, India.
Please see the link here: https://stm.bookpi.org/NVMMS-V5/article/view/14151
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