Endocrine Abstracts

Background: Endogenous Cushing’s syndrome is caused by chronically elevated glucocorticoid levels. The most common etiology is ACTH hypersecretion from a pituitary adenoma; other causes include hypersecretion of cortisol from an adrenal source or ectopic ACTH secretion. Following successful treatment, a period of adrenal insufficiency is expected due to chronic suppression of the HPA axis. Onset and exacerbation of steroid-responsive conditions have been reported following remission of Cushing’s syndrome, leading to challenges in distinguishing a new condition vs. expected symptomatology following remission.

Objective: Describe a case of a 44-year-old man presenting with new-onset sarcoidosis diagnosed 12 months following surgical cure of Cushing’s syndrome secondary to adrenal adenoma, and synthesize existing literature reporting on de-novo steroid-responsive conditions presenting after Cushing’s syndrome remission.

Methods: A scoping review was conducted in September 2022 in Medline, Epub, Ovid, and PubMed. Case reports and case series detailing adult patients with biochemical and clinical remission of endogenous Cushing’s syndrome presenting with new-onset steroid-responsive conditions were included.

Results: 1641 articles were screened, 138 full-text studies were assessed for eligibility, and 45 studies were included and underwent data extraction and analysis, of which 83 cases were identified. Thyroid diseases (Graves, Hashimoto’s thyroiditis, subacute thyroiditis) were the most commonly reported conditions (41/83; 49%), followed by sarcoidosis (14/83; 16.8%). Psoriasis, lymphocytic hypophysitis, idiopathic intracranial hypertension, multiple sclerosis, rheumatoid arthritis, lupus, and seronegative arthritis were reported in more than one case. All patients achieved clinical remission of Cushing’s syndrome following surgical management via transsphenoidal resection or adrenalectomy with or without additional medical or radiotherapy, and one patient via medical management alone. The average duration between Cushing’s remission and de novo condition’s symptomatology was 11 months. 57% of patients were receiving corticosteroid therapy at the time of onset.

Conclusion: First presentations of several distinct steroid-responsive conditions have been reported as following remission of Cushing’s syndrome. Of these, thyroid disorders and sarcoidosis were the most commonly reported and are well-characterised in the literature. Clinicians should have a low index of suspicion for the emergence of autoimmune and inflammatory conditions in the first year following surgery or initiation of treatment of Cushing’s syndrome.