Endocrine Abstracts

Introduction: Autoimmune disease is a rare event occurring after remission of Cushing’s syndrome. We report on the appearance of new onset vitiligo in a patient treated for Cushing disease due to a an invasive pituitary adenoma, after bilateral adrenalectomy.

Case report: A 64-year-old man presented to our endocrinology clinic with progressive lumbar pain that had developed two months earlier. He had undergone bilateral adrenalectomy for Cushing’s syndrome (CS) four years ago and transsphenoidal resection for Nelson tumor two years later. On physical examination, the patient had remarkable hyperpigmentation due to ACTH hypersecretion periorbital, peri-auricular and in the lower-neck region (figure 1). These findings were evident in his case due to the extensive facial vitiligo. Findings on bone scintigraphy were suggestive for metastatic lesions (figure 2) and CT-guided bone biopsy confirmed our suspicion of bone-invasive pituitary carcinoma. The patient was referred to the oncology department as a candidate for immunotherapy but opted for palliative care because of his weakened general condition.

Discussion: This case highlights the fact that hypercortisolism induces a state of immunosuppression. After treatment and normalization of cortisol hypersecretion in Cushing’s syndrome, rebound immunity may result in overt autoimmune diseases, in casu vitiligo. In a study by de Mota et al. it was reported that 8 out of 78 (10.3 %) adult patients with endogenous CS presented with a new autoimmune or allergic disease after treatment [1]. Other studies have reported an increased incidence of autoimmune thyroid disease in patients after treatment of CS [2, 3]. There have been descriptions of other forms of autoimmunity after cure of CS, mainly in case reports, such as rheumatoid arthritis, celiac disease, and systemic lupus erythematosus [2, 4-5]. This immunological phenomenon has been described in both ACTH-dependent and -independent cases but a lot of questions remain open to discussion. What is the underlying pathophysiological mechanism? Would autoimmunity be there if CS had not occurred? Is autoimmunity a transient phenomenon in these patients?

Conclusion: We present the case of a 64-year-old man with rapidly developing vitiligo, after bilateral adrenalectomy for an invasive pituitary ACTH secreting adenoma. The diagnosis of a metastasized pituitary carcinoma was confirmed. Patients with CS are considered immune-depressed. After treatment of CS, patients are at risk for developing autoimmune diseases. Early recognition of these autoimmune conditions and appropriate treatment are warranted.

References: 1. da Mota F, Murray C, Ezzat S. Overt immune dysfunction after Cushing’s syndrome remission: A consecutive case series and review of the literature. J Clin Endocrinol Metab 2011; 96: E1670–E1674.

2. Colao A, Pivonello R, Faggiano A, Filippella M, Ferone D, Di Somma C, Cerbone G, Marzullo P, Fenzi G, Lombardi G. Increased prevalence of thyroid autoimmunity in patients successfully treated for Cushing’s disease. Clin Endocrinol (Oxf) 2000; 53: 13–19.

3. Mussig K, Gallwitz B. Haring HU, Seif FJ. Manifestation of thyroid autoimmunity in patients successfully treated for hypercortisolism. Clin Endocrinol (Oxf) 2004; 61: 284.

4. Yakushiji F, Kita M, Hiroi N, Ueshiba H, Monma I, Miyachi Y. Exacerba- tion of rheumatoid arthritis after removal of adrenal adenoma in Cushing’s syndrome. Endocr J 1995; 42: 219–223.

5. Candrina R, Di Stefano O. Exacerbation of celiac disease after cure of Cushing’s disease. Am J Med 1993; 95: 341