Nephrogenic Diabetes Insipidus as the First Manifestation of Ectopic ACTH Syndrome in a HIV Infected Patient

Authors

  • Everlayny Fiorot Costalonga UNIVERSIDADE FEDERAL DO ESPÍRITO SANTO
  • Amanda Weberling Coelho Moreira
  • Francisca Germanya Morais Borges
  • Veronica Gabriela Guerra
  • Thais Leibel Sant’ Anna Santos
  • Leon Cleres Penido Pinheiro
  • Susan Caroline Azevedo Aredes
  • Weverton Machado Luchi

DOI:

https://doi.org/10.3823/2029

Keywords:

Ectopic ACTH Syndrome, Cushing Syndrome, diabetes insipidus, lung neoplasms, HIV infection

Abstract

The Ectopic Adrenocorticotrophic Hormone (ACTH) Syndrome (EAS) is a high mortality paraneoplastic syndrome, which represents 10-15% of Cushing Syndromes (CS). Around 70% of tumors are located in the chest, neck or adrenals. It frequently has atypical manifestations, leading to delay in diagnosis. Here, we report a particular case of EAS manifested predominantly by nephrogenic diabetes insipidus (NDI). A 48 years old man, HIV infected, was admitted with polyuria, polidipsia and lower limb weakness. He had hipertension, crackles in the right hemithorax and linphanedophathy. Laboratory assessments showed hyperglycemia, hypernatremia, severe hypocalemia, metabolic alkalosis and low urinary osmolarity, which persisted after water deprivation test and desmopressin administration, characterizing a NDI. Chest images showed a lung tumor, which, in conjunction with the clinical and laboratorial picture lead to the suspicion of EAS. Cortisol levels after dexametasona were 143μg/dL and serum ACTH was 630pg/mL. Pituitary was normal and adrenals showed bilateral hyperplasia. Lymph node biopsy revealed a neuroendocrine tumor with positive immunohistochemistry for ACTH. Patient died a few days later due to nosocomial pneumonia. The EAS can be associated with very high cortisol levels, which evolves rapidly, resulting in the absence of typical features of CS. The knowledge of the different clinical presentations is essential to establish prompt diagnostic and support. This syndrome has to be considered in patients with a neoplasm and diabetes insipidus, especially if accompanied by signs of excess of mineralocorticoid action. Although the ideal treatment is excision of the tumor, adrenolitic drugs may help to reduce complications and improve survival.

References

Alexandraki KI, Grossman AB. The ectopic ACTH syndrome. Rev Endocr Metab Disord2010 Jun;11(2):117-26.

Isidori AM, Lenzi A. Ectopic ACTH syndrome. Arq Bras Endocrinol Metabol2007 Nov;51(8):1217-25.

Salgado LF, MCBV; Knoepfelmacher,M; Machado, MC; Domenice,S; Pereira, MAA; Mendonca,BB. Ectopic ACTH syndrome: our experience with 25 cases. European Journal of Endocrinology2006;155.

Agha A, Brennan S, Moore KB, Grogan L, Thompson CJ. Small-cell lung cancer presenting as diabetes insipidus and Cushing's syndrome. Pituitary2005;8(2):105-7.

Lococo F, Negro A, Zanelli F, Valli R, Filice A, Rapicetta C, et al. Severe Cushing's syndrome related to a small pulmonary carcinoid with widespread dissemination. Intern Med2015;54(5):477-80.

Raff H. Cushing syndrome: update on testing. Endocrinol Metab Clin North Am2015 Mar;44(1):43-50.

Raff H, Carroll T. Cushing's syndrome: from physiological principles to diagnosis and clinical care. J Physiol2014 Feb 1;593(3):493-506.

Papanek PE, Raff H. Physiological increases in cortisol inhibit basal vasopressin release in conscious dogs. Am J Physiol1994 Jun;266(6 Pt 2):R1744-51.

Raff H. Glucocorticoid inhibition of neurohypophysial vasopressin secretion. Am J Physiol1987 Apr;252(4 Pt 2):R635-44.

Baas JJ, Schaeffer F, Joles JA. The influence of cortisol excess on kidney function in the dog. Vet Q1984 Jan;6(1):17-21.

Joles JA, Rijnberk A, van den Brom WE, Dogterom J. Studies on the mechanism of polyuria induced by cortisol excess in the dog. Vet Q1980 Oct;2(4):199-205.

Giebisch G, Lozano R. The effects of adrenal steroids and potassium depletion on the elaboration of an osmotically concentrated urine. J Clin Invest1959 May;38(5):843-53.

Marples D, Frokiaer J, Dorup J, Knepper MA, Nielsen S. Hypokalemia-induced downregulation of aquaporin-2 water channel expression in rat kidney medulla and cortex. J Clin Invest1996 Apr 15;97(8):1960-8.

Kim JK, Summer SN, Berl T. The cyclic AMP system in the inner medullary collecting duct of the potassium-depleted rat. Kidney Int1984 Oct;26(4):384-91.

Raisz LG, McNeely WF, Saxon L, Rosenbaum JD. The effects of cortisone and hydrocortisone on water diuresis and renal function in man. J Clin Invest1957 Jun;36(6 Part 1):767-79.

Hall AM. Update on tenofovir toxicity in the kidney. Pediatr Nephrol2013 Jul;28(7):1011-23.

Zaidan M, Lescure FX, Brocheriou I, Dettwiler S, Guiard-Schmid JB, Pacanowski J, et al. Tubulointerstitial nephropathies in HIV-infected patients over the past 15 years: a clinico-pathological study. Clin J Am Soc Nephrol2013 Jun;8(6):930-8.

Libório AB, Andrade L, Pereira LV, Sanches TR, Shimizu MH, Seguro AC. Rosiglitazone reverses tenofovir-induced nephrotoxicity. Kidney Int2008 Oct;74(7):910-8.

Newell-Price J, Grossman AB. Differential diagnosis of Cushing's syndrome. Arq Bras Endocrinol Metabol2007 Nov;51(8):1199-206.

Hammami MM, Duaiji N, Mutairi G, Aklabi S, Qattan N, Abouzied Mel D, et al. Case report of severe Cushing's syndrome in medullary thyroid cancer complicated by functional diabetes insipidus, aortic dissection, jejunal intussusception, and paraneoplastic dysautonomia: remission with sorafenib without reduction in cortisol concentration. BMC Cancer2015;15:624.

Cuevas-Ramos D, Fleseriu M. Treatment of Cushing's disease: a mechanistic update. J Endocrinol2014 Nov;223(2):R19-39.

Feelders RA, Hofland LJ. Medical treatment of Cushing's disease. J Clin Endocrinol Metab2013 Feb;98(2):425-38.

Nieman LK, Biller BM, Findling JW, Murad MH, Newell-Price J, Savage MO, et al. Treatment of Cushing's Syndrome: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab2015 Aug;100(8):2807-31.

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Published

2016-08-05

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Section

Internal Medicine & Hospital Medicine