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Hypothalamic–pituitary sarcoidosis with vision loss and hypopituitarism: case series and literature review

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Abstract

Purpose

Hypothalamic–pituitary (HP) neurosarcoidosis (NS) accounts for 0.5 % cases of sarcoidosis and 1 % of HP masses. Correlative data on endocrine and neurological outcomes is lacking.

Methods

Retrospective case series and literature review of presentation, treatment and outcome of HP NS.

Results

Our series includes 4 men, ages 34–59, followed for a median of 7.3 years (range 1.5–17). All had optic neuropathy, multiple pituitary hormone abnormalities (PHAs) and other organ involvement by sarcoidosis (lung, sino-nasal, brain/spine and facial nerve). Two patients had central diabetes insipidus and one impaired thirst with polydipsia. After treatment with high-dose glucocorticoids, optic neuropathy improved in one case and stabilized in the others. After treatment, HP lesions improved radiologically, but PHAs persisted in all cases. Review of four published series on HP NS in addition to ours yielded 46 patients, age 37 ± 11.8 years, 65 % male. PHAs consisted of anterior hypopituitarism (LH/FSH 88.8 %, TSH 67.4 %, GH 50.0 %, ACTH 48.8 %), hyperprolactinemia (48.8 %) and diabetes insipidus (65.2 %). PHAs were the first sign of disease in 54.3 % patients. Vision problems occurred in 28.3 % patients, but optic neuropathy was not well documented in previous series. Most patients (93.5 %) received high-dose glucocorticoids followed by taper; 50 % also received other immunomodulators, including methotrexate, mycophenolate mofetil, cyclosporine, azathioprine, infliximab and hydrochloroquine. Only 13 % patients showed improvement in PHAs. All-cause mortality was 8.7 %.

Conclusion

HP NS is a serious disease requiring multidisciplinary treatment and lifelong follow-up. Prospective multicentric studies are needed to determine a more standardized approach to HP NS and outline predictors of disease outcome.

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References

  1. Porter N, Beynon HL, Randeva HS (2003) Endocrine and reproductive manifestations of sarcoidosis. QJM Month J Assoc Phys 96:553–561

    CAS  Google Scholar 

  2. Nozaki K, Judson MA (2012) Neurosarcoidosis: clinical manifestations, diagnosis and treatment. Presse Med 41:e331–e348

    Article  PubMed  Google Scholar 

  3. Statement on Sarcoidosis (1999) Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am J Respir Crit Care Med 160:736–755

  4. Randeva HS, Davison R, Chamoun V, Bouloux PM (2002) Isolated neurosarcoidosis—a diagnostic enigma: case report and discussion. Endocrine 17:241–247

    Article  PubMed  CAS  Google Scholar 

  5. Stern BJ, Krumholz A, Johns C et al (1985) Sarcoidosis and its neurological manifestations. Arch Neurol 42:909–917

    Article  PubMed  CAS  Google Scholar 

  6. James DG (1998) Life-threatening situations in sarcoidosis. Sarcoidosis, vasculitis, and diffuse lung diseases. Off J World Assoc Sarcoidosis Other Granulomatous Disord 15:134–139

    CAS  Google Scholar 

  7. Sharma OP (1997) Neurosarcoidosis: a personal perspective based on the study of 37 patients. Chest 112:220–228

    Article  PubMed  CAS  Google Scholar 

  8. Hoitsma E, Faber CG, Drent M, Sharma OP (2004) Neurosarcoidosis: a clinical dilemma. Lancet Neurol 3:397–407

    Article  PubMed  Google Scholar 

  9. Nowak DA, Widenka DC (2001) Neurosarcoidosis: a review of its intracranial manifestation. J Neurol 248:363–372

    Article  PubMed  CAS  Google Scholar 

  10. Zajicek JP, Scolding NJ, Foster O et al (1999) Central nervous system sarcoidosis–diagnosis and management. QJM Month J Assoc Phys 92:103–117

    CAS  Google Scholar 

  11. Toogood AA, Stewart PM (2008) Hypopituitarism: clinical features, diagnosis, and management. Endocrinol Metab Clin N Am 37:235–261, x

  12. Loh JA, Verbalis JG (2008) Disorders of water and salt metabolism associated with pituitary disease. Endocrinol Metab Clin N Am 37:213–234, x

  13. Molitch ME, Clemmons DR, Malozowski S et al (2011) Evaluation and treatment of adult growth hormone deficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab 96:1587–1609

    Article  PubMed  CAS  Google Scholar 

  14. Bullmann C, Faust M, Hoffmann A et al (2000) Five cases with central diabetes insipidus and hypogonadism as first presentation of neurosarcoidosis. Euro J Endocrinol Euro Fed Endocr Soc 142:365–372

    Article  CAS  Google Scholar 

  15. Tabuena RP, Nagai S, Handa T et al (2004) Diabetes insipidus from neurosarcoidosis: long-term follow-up for more than eight years. Intern Med 43:960–966

    Article  PubMed  Google Scholar 

  16. Bihan H, Christozova V, Dumas JL et al (2007) Sarcoidosis: clinical, hormonal, and magnetic resonance imaging (MRI) manifestations of hypothalamic–pituitary disease in 9 patients and review of the literature. Medicine 86:259–268

    Article  PubMed  Google Scholar 

  17. Langrand C, Bihan H, Raverot G et al (2012) Hypothalamo-pituitary sarcoidosis: a multicenter study of 24 patients. QJM Month J Assoc Phys 105:981–995

    CAS  Google Scholar 

  18. Smith D, Cullen MJ (2005) Two cases of hypothalamic–pituitary sarcoidosis. J R Soc Med 98:167–169

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  19. Dessouky OY (2008) Isolated sinonasal sarcoidosis with intracranial extension: case report. Acta Otorhinolaryngol Italica Organo Ufficiale Della Soc Italiana Otorinolaringologia e Chirurgia Cervico-Facciale 28:306–308

    CAS  Google Scholar 

  20. Caturegli P, Newschaffer C, Olivi A et al (2005) Autoimmune hypophysitis. Endocr Rev 26:599–614

    Article  PubMed  CAS  Google Scholar 

  21. Stuart CA, Neelon FA, Lebovitz HE (1980) Disordered control of thirst in hypothalamic–pituitary sarcoidosis. N Engl J Med 303:1078–1082

    Article  PubMed  CAS  Google Scholar 

  22. Lustig RH (2008) Hypothalamic obesity: causes, consequences, treatment. Pediatr Endocrinol Rev 6:220–227

    PubMed  Google Scholar 

  23. Arle JE, Judkins AR, Kotapka MJ (1999) Neurosarcoidosis presenting in the pituitary gland with normal endocrine studies. Skull Base Surg 9:149–153

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  24. Phillips YL, Eggenberger ER (2010) Neuro-ophthalmic sarcoidosis. Curr Opin Ophthalmol 21:423–429

    Article  PubMed  Google Scholar 

  25. Koczman JJ, Rouleau J, Gaunt M et al (2008) Neuro-ophthalmic sarcoidosis: the University of Iowa experience. Semin Ophthalmol 23:157–168

    Article  PubMed  Google Scholar 

  26. Menezo V, Lobo A, Yeo TK et al (2009) Ocular features in neurosarcoidosis. Ocular Immunol Inflamm 17:170–178

    Article  Google Scholar 

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Authors and Affiliations

  1. Specialty Care, Atlanta Veterans Affairs Medical Center, Decatur, GA, USA

    Jeremy Anthony

  2. Division of Endocrinology, Department of Medicine, Emory University, 1365 B Clifton Rd., NE, B6209, Atlanta, GA, 30322, USA

    Jeremy Anthony & Adriana Ioachimescu

  3. Department of Neurology, Emory University, Atlanta, GA, USA

    Gregory J. Esper

  4. Department of Neurosurgery, Emory University, Atlanta, GA, USA

    Adriana Ioachimescu

Authors
  1. Jeremy Anthony
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  2. Gregory J. Esper
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  3. Adriana Ioachimescu
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Correspondence to Adriana Ioachimescu.

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The authors did not receive funding for this research and have no conflicts of interest.

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Anthony, J., Esper, G.J. & Ioachimescu, A. Hypothalamic–pituitary sarcoidosis with vision loss and hypopituitarism: case series and literature review. Pituitary 19, 19–29 (2016). https://doi.org/10.1007/s11102-015-0678-x

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  • DOI: https://doi.org/10.1007/s11102-015-0678-x

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