Hypophysitis: clinical experience in an Australian case series — ASN Events

Hypophysitis: clinical experience in an Australian case series (#322)

Monique Costin 1 , Vicki Maltby 2 3 , Anna Duke 4 , Mark McLean 4 , Amy Wagstaff 4 , David Chipps 4 , Andrew Weissberger 1 , Roderick Clifton-Bligh 5 , Suja Padmanabhan 4 , Aidan McElduff 6 , Jane Holmes-Walker 4 , Weiwen Chen 1 , Rachel Bradbury 7 , Richard Harvey 8 , Patricia Crock 2 3 , Ganesh Chockalingam 9 , Katherine Samaras 1 , Peter Earls 10 , Ann McCormack 1
  1. Department of Endocrinology, St Vincent's Hospital, Darlinghurst, NSW, Australia
  2. John Hunter Children’s Hospital, New Lambton Heights, NSW, Australia
  3. University of Newcastle, Hunter Medical Research Institute, New Lambton Heights, NSW, Australia
  4. Department of Endocrinology, Westmead Hospital, Westmead, NSW, Australia
  5. Department of Endocrinology, Royal North Shore Hospital, St Leonards, NSW, Australia
  6. Northern Sydney Endocrine Centre, St Leonards, NSW, Australia
  7. Specialist Medical Centre, Hawksbury Rd, Westmead, NSW, Australia
  8. Sydney Ear Nose and Throat Clinic, Darlinghurst, NSW, Australia
  9. Department of Endocrinology, Canberra Hospital, Canberra, ACT, Australia
  10. Department of Anatomical Pathology, St Vincent's Hospital, Darlinghurst, NSW, Australia

Introduction: Hypophysitis, an inflammatory condition of the pituitary, is uncommon and can be difficult to diagnose clinically. Knowledge remains limited regarding its natural history and the best approach to management.

Methods: A multi-center clinical case series was assembled. Hypophysitis was diagnosed histopathologically or clinically in the presence of: hypopituitarism, DI, sella/pituitary stalk-based mass on MRI, autoimmune history, positive pituitary antibodies or ipilimumab use.

Results: Data was collected on 20 patients (6 men, 14 women): 14 had histologically-confirmed hypophysitis, 6 clinically-diagnosed. Average follow-up was 61 months. Hypopituitarism-related symptoms was the most common presentation, confirmed in 19 patients: 11 had 2-3 affected axes, 4 isolated ACTH deficiency and 6 DI. Headache was common (12), with abnormal vision in 5. 9 patients had an autoimmune history. Pituitary antibodies were tested in 2 patients, both positive, one with no known autoimmune disease. A mass lesion, commonly enhancing, was the most frequent radiologic abnormality (14), with an enlarged pituitary in 5. 7 patients had stalk thickening with a dural tail in 3. One patient with ipilimumab use had no significant MRI abnormalities. 14 patients underwent pituitary surgery. Hypophysitis was suspected preoperatively in only 4; the remaining patients had a presumed macroadenoma. Histopathological-diagnoses were lymphocytic (10), granulomatous (3) and xantho-granulomatous (1). Postoperatively, headaches improved in 9/10, pituitary function improved in only 2, radiologic changes improved in 5/10. 3 patients had symptomatic relapses treated with supra-physiological corticosteroids; in 2 with multiple relapses steroid-sparing agents were successfully used. 10 of 11 with long-term control received only replacement corticosteroid doses. The 6 patients with clinically-diagnosed hypophysitis had less severe clinical presentation and more subtle MRI findings. Apart from 1 case with DI alone, all had ACTH deficiency with other affected axes in 3. Symptoms resolved in all patients with only pituitary replacement therapy. 2 patients with progress imaging had no progression.

Conclusions: In clinically-suspected hypophysitis, patients appear to do well with pituitary replacement therapy alone. Patients with mass lesions may benefit from surgical debulking for symptom relief with supra-physiological and steroid-sparing agents reserved for the minority with relapsing disease.