Artikel
Treatment of hypophysitis by high dose methylprednisolone pulse therapy
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Veröffentlicht: | 20. Mai 2009 |
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Objective: Hypophysitis is a rare condition. We present 22 cases from our department initially suspicious of hypophysitis. We review the clinical course, management, outcome and the differential diagnosis.
Methods: The charts during a 15-year period were reviewed for cases initially suspicious of hypophysitis. The clinical and endocrinological data as well as imaging material and the biopsy specimen were reanalysed.
Results: 22 patients were identified suspicious of hypophysitis, n=16 (73%) were female and n=6 (27%) male. The mean age of manifestation was 38.1 (range: 15-65) years. The main clinical presentations were anterior pituitary axis failure (n=16; 73%), diabetes insipidus (n=9; 41%) and headaches (n=8; 36%). Eight cases (36%) were associated with autoimmune diseases, most commonly with thyreoditis (n=4).
In 17 patients the presumptive diagnosis of hypophysitis was established by means of clinical presentation, endocrinological and MRI-findings. They were all treated by High Dose Methylprednisolone Pulse Therapy (HDMT). Eight of these patients improved clinically, endocrinologically or on MRI, and remained improved for a mean follow-up of 3.9 years. Eight patients did not improve or worsened following HDMT and another one had worsened after a short period of clinical improvement. Thus, a biopsy was performed in 5 patients in order to clarify histology disclosing germinoma, Langerhanscell histiocytosis, metastasis (each one case) and two cases of lymphocytic hypophysitis. Of the other 4 patients, one refused biopsy, in three patients systemic diseases were revealed.
In 5 patients the diagnosis was established primary by biopsy (4 lymphocytic, one granulomatous hypophysitis). 4 of these patients were treated thereafter by HDMT. A clinical, endocrinological or MRI improvement occurred in all 4 patients, and lasted for a mean follow-up of 9 years. However, in one of these patients, histology was reconsidered to be a xanthomathous hypophysitis. Another patient developed neurosarcoidosis in the further follow-up.
Thus, an overall of 13 of 15 clinically or histologically diagnosed patients with hypophysitis improved following HDMT for a mean follow-up of 5.2 years.
Conclusions: HDMT can be considered as first-line therapy of hypophysitis. However, if the presumptive hypophysitis does not improve following HDMT, histological verification of the diagnosis is mandatory.