Endocrine Abstracts

We present the case of a 38 yo female with primary adrenal insufficiency and multiple autoimmune conditions. She initially presented to the medical assessment unit in late 2017 with a two week history of fatigue. She was noted to be hypotensive, hyponatraemic at 130 mmol/l and a random cortisol returned at 82 nmol/l. Short synacthen testing confirmed adrenal insufficiency with a 0 minute cortisol of 74 nmol/l and 60 minute cortisol of 70 nmol/l. ACTH was elevated at 212.6 pmol/l and 21-hydroxylase antibodies were positive. She was commenced on hydrocortisone and fludrocortisone replacement with doses adjusted based on symptoms, cortisol day curve levels and renin levels respectively. She had several admissions due to viral illnesses requiring stress dose steroids and had difficulty reducing the hydrocortisone dose due to symptoms so is maintained on hydrocortisone 15 mg mane, 10 mg midi and 5 mg tarde as per patient preference. At the time of presentation LFTs were deranged however a non-invasive liver screen was unremarkable. Anti-TTG and anti-endomysial antibodies were mildly raised so she proceeded to an OGD; histology confirmed coeliac disease and a gluten free diet was recommended. Despite adherence to the diet she had a persistently elevated ALT and IgG. Repeat antibody testing returned a positive ANA and anti-smooth muscle antibody. A liver biopsy confirmed a diagnosis of autoimmune hepatitis. Following a period of monitoring she was commenced on budesonide and subsequently azathioprine with resultant improvement in LFTs. Six months after being diagnosed with adrenal insufficiency she reported amenorrhoea and on investigation was noted to have an oestradiol level of <37 pmol/l with LH 19.8 IU/l and FSH 15.1 IU/l, with the same pattern on repeat testing, consistent with premature ovarian insufficiency and hormone replacement therapy was commenced. She desired a pregnancy and underwent IVF with a successful pregnancy on her 4th cycle and then had a subsequent pregnancy with a spontaneous conception. Her case has been challenging to manage due to multiple new diagnoses of autoimmune conditions, admissions with crises, the use of budesonide with supraphysiological doses of hydrocortisone and navigating IVF and pregnancy with these.