Primary adrenal insufficiency: a Portuguese multicentre study by the adrenal tumour study group

Lia Ferreira; Joao Silva; Ana Martins; Carlos Belo; Diana Oliveira; Helder Simoes; Isabel Paiva; Joana Guimaraes; Marta Ferreira; Rita Silva; Susana Garrido; Tiago Silva; Vera Fernandes; Maria Lopes Pereira

doi:10.1530/endoabs.49.EP57

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Endocrine Abstracts (2017) 49 EP57 | DOI: 10.1530/endoabs.49.EP57

ECE2017 Eposter Presentations: Adrenal and Neuroendocrine Tumours Adrenal cortex (to include Cushing's) (86 abstracts)

Primary adrenal insufficiency: a Portuguese multicentre study by the adrenal tumour study group

Lia Ferreira ¹ , João Silva ² , Ana Martins ³ , Carlos Belo ⁴ , Diana Oliveira ⁵ , Hélder Simões ⁶ , Isabel Paiva ⁵ , Joana Guimarães ⁷ , Marta Ferreira ⁸ , Rita Silva ⁹ , Susana Garrido ¹⁰ , Tiago Silva ¹¹ , Vera Fernandes ¹² & Maria Lopes Pereira ¹²

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Author affiliations

¹Centro Hospitalar do Porto, Porto, Portugal; ²Hospital das Forças Armadas, Lisboa, Portugal; ³Centro Hospitalar Lisboa Norte, Lisboa, Portugal; ⁴Centro Hospitalar Lisboa Ocidental, Lisboa, Portugal; ⁵Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal; ⁶Instituto Português de Oncologia Lisboa, Lisboa, Portugal; ⁷Centro Hospitalar do Baixo Vouga, Aveiro, Portugal; ⁸Centro Hospitalar Leiria, Leiria, Portugal; ⁹Centro Hospitalar São João, Porto, Portugal; ¹⁰Hospital Tâmega e Sousa, Penafiel, Portugal; ¹¹Hospital Garcia da Orta, Lisboa, Portugal; ¹²Hospital de Braga, Braga, Portugal.

Introduction: Primary adrenal insufficiency (PAI) is a rare but severe and potentially life-threatening condition. There are no studies characterizing portuguese patients with PAI.

Aims: To characterize the clinical presentation, diagnostic workup, treatment and follow-up of patients with confirmed PAI.

Methods: A multicentre retrospective study of PAI patients followed in 12 portuguese hospitals.

Results: We investigated 278 patients with PAI (55.8% were females) with mean age of diagnosis of 33.6±19.3 years, 7.3 years after the beginning of the symptom presentation. The most frequently reported symptoms and signs were asthenia (60.1%), hyperpigmentation (55.0%), weight loss (43.2%), hypotension (42.8%) and hypoglycaemia (8.6%). Hyponatremia was documented in 36.3% of cases and hyperkalaemia in 25.9%; 29.1% of patients were diagnosed in adrenal crisis. In 122 patients the diagnosis was established using morning cortisol <5 g/dl and plasma ACTH 2-fold the upper limit of the reference range and in 39 cases by the corticotropin stimulation test. The main causes were autoimmune adrenalitis (140 cases), idiopathic (48 cases), congenital adrenal hyperplasia (27 cases) and tuberculosis (17 cases). Concerning associated autoimmune diseases, the most common were autoimmune thyroiditis (81 patients) and type 1 diabetes mellitus (25 patients). Seventy-nine percent were treated with hydrocortisone (mean dose 26.3±8.3 mg/day) in three (57.5%), two (37.4%), one (3.7%) or four (0.5%) daily doses; the remainder with prednisolone (10.1%), dexamethasone (6.2%) and methylprednisolone (0.7%); 66.2% were medicated with fludrocortisone (median dose of 100 μg/day). Since diagnosis 33.5% have been hospitalized due to disease decompensation. At the last consultation, 17.2% of the patients had complaints (7.6% asthenia and 6.5% depression) and 9.7% presented changes in the ionogram.

Conclusion: This is the first multicentre Portuguese study about PAI. As described in international studies a significant number of PAI patients continue to have symptoms of over or under-substitution despite optimal steroid replacement.