Adrenal Insufficiency in Cystic Fibrosis: A Rare Phenomenon?

Sébastien Préville-Ratelle,Isabelle Bourdeau,Adèle Coriati,Yves Berthiaume,Aurélie Ménard,François Tremblay

doi:10.1155/2018/3629031

Abstract

Background The prevalence of adrenal insufficiency (AI) in cystic fibrosis (CF) is unknown. The frequent use of glucocorticoids (inhaled or systemic) may induce the long-term suppression of the hypothalamic-pituitary-adrenal axis. Methods We reviewed the results of adrenocorticotropic hormone (ACTH) stimulation tests done over a 10-year period to evaluate adrenal function in 69 CF patients of the CHUM CF clinic. Clinical characteristics of AI patients were compared to adrenal-sufficient (AS) patients. Results AI was confirmed in 33 of the 69 CF patients. A higher rate of dysglycemia (P=0.022) and of Aspergillus positive culture (P=0.006) was observed in AI patients compared to AS patients. Weight, CFTR genotype, and pulmonary function were comparable between AI and AS patients. The use of systemic corticosteroids (SC) prior to the diagnosis of AI was observed in 42.4% of patients. Compared to AI patients without SC, SC-treated AI patients were older and had a higher rate of allergic bronchopulmonary aspergillosis. Conclusion This study is the first to systematically examine the presence of AI in the largest cohort of CF patients studied to date with a prevalence of 8%. Patients treated with corticosteroids and those colonized with Aspergillus have a greater risk of AI.

Highlights

Adrenal insufficiency (AI) is the result of decreased hormonal production from the cortex of the adrenal gland
Only individual case reports relating cystic fibrosis (CF) and AI have appeared in the literature. is is the first study that characterizes a large group of adult patients with CF that had developed AI
Our observations show that, compared to the AS subgroup, the AI subgroup of CF patients exhibited a higher frequency of dysglycemia and had a higher percentage of patients colonized with Aspergillus/fungi

Summary

Introduction

Adrenal insufficiency (AI) is the result of decreased hormonal production from the cortex of the adrenal gland. To ensure appropriate testing at our CHUM clinic, the hospital pharmacist is responsible for preparing the Cortrosyn syringe and the nurse uses this preparation to perform the test In both tests, plasma cortisol levels were measured at baseline as well as 30 and 60 minutes after intravenous injection. Demographics (age, sex, weight, and body mass index (BMI)), pulmonary disease characteristics (forced expiratory volume in 1 sec (FEV1), forced vital capacity (FVC), residual volume (RV), oxygen dependence, CFTR genotype, and noninvasive ventilation support), comorbidities (pancreatic and hepatic dysfunction as evidenced by abnormal level of hepatic enzymes or presence of cirrhosis), dysglycemia (diabetes or impaired glucose tolerance), allergic bronchopulmonary aspergillosis (ABPA), microbiological data (bacteria, fungi, and non-TB mycobacteria), biochemical and hematological data (median value over the past year of hemoglobin and white blood cells, and fasting serum cortisol), and results of the functional ACTH test were collected from all of the patients. A probability value of P ≤ 0.05 was considered statistically significant

Results

Discussion

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Systemic corticosteroids