Sporadic (i.e. apparently not familial) PBMAH usually presents with mild hypercortisolism [19], but germline ARMC5 pathogenic variants, responsible for around 20 to 25% of PBMAH index-cases, are associated with a more pronounced phenotype than observed in wild-type patients, in terms of cortisol secretion, adrenal morphology and complications of Cushing’s syndrome [13, 20]. This evidence concerns the gene ARMC5 and Cushing syndrome due to macronodular adrenal hyperplasia.