Formulation of the concept of a "general adaptation syndrome" by Selye1 and speculation that this might be of significance in the pathogenesis of the connective tissue diseases stimulated many investigations as to the functional status of the pituitary-adrenal axis in these disease states. Much impetus for this approach was provided by the favorable effect of cortisone on acute, active rheumatoid arthritis.2 West3 and Kelley and Ely4 have reviewed the pattern of pituitary-adrenal function which has emerged from these studies. It would appear that although no defect has been delineated which is as striking and clear-cut as those present in the congenital enzymatic defects of the adrenogenital syndrome, adrenocortical function cannot be considered wholly normal in the majority of patients with connective tissue disease.
Adrenal steroidogenesis is a complex process. The biosynthetic pathways involved are known in some detail.5 In the Figure is shown a
HUGHES ER, ELY RS, KELLEY VC. Plasma Adrenocortical Hormones in Connective Tissue Diseases. Am J Dis Child. 1962;104(6):610-613. doi:10.1001/archpedi.1962.02080030610005