Abstract
ObjectiveHeterogeneous clinical characteristics are observed in acquired isolated adrenocorticotropic hormone (ACTH) deficiency (IAD); however, its classification remains to be established because of its largely unknown pathophysiology. In IAD, anti-pituitary antibodies have been detected in some patients, although their significance remains unclear. Therefore, this study aimed to classify patients with IAD and to clarify the significance of anti-pituitary antibodies.Design and MethodsWe analyzed 46 consecutive patients with IAD. Serum anti-pituitary antibodies were analyzed via immunofluorescence staining using a mouse pituitary tissue. Principal component and cluster analyses were performed to classify IAD patients based on clinical characteristics and autoantibodies.ResultsImmunofluorescence analysis using the sera revealed that 58% of patients showed anti-corticotroph antibodies and 6% of patients showed anti-follicular stellate cell (FSC) antibodies. Principal component analysis demonstrated that three parameters could explain 70% of the patients. Hierarchical cluster analysis showed three clusters: Groups A and B comprised patients who were positive for anti-corticotroph antibodies, and plasma ACTH levels were extremely low. Groups A and B comprised middle-aged or elderly men and middle-aged women, respectively. Group C comprised patients who were positive for the anti-FSC antibody and elderly men; plasma ACTH levels were relatively high.ConclusionsPatients with IAD were classified into three groups based on clinical characteristics and autoantibodies. The presence of anti-corticotroph antibody suggested severe injury to corticotrophs. This new classification clearly demonstrated the heterogeneity in the pathogenesis of IAD.
Highlights
Isolated adrenocorticotropic hormone (ACTH) deficiency (IAD) is characterized by secondary adrenal insufficiency with low or no cortisol production and normal secretion of pituitary hormones other than ACTH [1]
IAD is categorized as congenital [e.g., T-box transcription factor (TPIT) or proopiomelanocortin (POMC) mutation [2, 3]] and acquired
Some cases of acquired IAD have been reportedly associated with autoimmune diseases, including autoimmune thyroid diseases [5]; anti-pituitary antibodies, such as anti-corticotroph antibody, in the serum [6, 7]; and hypophysitis related with anti-programmed death 1 or antiprogrammed death ligand 1 antibodies [8]
Summary
Isolated adrenocorticotropic hormone (ACTH) deficiency (IAD) is characterized by secondary adrenal insufficiency with low or no cortisol production and normal secretion of pituitary hormones other than ACTH [1]. Some cases of acquired IAD have been reportedly associated with autoimmune diseases, including autoimmune thyroid diseases [5]; anti-pituitary antibodies, such as anti-corticotroph antibody, in the serum [6, 7]; and hypophysitis related with anti-programmed death 1 or antiprogrammed death ligand 1 antibodies [8]. These data strongly suggest that autoimmune etiology is involved in the pathogenesis of acquired IAD [1, 9].
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
