• Dennis Delasi Nyanyo, Masaaki Mikamoto, Francesca Galbiati, Ilan Remba-Shapiro, Kevin Bode, Sara Schoenfeld, Pamela S Jones, Brooke Swearingen, and Lisa B Nachtigall.
• The Neuroendocrine Unit, Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts (D.D.N., F.G., I.R., K.B., L.B.N.).
• Ann. Intern. Med. 2024 Mar 1; 177 (3): 315323315-323.

BackgroundGlucocorticoids suppress inflammation. Autoimmune disease may occur after remission of Cushing's disease (CD). However, the development of autoimmune disease in this context is not well described.ObjectiveTo determine 1) the incidence of autoimmune disease in patients with CD after surgical remission compared with patients with nonfunctioning pituitary adenomas (NFPAs) and 2) the clinical presentation of and risk factors for development of autoimmune disease in CD after remission.DesignRetrospective matched cohort analysis.SettingAcademic medical center/pituitary center.PatientsPatients with CD with surgical remission and surgically treated NFPA.MeasurementsCumulative incidence of new-onset autoimmune disease at 3 years after surgery. Assessment for hypercortisolemia included late-night salivary cortisol levels, 24-hour urine free cortisol (UFC) ratio (UFC value divided by the upper limit of the normal range for the assay), and dexamethasone suppression tests.ResultsCumulative incidence of new-onset autoimmune disease at 3 years after surgery was higher in patients with CD (10.4% [95% CI, 5.7% to 15.1%]) than in those with NFPAs (1.6% [CI, 0% to 4.6%]) (hazard ratio, 7.80 [CI, 2.88 to 21.10]). Patients with CD showed higher prevalence of postoperative adrenal insufficiency (93.8% vs. 16.5%) and lower postoperative nadir serum cortisol levels (63.8 vs. 282.3 nmol/L) than patients with NFPAs. Compared with patients with CD without autoimmune disease, those who developed autoimmune disease had a lower preoperative 24-hour UFC ratio (2.7 vs. 6.3) and a higher prevalence of family history of autoimmune disease (41.2% vs. 20.9%).LimitationThe small sample of patients with autoimmune disease limited identification of independent risk factors.ConclusionPatients achieving surgical remission of CD have higher incidence of autoimmune disease than age- and sex-matched patients with NFPAs. Family history of autoimmune disease is a potential risk factor. Adrenal insufficiency may be a trigger.Primary Funding SourceRecordati Rare Diseases Inc.

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