CASE REPORT Focal segmental glomerulosclerosis in a patient with isolated ACTH deficiency and reversible hypothyroidism Shunsuke Yamada Sachiko Bandai Kohsuke Masutani Akihiro Tsuchimoto Hideko Noguchi Mikio Munakata Kazuhiko Tsuruya Mitsuo Iida Received: 21 April 2009 / Accepted: 2 September 2009 / Published online: 7 October 2009 Ó Japanese Society of Nephrology 2009 Abstract A 23-year-old man was admitted to our hospital for fatigue, anorexia, proteinuria, and peripheral edema. Proteinuria was first pointed out at the age of 15, but no further studies were performed. Six years prior to admis- sion, the patient noted becoming easily fatigued. Labora- tory tests on admission showed marked peripheral eosinophilia (29.2%, count: 1,071/lL) and proteinuria. Endocrinological studies revealed isolated adrenocortico- tropic hormone deficiency with primary hypothyroidism, but a lack of autoimmune thyroiditis. Renal biopsy showed focal segmental glomerulosclerosis. Hydrocortisone ther- apy (30 mg/day) for isolated adrenocorticotropic hormone deficiency resulted in marked improvement of adrenal and thyroid functions, and amelioration of proteinuria (from 2.8 to 1.0 g/day) over a two-month period. Renal function remains normal at five years after the start of hydrocorti- sone treatment. The findings suggest that both hydrocorti- sone therapy and normalized thyroid hormone status played a pivotal role in the improvement of proteinuria associated with focal segmental glomerulosclerosis. Keywords Adrenal insufficiency Á Focal segmental glomerulosclerosis Á Isolated adrenocorticotropic hormone deficiency Á Reversible hypothyroidism Introduction Focal segmental glomerulosclerosis (FSGS) is a form of glomerulonephritis often associated with mild to severe proteinuria [1, 2]. FSGS is categorized into primary and secondary forms, and the latter form is occasionally accompanied by endocrinological disorders [3–5]. Isolated deficiency of adrenocorticotropic hormone (ACTH) is a rare endocrinological disorder characterized by congenital or acquired loss of ACTH and resultant adrenal insufficiency, often presented with fatigue, leth- argy, central nervous disorder, hyponatremia, hyperkale- mia and eosinophilia [6, 7]. Several cases with glomerulonephritis, including FSGS, are accompanied by hypothalamic–pituitary–adrenal (HPA) axis dysfunction [8, 9]. To our knowledge, there are no reports of patients with FSGS and isolated ACTH deficiency. We present here a rare case of a patient with FSGS accompanied by isolated ACTH deficiency and reversible primary hypothyroidism. In the present case, the moderate level of proteinuria improved after treatment with a ther- apeutic dose of hydrocortisone. Case report A 23-year-old Japanese man was admitted to Kyushu University Hospital for general fatigue, anorexia and peripheral edema. The patient had been in good health with no history of serious conditions during infancy or S. Yamada Á S. Bandai Á K. Masutani Á A. Tsuchimoto Á H. Noguchi Á K. Tsuruya Á M. Iida Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan M. Munakata Department of Pathology, Fukuoka Red Cross Hospital, Fukuoka, Japan K. Tsuruya (&) Department of Integrated Therapy for Chronic Kidney Disease, Graduate School of Medical Sciences, Kyushu University, Maidashi 3-1-1, Higashi-ku, Fukuoka 812-8582, Japan e-mail: tsuruya@intmed2.med.kyushu-u.ac.jp 123 Clin Exp Nephrol (2010) 14:168–172 DOI 10.1007/s10157-009-0228-9