Hypothalamic-Pituitary-Adrenal Axis Dysfunction in Cases with Polymyalgia Rheumatica

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: SUN 50-71-HPA Axis
Clinical
Sunday, June 16, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board SUN-63
Shinsuke Tokumoto*, Hisato Tatsuoka, Yoshiyuki Hamamoto, Kanta Fujimoto, Atsuko Matsuoka, Sachiko Honjo, Yoshiharu Wada, Hiroki Ikeda and Hiroyuki Koshiyama
Tazuke Kofukai Foundation, Medical Research Institute, Kitano Hospital, Osaka, Japan
Introduction:Polymyalgia rheumatica(PMR) is one of the most prevalent inflammatory diseases in elderly people characterized by acute onset of muscle pain and stiffness in the shoulders and pelvic girdles. Although its etiology has not been elucidated, the correspondence of the clinical features with steroid withdrawal syndrome or adrenal insufficiency suggests the abnormality of Hypothalamic-Pituitary-Adrenal (HPA) axis as one of predisposing factors(1). Here, by reporting 2 cases and reviewing literature in Japan, we provide new evidence for HPA axis dysfunction in PMR. Clinical cases:The first case was a 61 year-old female diagnosed as adrenal insufficiency and treated with hydrocortisone (HDC) 5mg/day. She was referred to our center for muscle pain in the shoulder and pelvic girdle, fever, and morning stiffness sustained for one month. Upon admission, we found elevated CRP(27.5 mg/dl) and high ESR levels(52 mm/h). Ruling out other autoimmune diseases, PMR was diagnosed based on Bird/Wood(1979) criteria. The results with rapid ACTH stimulation test and CRH/TRH/GRF/GnRH loading test indicated isolated ACTH or CRH deficiency. Administration of HDC 100mg/day improved her symptoms. Finally she was discharged by continuing HDC 10mg/day without relapse afterward. The second case was a 79 year-old male complaining of bilateral shoulder pain, morning stiffness and difficulty in elevating upper limbs with elevated CRP (11.6 mg/dl) and ESR (66 mm/hr). Laboratory examination revealed low baseline plasma ACTH (<5.0 pg/ml) and low serum cortisol(1.8 μg/dl) in early morning. Isolated ACTH deficiency was suspected. Thereafter, basal ACTH and cortisol levels were increased after rapid ACTH stimulation test, but CRH loading test indicated no response despite of normal basal ACTH level, suggesting that his adrenal insufficiency was temporal. There was no abnormal finding of pituitary gland in magnetic resonance imaging (MRI). After administration of HDC 10mg/day for 2 days, his symptoms were eliminated. Discussion:We experienced 2 cases of PMR concomitantly with HPA axis dysfunction, one case with central adrenal insufficiency and the other with reversible ACTH deficiency. There have been several reports suggesting HPA axis abnormality in cases with PMR. Taken together, HPA axis dysfunction and PMR might share some etiologies. Conclusion:HPA axis dysfunction is considered to underlie PMR, justifying the need to evaluate routinely the function of HPA axis in PMR patients.

(1)Cutolo M, Straub RH. Polymyalgia rheumatic:evidence for a hypothalamic-pituitary-adrenal axis-driven disease. ClinExpRheumatol 2000;18:655-658.

Nothing to Disclose: ST, HT, YH, KF, AM, SH, YW, HI, HK

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