polymyalgia rheumatica ( pmr ) is a common disorder in the elderly , characterized by aching and morning stiffness in the neck , shoulders , and pelvic girdle , along with constitutional symptoms ( malaise , weight loss , fever).1 cutolo and colleagues have proposed a hypothesis that pmr may share some clinical features with the steroid - withdrawal syndrome or adrenal insufficiency , such as dramatic and rapid disappearance of the symptoms after glucocorticoid treatment.14 here we present a case of pmr , which was indicated to have transient acth deficiency . a 79-year - old man was admitted to department of neurology because of severe bilateral shoulder pain and proximal muscle weakness of lower extremities , which began one month ago . neurological examinations indicated no abnormality . the neurologist suspected a possibility of muscle weakness caused by some endocrinopathy , and performed endocrinological examinations , which revealed low serum cortisol ( 1.8 g / day ) and undetectable plasma acth ( < 5 pg / ml ) level . since these results indicated a possibility of acth deficiency , the patient was transferred to department of diabetes and endocrinology . other pituitary hormone and thyroid hormone levels were normal , including their responses to provocative tests , such as thyrotropin - releasing hormone , luteinizing hormone - releasing hormone , and growth hormone - releasing hormone . nonsteroidal anti - inflammatory drug , ( etodalac 600 mg / day , po ) was given , which caused some , but not complete , improvement of his symptoms . serum crp was positive ( 11.64 mg / dl ) , and erythrocyte sedimentation rate was increased ( 66 mm / hr ) . acth showed slightly blunted response to crh from slightly high basala level ( 238 to 266 pg / ml ) . rapid acth stimulation proved a slightly blunted increase in cortisol from slightly high basal level ( 20.0 to 25.7 g / dl ) . hydrocortisone ( 10 mg / day ) was started with a rapid and complete improvement of his symptoms , such as shoulder pains , after 2 days . both serum acth and urinary 17-ohcs levels were also became normal range after three weeks ( 13.9 pg / ml and 6.0 mg / day , respectively ) . based on those results and clinical course , ie , elevated erythrocyte sedimentation rate , negative rheumatoid factor and the typical symptoms , which showed improvement to glucocorticoid therapy , the final diagnosis was pmr , which was associated with transient acth deficiency . this patient was found to have pmr and transient acth deficiency , although he did not show flexion contracture sometimes associated with adrenocortical insufficiency.5 cutolo and his colleagues suggested that pmr shares clinical features with the steroid - withdrawal syndrome or adrenal insufficiency , such as the abrupt onset , the clinical response to glucocorticoid therapy.13 they found that reduced production of adrenal hormone ( cortisol , dehydroepiandrostendione sulfate ) at baseline in patients with active and untreated pmr.1 acth induced a significantly higher peak of 17-hydroxyprogesterone ( 17-ohp ) in patients with pmr than in controls , although crh stimulation or acth simulation evoked a normal acth and cortisol response , respectively , suggesting that the defect seems mainly related to cyp21a2 21-hydroxylase deficiency.1,3 in our patients , the treatment with hydrocortisone , 10 mg per day , resulted in a improvement of his symtoms . this dose is generally not enough for management of pmr , which requires generally prednisone , 10 to 20 mg per day equivalent , which is in the range of 40 to 80 or so mg of hydrocortisone . this dose of 10 mg of hydrocortisone likely is a good dose for adrenal insufficiency , especially if transient , but generally not for pmr . these results suggest that hypothalamo - pituitary adrenal ( hpa ) axis may play a role in pathogenesis of pmr , justifying use of glucocorticoid in its treatment . it was also indicated that pmr patients without corticosteroid treatment had higher serum cortisol in relation to il-6 compared to pmr patients without long - standing disease and with corticosteroid treatment.3 however , the cortisol level in pmr patients was lower than expected under inflammatory conditions.3 they have proposed a hypothesis that pmr is associated with a decrease in hpa axis responsiveness to inflammatory stimuli , such as il-6 , which may be linked to aging process.3 in contrast , pacheco et al recently reported that the responses of cortisol and dehydroepiandrostendione to low - dose acth challenge were not significantly lower in patients with pmr than in healthy subjects.4 they also found that the acute phase reactants did not show a clear relationship with adrenal hormones , indicating that the abnormalities of hpa found in pmr were the consequences of chronic illness rather than a crucial factor contributing to the pathogenesis of pmr.4 this is the first report of a case of pmr , in which the hpa axis was examined in its very acute phase . it was indicated that the case was associated with the transient adrenocortical hypofunction , which was recovered during a short time . it is therefore possible that pmr may show a different responsiveness of hpa axis depending on its phases , and that the discrepancies between the reports1,6 might be attributable to the different phase of pmr examined .
we report a case of 79-year - old man , who was diagnosed to have transient acth deficiency associated with polymyalgia rheumatica ( pmr ) . the patient presented with sudden onset bilateral shoulder pain , which was gradually aggravated . 
 plasma acth was undetectable , and both serum cortisol and urinary 17-ohcs were very low . 
 other pituitary hormones were normal , suggesting that hypothalamo - pituitary - adrenal ( hpa ) axis is selectively damaged . 
 however , within several weeks , plasma acth returned to normal , and showed a normal increase response to corticotropin - releasing hormone stimulation test . 
 these results indicated that acth deficiency was only transient . 
 after hydrocortisone ( 10 mg / day ) was administered , his symptoms became suddenly improved . 
 based on those results and clinical course , ie , elevated erythrocyte sedimentation rate , negative rheumatoid factor and the typical symptoms , which showed improvement to glulcocorticoid therapy , the final diagnosis was pmr , which was associated with transient acth deficiency.this is the first report of a case of pmr , in which the hpa axis was examined in its very acute phase . 
 it was demonstrated that the case was associated with the transient adrenocortical hypofunction , which was recovered during a short time . 
 it is therefore possible that pmr may show a different responsiveness of hpa axis depending on its phases .