title: Selective hypoaldosteronism in a patient with Sjögren's syndrome: insensitivity to angiotensin II.
abstract: A 51-year-old Japanese woman with hypokalemia due to distal renal tubular acidosis associated with Sjögren's syndrome exhibited a decreased plasma aldosterone level despite elevated plasma renin activity. Our studies revealed selective hypoaldosteronism with normal adrenoglucocorticoid function. In the presence of a low level of serum potassium (3.6 mEq/l), plasma levels of deoxycorticosterone and corticosterone were normal, while plasma aldosterone was very low. The levels of these three mineralocorticoids showed only minor changes during infusion of angiotensin II. Furosemide administration under almost the same level of serum potassium (3.7 mEq/l) resulted in only a slight increase of plasma aldosterone. Since hypokalemia might possibly suppress the synthesis of aldosterone in the zona glomerulosa, angiotensin II was also infused under a normal level of potassium (4.3 mEq/l). However, angiotensin II also failed to stimulate any secretion of aldosterone, despite a progressive rise in blood pressure and sufficient suppression of plasma renin activity. On the other hand, rapid ACTH administration in the presence of 4.4 mEq/l of serum potassium increased both plasma aldosterone and cortisol. These results suggest that adrenal insensitivity to angiotensin II was the cause of the selective hypoaldosteronism in our patient, possibly due to a dysfunction of adrenal angiotensin II receptors, a disorder of postreceptors or both.
authors.affiliation: Department of Medicine, Kurume University, School of Medicine, Fukuoka-ken, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Otabe
authors.forename: S
authors.initials: S
authors.lastname: Muto
authors.forename: Y
authors.initials: Y
authors.lastname: Asano
authors.forename: E
authors.initials: E
authors.lastname: Ohbu
authors.forename: K
authors.initials: K
authors.lastname: Koyama
authors.forename: N
authors.initials: N
authors.lastname: Okita
authors.forename: K
authors.initials: K
authors.lastname: Yamada
authors.forename: K
authors.initials: K
authors.lastname: Nonaka
mesh_terms: D000141:Acidosis, Renal Tubular / Q000150:complications; D000311:Adrenal Glands / Q000187:drug effects / Q000503:physiopathology; D000450:Aldosterone / Q000097:blood; D000804:Angiotensin II / Q000494:pharmacology; D005260:Female; D006801:Humans; D006994:Hypoaldosteronism / Q000097:blood / Q000150:complications* / Q000209:etiology; D007008:Hypokalemia / Q000209:etiology; D008875:Middle Aged; D012083:Renin / Q000097:blood; D012859:Sjogren's Syndrome / Q000150:complications*
chemical_list: D000804:Angiotensin II; D000450:Aldosterone; D012083:Renin