Acquired Nephrogenic Diabetes Insipidus Secondary to Distal Renal Tubular Acidosis and Nephrocalcinosis Associated with Sjogren's Syndrome

Title: Acquired Nephrogenic Diabetes Insipidus Secondary to Distal Renal Tubular Acidosis and Nephrocalcinosis Associated with Sjogren's Syndrome
Authors: Nagayama, MD, Yuji; Shigeno, M.; Nakagawa, Y.; Suganuma, A.; Takeshita, A.; Fujiyama, K.; Ashizawa, K.; Kiriyama, T.; Yokoyama, N.; Nagataki, S.
Publisher: Journal of Endocrinological Investigation
Date Published: September 01, 1994
Reference Number: 91
A 52-year-old woman was referred to our hospital because of 16-year history of polyuria and polydipsia. Hyposthenuria, hyperchloremic metabolic acidosis and the inabilities to acidify the urine after acid-loading test and to concentrate the urine in responses to water-deprivation and antidiuretic hormone administration allowed us to diagnose renal tubular acidosis and nephrogenic diabetes insipidus. Radiographic examinations revealed bilateral nephrocalcinosis. The patient was also found to have clinical and laboratory findings characteristic for Sjogren's syndrome. Thus the longstanding, poorly monitored distal renal tubular acidosis associated with Sjogren's syndrome was considered to result in very rare renal complications-nephrocalcinosis and nephrogenic diabetes insipidus. In patients with renal tubular acidosis and/or nephrogenic diabetes insipidus of unknown etiology, therefore, Sjogren's syndrome should be considered as one of primary disorders.

This translation by the NDI Foundation is to assist the lay reader. To provide a clear, accessible interpretation of the original article, we eliminated or simplified some technical detail and complicated scientific language. We concentrated our translation on those aspects of the article dealing directly with NDI. The NDI Foundation thanks the researchers for their work toward understanding and more effectively treating this disorder.
© Copyright NDI Foundation 2007 (JC)

Nagayama, et al., report on a 52-year-old woman who was referred to their hospital because of a 16-year history of polyuria (chronic passing of large amounts of urine) and polydipsia (chronic, excessive thirst). Diagnosis revealed the woman was suffering from Sjogren's syndrome complicated by acquired nephrogenic diabetes insipidus (NDI) along with acidosis and nephrocalcinosis (excess calcium phosphate in the tubules of the kidney).

Sjogren's syndrome is characterized by dryness of the mouth or parotid gland swelling, dryness of the eyes and arthritis. Kidney complications are frequently associated with this syndrome.

The acidosis in the woman's kidney tubules rendered them unable to acidify urine. This results in high urinary calcium concentrations which leads to nephrocalcinosis, which adversely affects the kidney's ability to function.

Though it is not clear that the woman's nephrocalcinosis caused her NDI, the authors noted that the calcium deposits resulting from her nephrocalcinosis collect around the part of the tubule where the antidiuretic hormone, arginine vasopressin (AVP) exerts its influence. NDI is characterized by the kidney's inability to respond to AVP. But, it could also be that Sjogren's syndrome's ability to infiltrate kidney tissue may interfere with the tubules capacity to allow water to pass through them. It was clear, however, that the woman's long-standing tubular acidosis resulted in nephrocalcinosis and NDI. And the authors state that in patients with kidney tubular acidosis and/or NDI of unknown cause, the possibility of Sjogren's syndrome should be considered.