PATHOPHYSIOLOGY, EPIDEMIOLOGY AND THERAPY OF DISTAL RENAL TUBULAR-ACIDOSIS

The present study is a review of interactions between the Sjogren synd rome and distal renal tubular acidosis (dRTA). In addition, we describ e the pathophysiology of the dRTA and the diagnosis and the therapy of the disease. The reason for dRTA accompanying a Sjogren syndrome is t he absence of proton pumps the renal collecting duct. In serum from wo men suffering from renal stones, antinuclear autoantibodies specific f or the Sjogren syndrome (Ro (anti-SS-A), La (anti-SS-B)) often can be demonstrated, even when the women do not express the typical symptoms of the Sjogren syndrome. An incomplete dRTA, not apparent in the blood gas composition, also can lead to the formation of renal stones. In t his case diagnosis can be made, even in laboratories which do not have the capacity for NH4+ measurement by measuring the urinary ''osmotic gap'' or the ''anionic gap'', and with the aid of acidification tests. Treatment should be with citrate-containing drugs. Calcium-containing drugs do not facilitate stone formation but, indeed, may prevent the development of the osteoporosis often seen in dRTA. Calcium-low diets are contraindicated for the same reason. The present study includes a report of a case in which, for the first time, the dRTA due to Sjogren syndrome is accompanied by decreased serum cholinesterase activity.