Q J Med 2003; 96: 53-56
© 2003 Association of Physicians
Decreased salivary output in patients with Gaucher disease
From the 1 Gaucher Clinic and 2 Department of Medicine, Shaare Zedek Medical Center, Jerusalem, Israel
Received 7 June 2002 and in revised form 9 September 2002
Background: Gaucher disease, the most common sphingolipid storage disease, results in accumulation of glucocerebroside in macrophages or ‘Gaucher cells’. In a preliminary screening of 109 patients with type I disease, when asked specifically about dry mouth, approximately one quarter claimed to suffer from this symptom.
Aim: To ascertain whether decreased salivary output is a feature of Gaucher disease.
Design: Prospective case-control study.
Methods: Salivary output was measured in 65 adult patients and 65 healthy controls using the Saxon test with Hochberg's modification.
Results: Mean salivary output was 1.91±1.19 g/min in the patient group vs. 2.74±1.17 g/min in the control group (p<0.001). This difference was greater among males. These results were not improved in the patients receiving enzyme replacement therapy, which is effective in ameliorating most Gaucher-related signs and symptoms.
Discussion: Recent studies have implicated an association between sicca syndrome and viral hepatitis C infection, which may imply an immunological trigger for these findings, but in this specific cohort, only three patients were reactive for hepatitis C. Follow-up of patients, both untreated and receiving enzyme therapy, is needed to delineate the association with salivary hypofunction, and ascertain whether enzyme therapy may induce sicca symptoms.
Address correspondence to Dr D. Elstein, Gaucher Clinic, Shaare Zedek Medical Center, POB 3235, Jerusalem 91031, Israel. e-mail: gaucher{at}szmc.org.il