FO004PATTERN OF SALIVARY GLAND INVOLVEMENT IN PATIENTS WITH SJOGREN’S SYNDROME PRESENTING WITH DISTAL RTA

Abstract

Introduction and Aims: Sjogren's syndrome (SS) is a chronic progressive autoimmune multisystem disease characterized by hypofunction of exocrine glands. It is also known as "Mikulicz disease" and "Sicca syndrome" and is characterized by the dry-ness of eyes and mouth due to involvement of lacrimal and salivary glands. Salivary gland scintigraphy with 15mCi of Tc pertechnetate is a non-invasive and readily available test with advantage of objective and functional assessment of salivary glands. AIM: To study pattern of salivary gland involvement using radionuclide scan in patients with Sjogren's syndrome presenting with distal RTA. Method(s): A prospective study of patients of SS was done to analyze salivary gland involvement using radionuclide scan. These patients were referred for evaluation of renal tubular acidosis/electrolyte imbalance in Nephrology clinic. The patients were evaluated in details for confirmation of diagnosis of SS, distal RTA and pattern of salivary gland involvement. All documented and confirmed cases of SS with dRTA were included in present study. Result(s): Total 11 cases of SS with dRTA were included in present study. All are female patients with mean age of 34.3569.22 years. Major presenting symptoms included paraparesis (10/11), polyuria (7/11), asthenia (7/11), polyarthritis (5/11), dry-ness of eyes (4/11) and dryness of mouth (4/11). Anti Ro/Anti La antibody was positive in all patients. RA factor was positive in 2/11 cases and ANA in all cases. All patients had non anion gap metabolic acidosis (Mean pH-7.22 6 0.05), hypokalemia (Potassium, 2.58 60.44 Meq/L) and low HCO3 (11.7561.69). Mean presenting Urine pH 6.3460.27, urine potassium (7.9162.24 Meq/L).Pattern of salivary gland involvement on radionuclide scan showed moderate reduction of activity in bilateral submandibular glands in 10/11 patients. Similarly reduction of tracer uptake and activity was seen in Right parotid gland (6/11) and Left parotid gland (2/11). All patients with predominant complaint of dryness of mouth had both submandibular and parotid gland involve-menton scan. Conclusion(s): RTA is one of presenting manifestation in patients with SS. Salivary scintigraphy is non invasive and reliable diagnostic tool to assess salivary gland involvement in SS. The diagnostic and functional assessment of salivary gland using radionu-clide scan obviates the need of invasive biopsy. Submandibular gland is almost always involved in patients with SS with RTA. Dryness of mouth as a presenting symptom is a predictor of simultaneous parotid and submandibular gland involvement.