A SUPERFEMALE WITH PRIMARY SJOGRENS-SYNDROME WHICH INVOLVED SYSTEMIC ORGANS

A 52-year-old Japanese woman complicated by a sex chromosomal anomaly as a superfemale, a mosaic of XXXXX/XXXX/XXX/XX/XO, with mild mental r etardation, was hospitalized for dry mouth, dry eyes, and proteinuria. The sialography of the right parotid gland showed a globular-type gla nd enlargement. A definite diagnosis of primary Sjogren's syndrome (SS ) was made, and further examinations revealed not only typical sicca s yndrome but also systemic extraglandular lymphocytic infiltration, int erstitial pneumonitis, glomerular-and interstitial nephritis, superfic ial gastritis, thyroiditis, and a severe excitation conductive impairm ent of heart. We report a very rare case of superfemale with primary S S which involved systemic organs.