A superfemale with primary Sjögren's 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 retardation, was hospitalized for dry mouth, dry eyes, and proteinuria. The sialography of the right parotid gland showed a globular-type gland enlargement. A definite diagnosis of primary Sjögren's syndrome (SS) was made, and further examinations revealed not only typical sicca syndrome but also systemic extraglandular lymphocytic infiltration; interstitial pneumonitis, glomerular- and interstitial nephritis, superficial gastritis, thyroiditis, and a severe excitation conductive impairment of heart. We report a very rare case of superfemale with primary SS which involved systemic organs.
Miyakawa
H
H
Department of Internal Medicine, Kumamoto University School of Medicine, Japan.
Iyonaga
K
K
Arima
S
S
Yonekawa
Y
Y
Suga
M
M
Ando
M
M
D001980:Bronchi / Q000473:pathology; D005260:Female; D006801:Humans; D007668:Kidney / Q000473:pathology; D008168:Lung / Q000473:pathology; D008875:Middle Aged; D009030:Mosaicism; D012729:Sex Chromosome Aberrations / Q000235:genetics / Q000473:pathology; D012859:Sjogren's Syndrome / Q000150:complications* / Q000235:genetics / Q000473:pathology; D013270:Stomach / Q000473:pathology; D013961:Thyroid Gland / Q000473:pathology