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A superfemale with primary Sjögren's syndrome which involved systemic organs
Author(s) -
MIYAKAWA H.,
IYONAGA K.,
ARIMA S.,
YONEKAWA Y.,
SUGA M.,
ANDO M.
Publication year - 1997
Publication title -
journal of internal medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.625
H-Index - 160
eISSN - 1365-2796
pISSN - 0954-6820
DOI - 10.1046/j.1365-2796.1997.00190.x
Subject(s) - medicine , interstitial nephritis , systemic disease , sialography , pathology , primary systemic amyloidosis , salivary gland , dermatology , gastroenterology , kidney , immunopathology
Miyakawa H, Iyonaga K, Arima S, Yonekawa Y, Suga M, Ando M (First and Third Departments of Internal Medicine, Kumamoto University School of Medicine, Kumamoto, Japan). A superfemale with primary Sjögren's syndrome which involved systemic organs (Case report). J Intern Med 1997; 242 : 261–5. A 52‐year‐old Japanese woman complicated by a sex chromosomal anomaly as a superfemale, a mosaic of /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.