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Primary Sjogren's syndrome presenting as hypokalemic paralysis: A case series


1 Department of Endocrinology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India
2 Department of Nephrology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India

Correspondence Address:
M Goroshi,
Department of Endocrinology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra
India
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Source of Support: None, Conflict of Interest: None

Primary Sjögren's syndrome (pSS) primarily involves exocrine glands, and renal tubular acidosis (RTA) is seen in one-third of the cases. RTA with hypokalemic paralysis as a presenting feature of pSS is described in few case reports in literature. We report 13 cases who presented as hypokalemic paralysis, and on evaluation were diagnosed to be pSS, as per the diagnostic criteria laid by the Sjφgren's International Collaborative Clinical Alliance (2012). All patients were female, with a mean age at presentation being 33.1 ± 8.22 years (range, 25-48 years). Eleven patients had a complete distal RTA and two patients had incomplete distal RTA at the time of presentation. 62% (8/13) of patients had no signs and symptoms of exocrine gland involvement. All the cases were managed with oral alkali therapy, and six patients received additional immunomodulating agents. No improvement in renal tubular dysfunction (in the form of a reduction in the alkali dose) after immunomodulating therapy was observed over a mean follow-up of 2.8 years. Renal tubular dysfunction can be the presenting manifestation of pSS. It is important to consider the possible presence of this disorder in adults with otherwise unexplained distal RTA or hypokalemia.


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    -  Goroshi M
    -  Khare S
    -  Jamale T
    -  Shah N S
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Online since 12th February '04
© 2004 - Journal of Postgraduate Medicine
Official Publication of the Staff Society of the Seth GS Medical College and KEM Hospital, Mumbai, India
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