Metabolic bone disease as a presenting manifestation of primary Sjögren's syndrome: Three cases and review of literature.
Primary Sjögren's syndrome (pSS) is a chronic autoimmune disease characterized by a progressive lymphocytic infiltration of the exocrine glands with varying degrees of systemic involvement. Chronic inflammation compromises the glands' function that leads to dry symptoms in the mouth/eyes. Renal involvement is a well recognized extraglandular manifestation of pSS. Metabolic bone disease (MBD), however, rarely occurs as the primary manifestation of a renal tubule disorder due to pSS. To the best of our knowledge there are only 6 reported cases of metabolic bone disease as the primary manifestation of pSS to date. Four of these had distal renal tubular acidosis (RTA), and 2 had a combined picture of distal and proximal tubular dysfunction. We herein present our experience of 3 cases who presented to us with a clinical picture suggestive of MBD. While investigating these patients, we found evidence of RTA, which was found to be secondary to pSS.
Department of Endocrinology and Metabolism, All India Institute of Medical Sciences, New Delhi, India.
Deepak
D
Khandelwal
Saptarshi
S
Bhattacharya
Ankur
A
Gadodia
Rajesh
R
Khadgawat
Nikhil
N
Tandon
A C
AC
Ammini