Primary Sjögren's syndrome manifesting as sclerotic metabolic bone disease.

Hiya Boro, Alpesh Goyal, Shailendra Singh Naik, Nikhil Tandon

BMJ case reports 2021 Jan 11

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Primary Sjögren's syndrome (pSS) is a chronic slowly progressive autoimmune disease characterised by lymphocytic infiltration of salivary and lacrimal glands with varying degree of systemic involvement. Renal involvement, a recognised extraglandular manifestation of pSS, is commonly related to tubular dysfunction and generally manifests as distal renal tubular acidosis (RTA), proximal RTA, tubular proteinuria and nephrogenic diabetes insipidus. Untreated long-standing RTA is known to cause metabolic bone disease. Here, we present the report of a patient with sclerotic metabolic bone disease related to pSS with combined distal and proximal RTA and negative workup for other causes of sclerotic bone disease. A significant clinical and biochemical improvement, including recovery of proximal tubular dysfunction, was noted with alkali therapy. This case suggests the need to consider pSS in the diagnostic algorithm of a patient presenting with sclerotic bone disease. © BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.

Citation

Hiya Boro, Alpesh Goyal, Shailendra Singh Naik, Nikhil Tandon. Primary Sjögren's syndrome manifesting as sclerotic metabolic bone disease. BMJ case reports. 2021 Jan 11;14(1)