Primary Sjögren’s syndrome manifesting as sclerotic metabolic bone disease

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 tubul...

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Bibliographic Details
Published in:BMJ case reports 2021-01, Vol.14 (1), p.e237987
Main Authors: Boro, Hiya, Goyal, Alpesh, Naik, Shailendra Singh, Tandon, Nikhil
Format: Article
Language:English
Subjects:
Absorptiometry, Photon
Acidosis
Acidosis, Renal Tubular - blood
Acidosis, Renal Tubular - diagnosis
Acidosis, Renal Tubular - drug therapy
Acidosis, Renal Tubular - immunology
Adult
Alkaline Phosphatase - blood
Antibodies
Back Pain - blood
Back Pain - immunology
Biopsy
Bisphosphonates
Bone density
Bone Density - immunology
Bone diseases
Bone Diseases, Metabolic - blood
Bone Diseases, Metabolic - diagnosis
Bone Diseases, Metabolic - drug therapy
Bone Diseases, Metabolic - immunology
Case Report
Case reports
Creatinine
Female
Fractures
Humans
Laboratories
Metabolism
Phosphatase
Potassium Citrate - therapeutic use
Radionuclide Imaging
Scintigraphy
Sjogren's Syndrome - blood
Sjogren's Syndrome - complications
Sjogren's Syndrome - diagnosis
Sjogren's Syndrome - immunology
Skeleton - diagnostic imaging
Sodium Bicarbonate - therapeutic use
Urine
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Description
Summary: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.
ISSN:1757-790X
1757-790X
DOI:10.1136/bcr-2020-237987