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


Journal

BMJ case reports
ISSN: 1757-790X
Titre abrégé: BMJ Case Rep
Pays: England
ID NLM: 101526291

Informations de publication

Date de publication:
11 Jan 2021
Historique:
entrez: 12 1 2021
pubmed: 13 1 2021
medline: 23 2 2021
Statut: epublish

Résumé

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.

Identifiants

pubmed: 33431459
pii: 14/1/e237987
doi: 10.1136/bcr-2020-237987
pmc: PMC7802734
pii:
doi:

Substances chimiques

Sodium Bicarbonate 8MDF5V39QO
Alkaline Phosphatase EC 3.1.3.1
Potassium Citrate EE90ONI6FF

Types de publication

Case Reports Journal Article

Langues

eng

Sous-ensembles de citation

IM

Informations de copyright

© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.

Déclaration de conflit d'intérêts

Competing interests: None declared.

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Auteurs

Hiya Boro (H)

Endocrinology and Metabolism, All India Institute of Medical Sciences, New Delhi, Delhi, India.

Alpesh Goyal (A)

Endocrinology and Metabolism, All India Institute of Medical Sciences, New Delhi, Delhi, India alpeshgoyal89@gmail.com.

Shailendra Singh Naik (SS)

Radiodiagnosis, Institute Rotary Cancer Hospital, New Delhi, Delhi, India.

Nikhil Tandon (N)

Endocrinology and Metabolism, All India Institute of Medical Sciences, New Delhi, Delhi, India.

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Classifications MeSH