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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.<\/jats:p>","DOI":"10.1136\/bcr-2020-237987","type":"journal-article","created":{"date-parts":[[2021,1,12]],"date-time":"2021-01-12T18:33:22Z","timestamp":1610476402000},"page":"e237987","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":5,"title":["Primary Sj\u00f6gren\u2019s syndrome manifesting as sclerotic metabolic bone disease"],"prefix":"10.1136","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7868-8026","authenticated-orcid":false,"given":"Hiya","family":"Boro","sequence":"first","affiliation":[{"name":"Endocrinology and Metabolism, All India Institute of Medical Sciences, New Delhi, Delhi, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0922-5022","authenticated-orcid":false,"given":"Alpesh","family":"Goyal","sequence":"additional","affiliation":[{"name":"Endocrinology and Metabolism, All India Institute of Medical Sciences, New Delhi, Delhi, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shailendra Singh","family":"Naik","sequence":"additional","affiliation":[{"name":"Radiodiagnosis, Institute Rotary Cancer Hospital, New Delhi, Delhi, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nikhil","family":"Tandon","sequence":"additional","affiliation":[{"name":"Endocrinology and Metabolism, All India Institute of Medical Sciences, New Delhi, Delhi, India"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2021,1,11]]},"reference":[{"key":"2025082623204314000_14.1.e237987.1","doi-asserted-by":"crossref","first-page":"16047","DOI":"10.1038\/nrdp.2016.47","article-title":"Sj\u00f6gren syndrome","volume":"2","author":"Brito-Zer\u00f3n","year":"2016","journal-title":"Nat Rev Dis Primers"},{"key":"2025082623204314000_14.1.e237987.2","doi-asserted-by":"publisher","DOI":"10.4103\/2230-8210.85599"},{"key":"2025082623204314000_14.1.e237987.3","doi-asserted-by":"crossref","first-page":"103","DOI":"10.2174\/1874312901408010103","article-title":"Sj\u00f6gren's, renal tubular acidosis and osteomalacia \u2014 an Asian Indian series","volume":"8","author":"Sandhya","year":"2014","journal-title":"Open Rheumatol J"},{"key":"2025082623204314000_14.1.e237987.4","doi-asserted-by":"crossref","DOI":"10.1097\/MD.0000000000006493","article-title":"Adult-onset hypophosphatemic osteomalacia associated with Sjogren syndrome: clinical case report","volume":"96","author":"Shen","year":"2017","journal-title":"Medicine"},{"key":"2025082623204314000_14.1.e237987.5","doi-asserted-by":"crossref","first-page":"771","DOI":"10.1007\/BF02734150","article-title":"Approach to renal tubular disorders","volume":"72","author":"Bagga","year":"2005","journal-title":"Indian J Pediatr"},{"key":"2025082623204314000_14.1.e237987.6","doi-asserted-by":"publisher","DOI":"10.1148\/rg.317115093"},{"key":"2025082623204314000_14.1.e237987.7","doi-asserted-by":"crossref","first-page":"330","DOI":"10.4103\/0971-4065.209347","article-title":"Renal tubular acidosis, osteopetrosis, and cerebral calcification: a rare syndrome caused by carbonic anhydrase II deficiency","volume":"27","author":"Laway","year":"2017","journal-title":"Indian J Nephrol"},{"key":"2025082623204314000_14.1.e237987.8","doi-asserted-by":"crossref","DOI":"10.1016\/j.kint.2017.10.012","article-title":"Osteopetrosis with renal tubular acidosis and cerebral calcification","volume":"93","author":"Kelly","year":"2018","journal-title":"Kidney Int"},{"key":"2025082623204314000_14.1.e237987.9","doi-asserted-by":"crossref","first-page":"865","DOI":"10.1007\/s10545-018-0147-6","article-title":"Outcome of adult patients with X-linked hypophosphatemia caused by PHEX gene mutations","volume":"41","author":"Chesher","year":"2018","journal-title":"J Inherit Metab Dis"},{"key":"2025082623204314000_14.1.e237987.10","first-page":"97","article-title":"Fluorosis in India: an overview","volume":"1","author":"Arlappa","year":"2013","journal-title":"Int J Res Dev Health"},{"key":"2025082623204314000_14.1.e237987.11","doi-asserted-by":"publisher","DOI":"10.1097\/01.ASN.0000023430.92674.E5"},{"key":"2025082623204314000_14.1.e237987.12","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(82)80288-6"},{"key":"2025082623204314000_14.1.e237987.13","unstructured":"Baer AN . 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