Identical Twins of Sjögren's Syndrome With Renal Tubular Acidosis

KOGA, Teruhito; Ebata, H; TANIGAWA, Kyuichi; Yamagata, Yuji

doi:10.2169/naika.69.1458

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“…A family history of tubular dysfunction, albeit observed in only a small number of patients, potentially hints at a subtle genetic contribution to RTA. Apart from this analysis, positive family history was reported in two families in a prospective study from India 10 and three case reports from Japan 41–43 . These papers were not included in the current data set because the Indian study did not provide individual‐level details of the patients and the reports from Japan did not fulfill the criteria for inclusion in this data set (both antibody and biopsy negative or not available).…”

Section: Discussionmentioning

confidence: 99%

Comprehensive analysis of clinical and laboratory features of 440 published cases of Sjögren's syndrome and renal tubular acidosis

Sandhya

2022

Int J of Rheum Dis

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Aim To describe the clinical and laboratory features of Sjögren's syndrome (SS) with renal tubular acidosis (RTA) from published literature. Methods A systematic search of indexed publications in all languages till December 2021 identified cases of SS with RTA (SS‐RTA) and were included if either antibody (anti‐SSA/anti‐SSB) or salivary gland histopathology were positive. Results There were 440 cases of SS‐RTA (63.9% from Asia, 95.5% women). The median (range) age was 37 (6‐78) years. Only 7.7% had a previous diagnosis of SS. Oral or ocular sicca symptoms were present in 63.7%. Positive ocular tests, oral tests, anti‐SSA, anti‐SSB and salivary gland histopathology were reported in 256/331 (77.3%), 123/128 (96%), 382/407 (93.9%), 298/379 (78.6%), and 246/268 (91.8%), respectively. Hypokalemic paralysis (HP) was the presenting feature in 63.6%; 25% had multiple episodes of HP and 8.4% had respiratory paralysis. Type 1, type 2, combined type 1 & 2, and type 4 RTA was seen in 388, 8, 38, and 3 patients, respectively. Proximal dysfunction and RTA complications were infrequently evaluated. Fanconi syndrome, nephrogenic diabetes insipidus, proteinuria, and low estimated glomerular filtration rate were found in 45, 21, 178, and 157, respectively. Nephrocalcinosis, renal stones, and osteomalacia were reported in 92, 79, and 72, respectively. Tubulointerstitial nephritis was found in 142 out of 152 renal biopsies. Conclusion SS‐RTA is an early manifestation of SS characterized by younger age and subclinical sicca symptoms. Although evaluated less frequently, oral sicca signs and salivary gland biopsy have a high positive yield. HP is the most common presentation. RTA is mostly distal; proximal dysfunction and complications were infrequently assessed.

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Section: Discussionmentioning

confidence: 99%

Comprehensive analysis of clinical and laboratory features of 440 published cases of Sjögren's syndrome and renal tubular acidosis

Sandhya

2022

Int J of Rheum Dis

View full text Add to dashboard Cite

show abstract

Identical Twins of Sjögren's Syndrome With Renal Tubular Acidosis

Cited by 1 publication

References 0 publications

Comprehensive analysis of clinical and laboratory features of 440 published cases of Sjögren's syndrome and renal tubular acidosis

Comprehensive analysis of clinical and laboratory features of 440 published cases of Sjögren's syndrome and renal tubular acidosis

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