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

© 2022 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd..

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.

Medienart:

E-Artikel

Erscheinungsjahr:

2023

Erschienen:

2023

Enthalten in:

Zur Gesamtaufnahme - volume:26

Enthalten in:

International journal of rheumatic diseases - 26(2023), 2 vom: 03. Feb., Seite 278-285

Sprache:

Englisch

Beteiligte Personen:

Sandhya, Pulukool [VerfasserIn]

Links:

Volltext

Themen:

Distal tubular acidosis
Fanconi syndrome
Hypokalemic paralysis
Journal Article
Renal disease
Renal tubular acidosis
Sjögren's syndrome
Tubulointerstitial nephritis

Anmerkungen:

Date Completed 30.01.2023

Date Revised 02.02.2023

published: Print-Electronic

Citation Status MEDLINE

doi:

10.1111/1756-185X.14483

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM348381522