第21卷 　 第二期 　 Vol. 21, No. 2, December, 2007

Original Aiticles 原著

Renal tubular acidosis in patients with primary Sjögren's syndrome

Li-Hsuan Chen1,2, Ping-Ning Hsu1,3, Mao-Yuan Chen1, Kuang-Lun Lee1, Song-Chou Hsieh1, Chia-Li Yu1,4

1Division of Allergy, Immunology and Rheumatology, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan 2Division of Allergy, Immunology and Rheumatology, Department of Internal Medicine, E-DA Hospital, I-Show University, Kaohsiung, Taiwan 3Graduate Institute of Immunology, and 4Graduate Institute of Molecular Medicine, College of Medicine, National Taiwan University, Taipei, Taiwan

Objective: Clinical significant renal tubular acidosis (RTA) with paralysis is a rare presentation in primary Sjögren's syndrome. This study is to investigate the symptoms, serologic markers, and the therapeutic responses in patients with clinical significant RTA associated with primary Sjögren's syndrome in Taiwan. Methods: We retrospectively reviewed medical record for RTA associated with Sjögren's syndrome from National Taiwan University Hospital since January 1995 until December 2006. Eight patients of RTA were identified and they were associated with primary Sjögren's syndrome. Primary Sjögren's syndrome patients without RTA were used as control group. Results: RTA was noted to be the first presentation of Sjögren's syndrome in 6 cases (75%) of eight patients. The range of the symptoms could be from mild weakness to severe paralysis (50%). There was one patient with mild hypokalemia who did not need treatment. Paralysis recurred in two patients due to poor drug compliance. RTA was stabilized in 62.5% cases under the treatment of steroid and potassium supply. The differences between RTA and control groups were significant in potassium level, urine pH, serum creatinine level and steroid dose. Conclusion: Clinical significant RTA could be the first manifestation of primary Sjögren's syndrome and it is associated with impaired renal function. RTA could be life threatening due to respiratory paralysis or cardiac arrhythmia. Thus, we should pay attention to this rare complication and give appropriate treatment for better clinical outcomes.

keyword Renal tubular acidosis, Sjögren's syndrome, hypokalemia, paralysis