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Renal Tubular Acidosis in Patients with Primary Sjögren's Syndrome
原發性修格蘭症候群併發腎小管酸血症
摘要
目的：腎小管酸血症併發肌肉癱瘓是原發性修格蘭症候群少見的併發症之一，我們希望藉由分析這一群病人的臨床症狀，血清學檢驗及臨床上治療的反應，以做爲大家對於此一疾病診斷及治療上的參考。方法：我們回溯性的查閱在1995年1月到2006年12月當中在台大醫院所有因爲修格蘭症候群併發腎小管酸血症的病人，發現到共有8個病人因此病症而住院，而且所有的病人都是和原發性修格蘭症候群相關，我們並選取了41個原發性修格蘭症候群但未併發腎小管酸血症的病人做爲對照組。結果：腎小管酸血症在6個病人(75%)是原發性修格蘭症候群的第一個臨床表現，這8個病人的臨床症狀可以從輕微的肌肉無力到嚴重的全身癱瘓。在治療上，有一個病人只有輕微的低鉀血症而不須要藥物治療，二個病人因爲藥物遵從性不佳而重覆的因爲肌肉癱瘓住院，其他的病人在適當的藥物治療及鉀離子補充之下，疾病都控制的相當良好。和對照組比較之下，二組在血液中鉀離子濃度、肌酸酐值、尿液pH值以及類固醇的使用劑量上有明顯的差異。結論：臨床有症狀的腎小管酸血症可以是原發性修格蘭症候群的第一個臨床表現而且常常併發腎臟功能的異常，同時低鉀血症可因爲造成呼吸衰竭或心律不整而危及生命。因此，在臨床上我們應注意到此一少見的併發症並儘早的給予治療，對於病人預後將有很大的助益。
並列摘要
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.