Department of Rheumatology, University Hospital, Lund, Sweden.
Scand J Rheumatol. 2009;38(4):295-8. doi: 10.1080/03009740802629424.
Chronic renal disease other than scleroderma renal crisis (SRC) is not well documented in systemic sclerosis (SSc). We examined the occurrence of decreased glomerular filtration rate (GFR) in a large consecutive SSc cohort and analysed whether it was related to SSc or could be related to other causes.
During 1983-2004 GFR was measured by chromium-51-ethylenediaminetetraacetic acid (51Cr-EDTA) or iohexol clearance in 461 patients with SSc according to the American College of Rheumatology (ACR) criteria [356 with limited cutaneous SSc (lcSSc) and 105 with diffuse cutaneous SSc (dcSSc)] and the measurements were repeated once a year. Decreased GFR was defined as GFR<70% of the age-adjusted values. SRC was diagnosed in 4/360 lcSSc (1.1%) and in 10/115 dcSSc (8.7%). These patients were excluded from further analyses.
At the latest follow-up at a median duration of 7.7 (range 0.5-54) years, decreased GFR was found in 39 lcSSc (11%) and nine (8.6%) dcSSc patients. Among the 48 SSc patients with GFR< 70p% (percentage of predicted value = p%), hypertension was diagnosed in 29 (60%) and cardiac involvement in 25 (52%). Different nephropathies were found in eight (19%) patients by renal biopsy. Fifteen patients with decreased GFR were followed up for > or = 4 years and no progress was seen in 11/15.
A minority of patients with SSc develop renal dysfunction other than SRC. Decreased GFR was associated with other manifestations such as hypertension and cardiac involvement indicating possible pre-renal causes.
硬皮病肾危象(SRC)以外的慢性肾病在系统性硬化症(SSc)中记录甚少。我们检测了大量连续 SSc 患者中肾小球滤过率(GFR)降低的发生率,并分析其是否与 SSc 有关,或者是否可能与其他原因有关。
根据美国风湿病学会(ACR)标准[356 例局限性皮肤型 SSc(lcSSc)和 105 例弥漫性皮肤型 SSc(dcSSc)],1983-2004 年间对 461 例 SSc 患者进行了铬-51-乙二胺四乙酸(51Cr-EDTA)或碘海醇清除率的检测,每年重复一次。将 GFR 降低定义为 GFR<70%的年龄校正值。在 360 例 lcSSc 中发现了 4 例(1.1%)SRC,在 115 例 dcSSc 中发现了 10 例(8.7%)。这些患者被排除在进一步的分析之外。
在中位随访时间为 7.7 年(范围 0.5-54 年)时,39 例 lcSSc(11%)和 9 例(8.6%)dcSSc 患者发现 GFR 降低。在 48 例 GFR<70p%(预测值的百分比 = p%)的 SSc 患者中,29 例(60%)诊断为高血压,25 例(52%)有心脏受累。8 例(19%)患者经肾活检发现不同的肾病。15 例 GFR 降低的患者随访>或=4 年,其中 11 例无进展。
少数 SSc 患者会发生除 SRC 以外的肾功能障碍。GFR 降低与高血压和心脏受累等其他表现有关,表明可能存在肾前原因。