Avouac J, Sordet C, Depinay C, Ardizonne M, Vacher-Lavenu M C, Sibilia J, Kahan A, Allanore Y
René Descartes University, Medical Faculty, Hôpital Cochin, France.
Arthritis Rheum. 2006 Jul;54(7):2243-9. doi: 10.1002/art.21922.
To determine the prevalence of sicca symptoms and Sjögren's syndrome (SS) in a 2-center prospective series of patients with systemic sclerosis (SSc), using the American-European Consensus Group criteria for SS.
Consecutive SSc patients hospitalized for followup care were evaluated for sicca symptoms. When the initial clinical evaluation yielded positive findings, a labial salivary gland biopsy was performed; histologic analysis evaluated focal lymphocytic sialadenitis and/or glandular fibrosis. Computed tomography and respiratory function tests were used to assess pulmonary fibrosis.
We included 133 SSc patients (mean +/- SD age 55 +/- 13 years; mean +/- SD disease duration 6.5 +/- 6 years). Eighty-one patients had limited cutaneous SSc (lcSSc). Ninety-one patients (68%) had sicca syndrome. Histologic analysis revealed fibrotic involvement in 50 of these 91 patients, but labial salivary gland fibrosis was not associated with any organ involvement we evaluated. Nineteen of the 133 patients (14%) had SS. In this subgroup, lcSSc was present at a significantly higher frequency (18 of 19 patients) than in the remaining patients with sicca syndrome (39 of 72 patients) and the patients without sicca syndrome (24 of 42 patients). This subgroup also had a significantly higher frequency of anticentromere antibodies (18 of 19 patients) than did the remaining patients with sicca syndrome (19 of 72 patients) and the patients without sicca syndrome (5 of 42 patients). In addition, this subgroup had a significantly lower prevalence of pulmonary fibrosis (2 of 19 patients) than did the remaining patients with sicca syndrome (29 of 72 patients) and the patients without sicca syndrome (19 of 42 patients).
There was a 68% prevalence of sicca syndrome in this prospective series of SSc patients. Sicca syndrome was related primarily to glandular fibrosis, the hallmark of SSc. The prevalence of secondary SS, as defined by the American-European Consensus Group criteria, was 14% and was markedly associated with lcSSc. We believe that lcSSc should be regarded as a specific autoimmune subgroup of SSc.
采用美国 - 欧洲共识小组关于干燥综合征（SS）的标准，确定在一个两中心前瞻性系统性硬化症（SSc）患者系列中干燥症状和干燥综合征（SS）的患病率。
对因后续治疗而住院的连续性SSc患者进行干燥症状评估。当初始临床评估有阳性发现时，进行唇唾液腺活检；组织学分析评估局灶性淋巴细胞性涎腺炎和/或腺体纤维化。使用计算机断层扫描和呼吸功能测试评估肺纤维化。
我们纳入了133例SSc患者（平均±标准差年龄55±13岁；平均±标准差病程6.5±6年）。81例患者为局限性皮肤型SSc（lcSSc）。91例患者（68%）有干燥综合征。组织学分析显示这91例患者中有50例存在纤维化，但唇唾液腺纤维化与我们评估的任何器官受累均无关联。133例患者中有19例（14%）患有SS。在这个亚组中，lcSSc的出现频率（19例患者中的18例）显著高于其余有干燥综合征的患者（72例患者中的39例）和无干燥综合征的患者（42例患者中的24例）。这个亚组抗着丝点抗体的出现频率（19例患者中的18例）也显著高于其余有干燥综合征的患者（72例患者中的19例）和无干燥综合征的患者（42例患者中的5例）。此外，这个亚组肺纤维化的患病率（19例患者中的2例）显著低于其余有干燥综合征的患者（72例患者中的29例）和无干燥综合征的患者（42例患者中的19例）。
在这个前瞻性SSc患者系列中，干燥综合征的患病率为68%。干燥综合征主要与腺体纤维化有关，这是SSc的标志。根据美国 - 欧洲共识小组标准定义的继发性SS的患病率为14%，且与lcSSc显著相关。我们认为lcSSc应被视为SSc的一个特定自身免疫亚组。
