Lab. Autoimmune Dis. Josep Font, IDIBAPS, ICMiD, Hosp. Clínic, Barcelona; Biomedical Res. Unit 02, Clin.Epidemiol.Res. Unit, UMAE, Specialties Hosp., Western Med. Center, Mexican Inst.for Social Security, Guadalajara; & Univ. of Colima, Mexico.
Primary Care Research Group, IDIBAPS, Centre d'Assistència Primària ABS Les Corts, GESCLINIC, Barcelona, Spain.
Clin Exp Rheumatol. 2018 May-Jun;36 Suppl 112(3):121-129. Epub 2018 Aug 14.
To analyse the clinical features and outcomes of patients presenting with life-threatening systemic disease in a large cohort of Spanish patients with primary Sjögren's syndrome (SS).
The GEAS-SS multicentre registry was formed in 2005 with the aim of collecting a large series of Spanish patients with primary SS, and included more than 20 Spanish reference centres with substantial experience in the management of SS patients. By January 2018, the database included 1580 consecutive patients fulfilling the 2002 classification criteria for primary SS. Severe, life-threatening systemic disease was defined as an activity level scored as "high" in at least one ESSDAI domain.
Among 1580 patients, 208 (13%) were classified as presenting a severe, potentially life-threatening systemic disease: 193 presented one ESSDAI domain classified as high, 14 presented two high scored domains and only one presented three high activity domains. The ESSDAI domains involved consisted of lymphadenopathy in 78 (37%) cases, CNS in 28 (13%), PNS in 25 (12%), pulmonary in 25 (12%), renal in 21 (10%), cutaneous in 19 (9%), articular in 18 (9%), haematological in 7 (3%) and muscular in 4 (2%). Patients with severe systemic disease were more frequently men (p=0.001) and had a higher frequency of anaemia (p<0.001), lymphopenia (p<0.001), rheumatoid factor (p=0.021), low C3 levels (p=0.015), low C4 levels (p<0.001) and cryoglobulins (p<0.001). From a therapeutic point of view, systemic patients received more frequently glucocorticoids (p<0.001), immunosuppressants (p<0.001), intravenous immunoglobulins (p=0.008) and rituximab (p<0.001). We found an overall mortality rate of 20% in severe systemic patients, a rate that reached to 33% in patients presenting two or more high systemic involvements; these patients had a higher frequency of low C4 levels (p=0.012) and cryoglobulins (p=0.001) in comparison with those with a single severe organ involved.
13% of patients with primary SS develop a potentially life-threatening systemic disease (mainly lymphoma, but also severe internal organ involvements including nervous system, the lungs and the kidneys). This subset of patients requires intensive therapeutic management with a mortality rate of nearly 20% of cases.
分析西班牙原发性干燥综合征（SS）患者大样本中出现危及生命的全身性疾病患者的临床特征和结局。
GEAS-SS 多中心登记研究于 2005 年成立，旨在收集大量西班牙原发性 SS 患者，该研究纳入了 20 多家具有丰富 SS 患者管理经验的西班牙参考中心。截至 2018 年 1 月，该数据库纳入了 1580 例符合 2002 年原发性 SS 分类标准的连续患者。严重、危及生命的全身性疾病定义为至少一个 ESSDAI 域的活动水平评分为“高”。
在 1580 例患者中，208 例（13%）被归类为出现严重、潜在危及生命的全身性疾病：193 例存在一个 ESSDAI 域被评为高，14 例存在两个高评分域，只有 1 例存在三个高活动域。涉及的 ESSDAI 域包括淋巴结病 78 例（37%）、中枢神经系统 28 例（13%）、周围神经系统 25 例（12%）、肺部 25 例（12%）、肾脏 21 例（10%）、皮肤 19 例（9%）、关节 18 例（9%）、血液学 7 例（3%）和肌肉 4 例（2%）。严重系统性疾病患者更常为男性（p=0.001），且贫血（p<0.001）、淋巴细胞减少症（p<0.001）、类风湿因子（p=0.021）、低 C3 水平（p=0.015）、低 C4 水平（p<0.001）和冷球蛋白血症（p<0.001）的发生率更高。从治疗角度来看，系统性疾病患者更频繁地接受糖皮质激素（p<0.001）、免疫抑制剂（p<0.001）、静脉注射免疫球蛋白（p=0.008）和利妥昔单抗（p<0.001）。我们发现严重系统性疾病患者的总死亡率为 20%，在出现两个或更多高系统性受累的患者中，死亡率达到 33%；与仅存在单一严重器官受累的患者相比，这些患者的 C4 水平（p=0.012）和冷球蛋白血症（p=0.001）的发生率更高。
13%的原发性 SS 患者出现潜在危及生命的全身性疾病（主要为淋巴瘤，但也包括严重的内脏器官受累，包括神经系统、肺部和肾脏）。这组患者需要强化治疗管理，死亡率接近 20%。
