Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing, China.
Department of Rheumatology and Immunology, The Second Xiangya Hospital, Central South University, Changsha, Hunan, China.
Int Immunopharmacol. 2023 Oct;123:110716. doi: 10.1016/j.intimp.2023.110716. Epub 2023 Jul 26.
This study aims to describe patients' characteristics and treatment responses with primary Sjögren's syndrome (pSS) who experience immune thrombocytopenia (ITP) and ITP with clinical significance (ITPCS).
A retrospective study was conducted involving 164 patients diagnosed with pSS-related ITP after excluding secondary ITP. Patients were categorized into subgroups based on the risk of bleeding: major bleeding event (MBG), non-hemorrhagic group (NHG), and hematological involvement-only SS group (HOSG).
57 (34.8%) were diagnosed with ITP simultaneously with pSS, while 60 (36.6%) were diagnosed with ITP before pSS. ITP patients exhibited a high prevalence of interstitial lung disease (19.5%), and an up to 96.3% positive presence of anti-SSA/Ro-52 antibody. ITPCS was identified in 58.5% of patients, with 22.0% experiencing high-risk hemorrhagic events. A median (range) of 2 (1, 3) treatment lines for maintenance therapies were administered. Corticosteroids and hydroxychloroquine (HCQ) led to an ITP response in 76.1% of patients. Ciclosporin A (CsA) and other medicines contributed to a 76.6% response. The MBG, NHG, and HOSG groups consisted of 36 (22.0%), 68 (41.5%) and 53 (32.3%) patients, respectively. Notably, patients of MBG were more frequently diagnosed before SS onset (p = 0.035). They required more treatment lines (p = 0.001) with a lower risk of relapse (p < 0.001), which is confirmed in patients with only hematological involvement (HOSG group).
Patients with pSS-related ITP face an increased risk of bleeding, particularly in the MBG group, which necessitates more extensive treatment. Heterogeneous treatment regimens were observed for pSS-related ITP, and combinations involving corticosteroids, HCQ, and/or CsA appear viable options.
本研究旨在描述原发性干燥综合征（pSS）伴免疫性血小板减少症（ITP）和有临床意义的 ITP（ITPCS）患者的特征和治疗反应。
本研究为回顾性研究，纳入了 164 例排除继发性 ITP 后诊断为 pSS 相关 ITP 的患者。根据出血风险将患者分为亚组：重大出血事件（MBG）、非出血组（NHG）和仅血液学受累的干燥综合征组（HOSG）。
57 例（34.8%）同时诊断为 ITP 和 pSS，60 例（36.6%）先诊断为 ITP 后诊断为 pSS。ITP 患者间质性肺病（ILD）患病率较高（19.5%），抗 SSA/Ro-52 抗体阳性率高达 96.3%。58.5%的患者存在 ITPCS，22.0%的患者发生高危出血事件。中位数（范围）为 2（1，3）线维持治疗。皮质类固醇和羟氯喹（HCQ）使 76.1%的患者 ITP 得到缓解。环孢素 A（CsA）和其他药物的缓解率为 76.6%。MBG、NHG 和 HOSG 组分别有 36（22.0%）、68（41.5%）和 53（32.3%）例患者。值得注意的是，MBG 组患者更常先于 SS 发病（p=0.035）。他们需要更多的治疗线（p=0.001），复发风险更低（p<0.001），这在仅血液学受累的患者（HOSG 组）中得到证实。
pSS 相关 ITP 患者出血风险增加，尤其是 MBG 组，需要更广泛的治疗。pSS 相关 ITP 的治疗方案存在异质性，皮质类固醇、HCQ 和/或 CsA 联合治疗可能是可行的选择。
