Department of Rheumatology, université Paris Cité, Cochin Hospital, Paris, France.
Department of Rheumatology, université Paris Cité, Cochin Hospital, Paris, France; Inserm U1016, institut Cochin, CNRS UMR8104, Paris, France.
Joint Bone Spine. 2023 Jul;90(4):105560. doi: 10.1016/j.jbspin.2023.105560. Epub 2023 Mar 17.
Thrombocytopenia in the context of systemic sclerosis (SSc) is rare. It should primarily raise the possibility of scleroderma renal crisis. Immune thrombocytopenia (ITP) is another cause of low platelets that is common in systemic lupus erythematosus, but tremendously rare in patients with SSc. We herein report two cases of severe ITP in patients with SSc. The first case is a 29-year-old woman with very low platelet counts (2×10/L) that did not increase despite receiving corticosteroids, intravenous immunoglobulins (IVig), rituximab and romiplostim. Because of a symptomatic acute subdural haematoma, emergency splenectomy was performed and subsequently platelet counts normalised without neurological sequelae. The second case is a 66-year-old woman in whom self-limited mild epistaxis revealed low platelet counts (8×10/L). The patient did not improve after the use of IVig and corticosteroids. Secondarily rituximab and romiplostim normalised the platelet counts after 8 weeks. To the best of our knowledge this is the first reported case of severe ITP in a patient with diffuse cutaneous SSc and anti-topoisomerase antibodies.
系统性硬化症（SSc）伴发血小板减少症罕见，应主要考虑硬皮病肾危象。免疫性血小板减少症（ITP）也是导致血小板减少的另一种原因，常见于系统性红斑狼疮，但在 SSc 患者中极为罕见。我们在此报告两例 SSc 患者发生严重 ITP 的病例。第一例是一位 29 岁女性，血小板计数极低（2×10/L），尽管接受了皮质类固醇、静脉注射免疫球蛋白（IVIg）、利妥昔单抗和罗米司亭治疗，但血小板计数并未升高。由于发生有症状的急性硬脑膜下血肿，紧急进行了脾切除术，随后血小板计数恢复正常，无神经后遗症。第二例是一位 66 岁女性，自发性轻度鼻出血导致血小板计数（8×10/L）降低。尽管使用了 IVIg 和皮质类固醇治疗，但患者病情没有改善。随后使用利妥昔单抗和罗米司亭治疗 8 周后，血小板计数恢复正常。据我们所知，这是首例报道的弥漫性皮肤型 SSc 伴抗拓扑异构酶抗体患者发生严重 ITP 的病例。
