Management of Systemic Lupus Erythematosus complicated with Refractory Immune Thrombocytopenic Purpura and Pulmonary Artery Hypertension

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Abstract

The coexistence of immune thrombocytopenia (ITP) and pulmonary artery hypertension (PAH) is rarely observed as the initial manifestation of systemic lupus erythematosus (SLE), often leading to delayed diagnosis and poor outcome. We presented the case of an 18-year-old female of Asian origin with severe ITP and PAH as the initial manifestation of SLE. The patient was successfully treated with a combination of methylprednisolone, mycophenolate mofetil, sildenafil, and vinca alkaloid (vincristine). This case provided the opportunity to increase awareness of an uncommon association between SLE complicated with ITP and PAH and suggest a positive impact of early diagnosis and appropriate treatment on the patient’s outcome. The use of vincristine was considered as per the guideline on refractory ITP before referral for splenectomy.
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系统性红斑狼疮合并难治性免疫性血小板减少性紫癜和肺动脉高压的治疗
免疫性血小板减少症(ITP)和肺动脉高压(PAH)共存作为系统性红斑狼疮(SLE)的初始表现很少观察到,往往导致诊断延迟和预后不良。我们提出的情况下,18岁的女性亚洲血统的严重ITP和PAH作为SLE的初始表现。患者成功地接受了甲基强的松龙、霉酚酸酯、西地那非和长春花生物碱(长春新碱)的联合治疗。该病例提供了一个机会,提高了人们对SLE合并ITP和PAH之间罕见关联的认识,并提示早期诊断和适当治疗对患者预后有积极影响。在转诊行脾切除术前,根据难治性ITP指南考虑使用长春新碱。
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