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

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.