Yousif Patrick A, Moshrefi Hameadreza, Meysami Alireza, Alkhatib Ayad H
Department of Internal Medicine, Detroit Medical Center Sinai-Grace Hospital/Wayne State University, Detroit, MI, USA.
Department of Rheumatology, Henry Ford Hospital/Wayne State University, Detroit, MI, USA.
Am J Case Rep. 2020 Apr 14;21:e921299. doi: 10.12659/AJCR.921299.
BACKGROUND Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by autoantibody production leading to inflammation in multiple organs; it commonly affects young women in their child-bearing years. Clinical manifestations are diverse and range from mild arthritis to diffuse alveolar hemorrhage (DAH). DAH is a rare and devastating complication of SLE that carries a mortality rate of up to 50%, despite aggressive therapy. CASE REPORT A 21-year-old primigravida at 16 weeks gestation presents with a productive cough, rash, sore throat, and high-grade fever. Chest x-ray suggested multifocal pneumonia. Patient deteriorated despite antibiotics and intravenous (IV) fluids. She developed worsening anemia, leukopenia, and thrombocytopenia. Autoimmune workup was positive for Coombs, antinuclear antibody, anti-smith antibody, and hypocomplementemia. Skin biopsy was consistent with SLE. SLE vasculitis was suspected. She required mechanical intubation for rapid respiratory deterioration, with CT thorax suggesting ARDS. Bronchoscopy was done and confirmed DAH. Her course was further complicated with retinopathy and acute pancreatitis associated with SLE. She was treated with IV steroids, IV cyclophosphamide, and plasmapheresis, with significant clinical improvement and successful extubation. She delivered a healthy baby at 32 weeks gestation. CONCLUSIONS Early recognition and initiation of treatment is critical to survival in DAH and requires a high index of clinical suspicion. Treatment includes high-dose steroids, cyclophosphamide, and plasma exchange. Pregnancy increases the risk of adverse outcome in SLE. Seven cases of DAH in pregnant patients with SLE have been reported. Here, we report a catastrophic presentation of DAH, acute pancreatitis, and retinopathy in a pregnant patient with newly diagnosed SLE.
系统性红斑狼疮（SLE）是一种自身免疫性疾病，其特征是产生自身抗体，导致多个器官发生炎症；该病通常影响育龄期年轻女性。临床表现多样，从轻度关节炎到弥漫性肺泡出血（DAH）不等。DAH是SLE一种罕见且严重的并发症，尽管积极治疗，死亡率仍高达50%。
一名孕16周的21岁初产妇，出现咳嗽、皮疹、咽痛和高热。胸部X线提示多灶性肺炎。尽管使用了抗生素和静脉输液，患者病情仍恶化。她出现了贫血、白细胞减少和血小板减少加重。自身免疫检查发现抗人球蛋白试验、抗核抗体、抗史密斯抗体阳性及补体降低。皮肤活检结果符合SLE。怀疑为SLE血管炎。因呼吸迅速恶化，她需要机械通气，胸部CT提示急性呼吸窘迫综合征（ARDS）。进行了支气管镜检查，确诊为DAH。她的病情因与SLE相关的视网膜病变和急性胰腺炎而进一步复杂化。她接受了静脉注射类固醇、静脉注射环磷酰胺和血浆置换治疗，临床症状明显改善并成功脱机。她在孕32周时分娩了一个健康的婴儿。
早期识别和开始治疗对于DAH患者的生存至关重要，需要高度的临床怀疑指数。治疗包括大剂量类固醇、环磷酰胺和血浆置换。妊娠会增加SLE患者出现不良结局的风险。已有7例SLE孕妇发生DAH的报道。在此，我们报告了一名新诊断为SLE的孕妇发生DAH、急性胰腺炎和视网膜病变的灾难性病例。
