Severe Maternal Morbidity Associated with Systemic Lupus Erythematosus Flare in the Second Trimester of Pregnancy

Pregnancy in women with systemic lupus erythematosus (SLE) is associated with an increased risk of adverse maternal and fetal outcomes. Here, we present a case of severe maternal morbidity in a 23-year-old primigravida with SLE and secondary Sjögren's syndrome who experienced a life-threatening multisystem flare at 17 weeks of gestational age. She presented to the emergency department complaining of cough with hemoptysis and shortness of breath. She developed hypoxic respiratory failure and was admitted to the intensive care unit. Bronchoscopy confirmed diffuse alveolar hemorrhage. Physical exam and laboratory evaluation were consistent with an active SLE flare, pancytopenia, and new-onset lupus nephritis. After counseling regarding disease severity, poor prognosis, and recommendation for therapy with cytotoxic agents, she agreed to interruption of pregnancy which was achieved by medical induction. Her course was further complicated by thrombotic microangiopathy and generalized tonic-clonic seizures attributable to posterior reversible encephalopathy syndrome versus neuropsychiatric SLE. This case represents one of the most extreme manifestations of lupus disease activity associated with pregnancy that has been reported in the literature and emphasizes the importance of preconception evaluation and counseling and a multidisciplinary management approach in cases with a complex and evolving clinical course.