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

Abstract

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.

Lupus-Induced Vasculitis and Multiple Organ Dysfunction Syndrome as the First Presentation of Systemic Lupus Erythematosus (SLE) in Pregnancy

“…These complications can include preterm labor, pre-eclampsia, emergent C-section, deep vein thrombosis (DVT), infection, intrauterine growth restriction, intrauterine fetal death, and spontaneous miscarriage. There is also a 20-fold higher risk of maternal death [1]. Our patient was found to have preterm labor, likely related to SLE.…”

“…Managing SLE in pregnancy can be difficult, especially with no previous diagnosis, as seen in our patient. Our patient had a strong family history of SLE in her mother and grandmother, both suffering from their initial manifestation of SLE during their first and only pregnancy, stressing the importance of screening these patients prior to pregnancy [1].…”

“…There is a higher risk of complications in untreated and newly diagnosed patients with SLE during pregnancy [1,3]. However, it is not clear if parity has a higher risk of complications, as the data are controversial [3].…”

“…High clinical consideration, early initial diagnosis, and aggressive treatment with pulse methylprednisolone in combination with cyclophosphamide are the mainstays of treatment for DAH. It is important to have a discussion with the patient about the teratogenic effects of cyclophosphamide, as this drug can cross the placenta and is also associated with premature ovarian failure [1]. The risk of ovarian failure depends on the cumulative effect of the drug dose and the age of the patient, with older women having a higher risk due to less ovarian reserve [3].…”

“…It is most common in African-American and Hispanic women of child-bearing age. Untreated SLE in pregnancy has been related to more adverse outcomes [1,2]. In addition, uncontrolled SLE in pregnancy can be associated with exacerbations requiring immunosuppressive therapy [3], due to hormonal changes involving estradiol, progesterone, and prolactin, leading to a pro-inflammatory state [2].…”