INTRODUCTION: Antiphospholipid Syndrome (APS) is an autoimmune disorder clinically defined by the presence of antiphospholipid antibodies (aPL), history of arterial or deep venous thrombosis (DVT), and/or repeat episodes of early pregnancy loss. Due to the increased risk for thrombosis, often overlooked is a rare and seemingly paradoxical association between APS and diffuse alveolar hemorrhage (DAH) -a disorder characterized by non-localized alveolar bleeding. Rare manifestation of APS presenting with preeclampsia, DAH, and DVT offers a variety of multi-disciplinary challenges.CASE PRESENTATION: A 34-year-old G6P3 woman at 28 weeks gestation with a remote history of pulmonary embolism (PE), presented to the Emergency Department with a two-day history of dyspnea and hemoptysis. Exam was remarkable for sinus tachycardia, grade 3 systolic murmur at the apex, and increased work of breathing with bilateral pulmonary rhonchi. Computed tomography (CT) scan demonstrated centrally predominant opacities without signs of PE. Bronchoscopy was significant for diffusely hemorrhagic airways with progressively hemorrhagic serial lavage aliquots. Emergent delivery was performed in the setting of Preeclampsia while high-dose steroids were administered for DAH. The patient returned to baseline over the next 3 days without returning signs of hemoptysis. Lupus anticoagulant was positive but PT and PTT were both normal. Steroids were discontinued and therapeutic enoxaparin was started for a possible novel diagnosis of APS.Six days later, she reexhibited hemoptysis. Repeat CT revealed bilateral alveolar infiltrates consistent with the recurrence of DAH. Disproportionate left lower extremity edema and a femoral vein DVT were also discovered. Anticoagulation was again withheld, high-dose steroids were re-started, and an inferior vena cava filter was placed. Repeat lupus anticoagulant testing was positive. In addition, the dilute russell viper venom time (DRVV) was prolonged in the setting of normal PT/PTT, confirming APS diagnosis. She recovered within five days and was prescribed warfarin for chronic anticoagulation, glucocorticoids were tapered, while rituximab infusions were initiated as glucocorticoid-sparing therapy.DISCUSSION: This case highlights challenges associated with the diagnosis and management of rare simultaneous manifestations of APS. In particular, the management of DAH in this scenario is unique because unlike the glucocorticoid-responsive antibodies of ANCA-associated vasculitides, aPL are not known to cause alveolar capillaritis; therefore, the role of steroids is unclear.CONCLUSIONS: Chronic anticoagulation is an absolute requirement for long-term management of APS. Yet as this case exemplifies, in the case of concomitant DAH, administration of glucocorticoids, and subsequently glucocorticoid-sparing immunosuppression is recommended as the safest approach to avoid life-threatening hemorrhage.
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