LUNG IS A BATTLEFIELD: DIFFUSE ALVEOLAR HEMORRHAGE IN ANTIPHOSPHOLIPID SYNDROME

PV281 / #595 Case Report Poster Topic: AS03 - Antiphospholipid Syndrome Introduction This case highlights a rare and life-threatening complication of catastrophic antiphospholipid syndrome (CAPS) – diffuse alveolar hemorrhage (DAH). We emphasize that early recognition and treatment of DAH is vital due to significant morbidity and mortality. Case Presentation With Investigation A 41-year-old woman with a history of hypertension, stage 4 chronic kidney disease, and triple positive antiphospholipid syndrome (APS) complicated by ischemic stroke, cerebral venous sinuous thrombosis on warfarin, spontaneous abortion, and multiple deep vein thromboses presented with increasing confusion. Her creatinine and blood urea nitrogen were rising (baseline creatinine 2.65 mg/dL to 3.63 mg/dL; BUN 68 mg/dL to 84 mg/dL), she developed oliguria, wasadmitted for progressive renal failure requiring intermittent hemodialysis, and started on IV heparin She became febrile and developed acute hypoxic respiratory failure with worsening encephalopathy, prompting transfer to the medical ICU for intubation and continuous renal replacement therapy. She underwent imaging which was negative for pulmonary embolism but demonstrated diffuse patchy airspaces, pulmonary vascular congestion, airspace infiltration, and a left lower lobe consolidation (Figure 1A-B). She developed hemoptysis, was intubated due to decompensationand underwent bronchoscopy with gross bloody return. Hemoglobin and platelets trended down, raising concern for CAPS complicated by DAH. She met criteria for probable CAPS for triple positive antiphospholipid antibodies (aPL), and involvement of renal, respiratory, and neurological systems, though histological evidence of intravascular thrombosis was not obtained. Hematology was consulted, and high-dose glucocorticoids and plasma exchange therapy (PLEX) were initiated. She received daratumumab for immunosuppression. The patient was extubated on day 5 of PLEX and 2 days after starting daratumumab. Figure 1. (A) Chest X-ray, AP view, diffuse airspace infiltrates, pulmonary vascular congestion. (B) CT angiogram, coronal view, scattered groundglass and consolidative opacities, extensive airspace infiltration. Literature Review Antiphospholipid syndrome (APS) is characterized by recurrent thrombotic events, and 30-40% of those with systemic lupus erythematous are positive for aPL.[1-2] A rare complication of APS is CAPS, where microvascular thrombi result in multiorgan failure, under 10% with CAPS experience DAH.[2] DAH in APS is attributed to an inflammatory process of up-regulated endothelial cell adhesion with neutrophil recruitment causing tissue destruction, and complement 5 activation mediating capillaritis.[1] Both processes result in hemorrhage precipitated by aPL.[1] Anti-beta-2-glycoprotein I antibodies, specifically, bind to platelets leading thrombosis.[1-2] Management includes anticoagulation and ventilatory support with high positive end expiratory pressure to reduce active lung bleeding.[3] PLEX has been shown to be beneficial in removal of antibodies and other cytokine mediators.[1-3] High-dose glucocorticoids remain a mainstay of treatment.[1-3] Immunotherapy has expanded to include the use of daratumumab to target long-lived plasma cells to induce antibody-dependent cellular cytotoxicity and complement-dependent cytotoxicity creating a degree of synergism.[3] Discussion This case underscores that DAH, although rare, is life-threatening and necessitates prompt identification and intervention. Clinicians should have a high index of suspicion for DAH when patients with APS develop respiratory compromise and hemoptysis. This case also reinforces the value of a multidisciplinary approach in patients with APS, combining critical care, hematology, nephrology, and rheumatology expertise to manage the complex interplay of thrombotic, inflammatory, and bleeding risks. The literature supports the use of high-dose glucocorticoids and PLEX, but use of daratumumab in severe CAPS is rare, and clinical trials are still ongoing. References: [1.] Cartin-Ceba R. Arthritis Care Res (Hoboken) 2014;66:301-10. [2.] Loza C. Case Rep Rheumatol 2019:3284258. [3.] Yun Z. Front Immunol 2023;14:1144145.

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