A Fatal Case of Systemic Lupus Erythematosus Complicated with Diffuse Alveolar Hemorrhage

Abstract

Introduction: SLE is a multisystem autoimmune disease with variable clinical presentation. DAH is a rare but catastrophic manifestation of SLE with high mortality, requiring early, intensive therapy. Case Presentation: A 31-year-old female presented with low-grade fever and joint pains for six weeks associated with alopecia, anorexia, and weight loss. She had pale and had cervical lymphadenopathy, moderate hepatomegaly, and splenomegaly. Hb level was 9.2mg/dL, with a platelet count of 144 × 103/µL. The ESR was 65mm/hour, and the CRP level was 36 mg/L with a UPCR of 332mg/g. She had low C3, C4 levels, positive ANA, and dsDNA titer. A renal biopsy revealed class 3 lupus nephritis. Twenty days after, she was readmitted with acute onset of pleuritic chest pain, cough, and dyspnea, where she was hemodynamically unstable with SpO₂ of 85% on air. The Hb level was 8.6 g/dL with a platelet count of 106 × 103/µL. Her condition deteriorated despite standard medical care, where the Hb level and SpO₂ dropped to 5.6 d/dL and 65%, respectively, even after blood transfusions, intubation, and artificial ventilation. The chest X-ray revealed bilateral large whitish hazy shadows, and the HRCT scan revealed diffuse bilateral pulmonary hemorrhages. Then she was transferred to the ICU, and there she was started with broad-spectrum antibiotics, methylprednisolone, and plasmapheresis. However, after one week of ICU stay, her renal functions worsened, and she was initiated on CRRT. However, despite all the resuscitation efforts, she succumbed following a cardiac arrest. Conclusion: DAH is a rare catastrophic complication of SLE, which usually presents in patients with an established diagnosis of SLE, even on medical therapy. The diagnosis of DAH is problematic because it mimics a severe pulmonary infection. Early detection and aggressive management are warranted to improve affected patients' outcomes and quality of life.