Abstract: SA-PO0758
Refractory Diffuse Alveolar Hemorrhage and Complement-Mediated Thrombotic Microangiopathy with Lupus Nephritis
Session Information
- Glomerular Diseases: Lupus Nephritis, Monoclonal Gammopathy-Related Disease, and Transplantation
October 24, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Alamri, Nada, Madinah Health Cluster, Medina, Saudi Arabia
- Alduraibi, Fatima, King Faisal Specialist Hospital and Research Centre, Riyadh, Riyadh Province, Saudi Arabia
Introduction
Diffuse alveolar hemorrhage (DAH) and thrombotic microangiopathy (TMA) are rare complications of SLE. Evidence guiding escalation therapy, complement inhibition, and relapse management remains limited.
Case Description
A 28-year-old woman presented with new-onset nephrotic syndrome and was diagnosed with SLE. Lab showed positive ANA, anti-dsDNA >800, low C3/C4 , and negative antiphospholipid antibodies. On hospital day 3, she developed hypoxia and hemoptysis requiring ICU transfer. CT suggested DAH, confirmed by bronchoscopy showing bleeding and hemosiderin-laden macrophages. She received pulse methylprednisolone, cyclophosphamide, PLEX and IVIG. Despite daily then alternate-day PLEX with IVIG, she deteriorated, requiring high-FiO2 ventilation and CRRT for anuric kidney injury. Persistent DAH required additional methylprednisolone pulses. The scheduled second dose of cyclophosphamide was givenand two 1-g doses of rituximab were added for refractory DAH. On ICU day 25, after receiving 21 PLEX, her platelet fell to <20 with MAHA, purpura, and >10% schistocytes. ADAMTS13 was not consistent with TTP and antiphospholipid antibodies remained negative. Presumed complement-mediated TMA was considered and ravulizumab was given. By ICU day 31, her urine output improved, pulmonary bleeding stopped and she was weaned to tracheostomy with low-flow oxygen. Biopsy showed class III focal proliferative LN with class V membranous LN. Cyclophosphamide was stopped after 4 doses because of cytopenias and ESBL Klebsiella pneumonia. Gentic testing showed heterozygous CFHR1-CFHR3 deletion, a susceptibility risk factor of primary aHUS; ravulizumab was not continued after TMA resolution. She transitioned to mycophenolate and prednisone. Her tracheostomy was closed, she was discharged home on room air, and Cr stabilized at 200 µmol/L with improved anti-dsDNA and normalized complement. One year later, she devloped recurrent DAH, confirmed by bronchoscopy, with ESBL-positive sputum C/S but no recurrent TMA. She improved with 10 PLEX, IVIG, antibiotics, rituximab, and mycophenolic acid escalation. She was again discharged home. At 3 months, anti-dsDNA decreased and complement remained normal.
Discussion
This case presents a treatment pathway for severe LN-associated DAH with presumed CM- TMA, including rescue ravulizumab and relapse treatment with PLEX, IVIG, antibiotics, and intensified mycophenolate.