Abstract: PUB404
Rituximab to the Rescue in Refractory Anti-GBM Disease
Session Information
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Upadrista, Pratap Kumar, Northwell Health, New Hyde Park, New York, United States
- Sharma, Purva D., Northwell Health, New Hyde Park, New York, United States
- Bhenswala, Prashant N., Northwell Health, New Hyde Park, New York, United States
Introduction
Anti GBM antibody disease is an aggressive disease that presents with rapidly progressive glomerulonephritis and pulmonary involvement with diffuse alveolar hemorrhage. The standard treatment includes steroids, PLEX and Cytoxan until anti-GBM Ab titers become negative. However, some cases are refractory to this conventional therapy . We present here such a case of anti GBM disease with severe lung and kidney involvement that did not respond to PLEX, cyclophosphamide and steroids with persistently high anti GBM Ab titers treated successfully with Rituximab.
Case Description
A 65 y/o M with Hx of DM, HLD, MVR and CABG presented with dyspnea, fatigue, hemoptysis and oliguria for a week. He had a baseline serum creatinine of 0.84 mg/dL. He was a non-smoker and did not use any illicit drugs. At presentation, he was anemic (7.1 g%) from his baseline of 10 g% and had anuric AKI with a Scr of 3.6 mg/dL which peaked at 6.3 mg/dL and was started on HD. He had 2.9g proteinuria and hematuria. CT chest GGOs suggestive of hemorrhage vs pulmonary edema. Work up showed anti GBM antibodies >8.0. The pt was initiated on daily PLEX (1 Plasma volume with 5% albumin + Plasma) pulse followed by oral steroids and oral cyclophosphamide. Renal biopsy showed anti-GBM glomerulonephritis with segmental glomerular necrosis and cellular crescents (88%). As his Anti-GBM antibodies titers were still >8, even after 14 sessions of PLEX, Cytoxan and Steroids. Rituxan was started for refractory anti GBM abs with pulmonary symptoms. The patient continued to be on hemodialysis. Within 2 months, his anti GBM ab titers slowly improved to negative, with improved pulmonary symptoms. Kidney transplant eval is underway.
Discussion
Management of refractory anti GBM disease with dialysis dependent AKI and lung hemorrhage with persistently high Ab titers even after PLEX, Cytoxan and steroids is challenging. There is limited literature on use of Rituximab to achieve remission. Rituxan removes CD 20 positive B cells by inducing apoptosis, antibody and complement mediated cytotoxicity resulting in lowered anti GBM ab production. Our patient was successfully managed with Rituximab with improvement in lung hemorrhage and enabling him to get kidney transplant evaluation.