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Abstract: TH-PO527

A Case of Lupus Nephritis (LN) With Concomitant Renal Thrombotic Microangiopathy (TMA) Treated With Eculizumab as a Rescue Treatment

Session Information

Category: Glomerular Diseases

  • 1303 Glomerular Diseases: Clinical‚ Outcomes‚ and Trials

Authors

  • Guevara, Nehemias, St Barnabas Hospital, Bronx, New York, United States
  • Ali, Mahmoud, St Barnabas Hospital, Bronx, New York, United States
  • Morita, Sae, St Barnabas Hospital, Bronx, New York, United States
  • Flores Chang, Marjorie Mailing, St Barnabas Hospital, Bronx, New York, United States
  • Paredes, William Mauricio, St Barnabas Hospital, Bronx, New York, United States
  • Sarikhani, Hamed, St Barnabas Hospital, Bronx, New York, United States
  • Flores Chang, Bessy Suyin, St Barnabas Hospital, Bronx, New York, United States
Introduction

LN is a common and severe consequence of SLE, however, LN and TMA together are not common. When both are present the outcome is worse. To date, there are no clear guidelines for standard therapy, however, eculizumab has been used as rescue therapy with a favorable outcome

Case Description

28 year old female with a PMH of SLE since 2013, LN class IV/V, recurrent serositis, sickle cell trait with chronic pain, schizophrenia, and asthma consulted with encephalopathy and generalized pain. Vital signs: BP: 106/76, HR:100 beats/min, RR: 16 breaths/min, T: 97.5 F, SatO2: 100% on room air. The patient was found to be in acute respiratory failure due to fluid overload and was subsequently intubated. Physical examination: agitated, tachypneic, dry mucous membranes, lung crackles bilaterally in lower fields, S1 S2 present, bilateral lower pitting edema 2+ up to the thighs. Blood work was remarkable for normocytic anemia, hyperkalemia, acute renal failure superimposed to chronic renal failure, transaminitis, and elevated troponin levels. The patient was started on IV antibiotics and emergent hemodialysis. Pulse steroids with solumedrol were also initiated for a possible lupus flare. Immunological workup revealed low C3, C4 with positive anti-double-strand DNA antibodies. Patient underwent a kidney biopsy which revealed focal crescentic glomerulonephritis with membranous and sclerosing features consistent with lupus nephritis class IV (A/C) and V. As well revealed active chronic TMA, moderate tubular atrophy, and interstitial fibrosis. (Images 1,2 3) The patient did not improve with standard therapy (plasmapheresis, mycophenolate, steroids), prompting the use of eculizumab, after which the patient presented improvement (table 1), and dialysis was discontinued

Discussion

TMA associated LN represents a progressive and dismal outcome with variable responses to standard treatment. The mechanism of TMA in autoimmune diseases is associated with the alternative complement pathway where Eculizumab plays an important role and is currently being explored in the treatment for LN. Our patient presented with LN + TMA, failed standard treatment, and improved with Eculizumab. We hope in the near future Eculizumba will be the standard therapy for this type of association