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Kidney Week

Abstract: PO0299

Treatment of FSGS and Hemophagocytic Syndrome with Tocilizumab

Session Information

Category: Acute Kidney Injury

  • 102 AKI: Clinical, Outcomes, and Trials


  • Shields, Daniel Wayne, Naval Medical Center Portsmouth, Portsmouth, Virginia, United States
  • Dore, Michael, Naval Medical Center Portsmouth, Portsmouth, Virginia, United States
  • Plasse, Richard A., Naval Medical Center Portsmouth, Portsmouth, Virginia, United States

Hemophagocytic syndrome (HPS) is a rare and often life-threatening condition characterized by an overreaction of the immune system. HPS has a variety of triggers including: malignancy, infection, and rheumatologic conditions. Clinically, it is characterized by acute fever, cytopenia, lymphadenopathy, hepatosplenomegaly, intravascular coagulation, hyperferritinemia, and elevated liver associated enzymes. We present a case of reactive HPS complicated by focal segmental glomerulonephritis (FSGS) secondary to a febrile gastrointestinal illness in an otherwise healthy 36 year old male.

Case Description

A 36 year old male presented to the ER with a 3 day history of fevers, nausea, vomiting, and diarrhea. Labs demonstrated hyponatremia to 126 and acute renal injury. He was admitted for presumed viral gastroenteritis and treated supportively. He then developed elevated liver associated enzymes and pancytopenia. An infectious work up was unrevealing. Flow cytometry was negative for lymphoma or leukemia. Ferritin was elevated at 4450 ng/ml. A bone marrow biopsy demonstrated hemophagocytosis. He then developed multiple pulmonary embolisms, lower extremity edema, new onset ascites, and nephrotic range proteinura. A renal biopsy showed diffuse podocyte effacement with rare Focal Segmental Glomerulosclerosis (FSGS) with collapsing features and no immune complexes. His IL-2 soluble receptor was elevated at 2710 µg/mL. He was diagnosed with HPS, started on prednisone 1 mg/kg then transitioned to tocilizumab.


The pathogenesis of HPS is excessive activation and proliferation of T lymphocytes and macrophages leading to phagocytosis of hematopoietic cells in the bone marrow and hypersecretion of proinflammatory cytokines causing multi organ dysfunction. Due to the difficulty in diagnosis, the Hscore was developed to estimate the probability of HPS. Our patient’s Hscore was 209 equating to an 88-93% probability. Renal dysfunction occurs in ~16% of patients with HPS with symptoms ranging from hyponatremia to renal failure. Nephrotic syndrome is most commonly due to collpasing FSGS. Case reports have demonstrated the use of tocliziumab in the treatment of FSGS from other etiologies, but treatment of HPS and FSGS has not been reported. Our patient had resolution of his nephrotic syndrome with immunosuppressive therapy with steroids and remain in remission on tocilizumab.