Abstract: TH-PO948
Acute Lupus Hemophagocytic Syndrome as Initial Presentation of Membranous Lupus Nephritis: Case Report with Successful Treatment
Session Information
- Glomerular Trainee Case Reports
November 07, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Report
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Ansari, Sajid Q., University of Illinois at Chicago, Chicago, Illinois, United States
- Lingamaneni, Prasanth, John H. Stroger Jr. Hospital of Cook County, Flossmoor, Illinois, United States
- Osei, Albert M., John H. Stroger Jr. Hospital of Cook County, Flossmoor, Illinois, United States
- Sarmiento, Juan D., John H. Stroger Jr. Hospital, Chicago, Illinois, United States
- Vormittag-Nocito, Erica Rose, University of Illinois, Chicago, Illinois, United States
Introduction
Acute Lupus Hemophagocytic Syndrome(ALHS) is a rare entity. It is highly unusual to diagnose lupus nephritis in an adult presenting as ALHS. Both ALHS and SLE responded well to therapy with MMF with steroids, which has rarely been described in literature
Case Description
40-year-old Hispanic lady with fibromyalgia, hypothyroidism and hypertension, presented with fever, weakness, myalgia and photosensitivity. Physical examination was significant for alopecia and 3/5 muscle strength in lower extremities. No evidence of synovitis or organomegaly.
Investigation: creatinine 0.4, albumin 2.4, AST 168, ALT 52, LDH 387, ALP 108, triglycerides 674, ferritin 3211, hemoglobin 7.1, WBC 0.9, platelets 127, ANA+ (>1:160), +dsDNA, +smithAb, low C3 and C4. UA was positive for RBC and protein. Urine protein/creatinine ratio was 1.2g/day. Bone marrow biopsy was done, showing erythrophagocytosis. Soluble IL-2 receptor (CD25) level and CD8 immune competence panel were normal.
She fulfilled SLICC criteria for SLE. Renal biopsy showed membranous lupus nephritis class V
She was started on prednisone 60 mg daily and MMF 500 mg twice a day and later increased to 1000 mg twice a day and gradually, prednisone was tapered down to 5 mg daily.
Significant improvement in symptoms noted and labs including ferritin, triglycerides and blood counts bacame normal and protenuria resolved.
Discussion
HLH (Hemophagocytic lymphohistiocytosis) is a life-threatening disorder associated with high mortality Secondary HLH results from underlying infectious, malignant and autoimmune conditions. When HLH is attributed to SLE, it is called Acute Lupus Hemophagocytic Syndrome(ALHS). Incidence of HLH in SLE is estimated to be around 0.9% to 4.6% with a mortality rate varying from 5% to 35%. Clinical manifestations include high-grade fever, hepatosplenomegaly, lymphadenopathy and coagulopathy. Abnormal laboratory findings include cytopenias, hyperferritinemia and hypertriglyceridemia.There are no randomized trials to guide therapy. Treatment in ALHS is aimed at managing the underlying condition. If unresponsive Etoposide is used.
This case highlights the importance of early recognition of ALHS, to prevent high morbidity and mortality. MMF and prednisolone induction and maintenance therapy is a viable option in patients with ALHS