Successful Use of Intravenous Immunoglobulin (IVIG) in Severe Life-Threatening Hypoalbuminemia in a Young African American (AA) Male with FSGS
- Glomerular Diseases: From Inflammation to Fibrosis - II
November 03, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1401 Glomerular Diseases: From Inflammation to Fibrosis
- Hamed, Basant, SUNY Downstate Health Sciences University, New York City, New York, United States
- Sasidharan, Sandeep Raja, SUNY Downstate Health Sciences University, New York City, New York, United States
- Salifu, Moro O., SUNY Downstate Health Sciences University, New York City, New York, United States
- Bamgbola, Oluwatoyin F., SUNY Downstate Health Sciences University, New York City, New York, United States
- Mongia, Anil K., SUNY Downstate Health Sciences University, New York City, New York, United States
Nephrotic syndrome (NS) relapses are common and are mostly managed by intensified doses of steroids with or without IV albumin infusion. In some instances, the relapses may be steroid resistant thereby warranting alternative immunosuppressive modalities. IVIG therapy has shown beneficial effects in patients with certain forms of glomerulonephritis but has been rarely used in steroid resistant NS patients. We hereby report an AA male adolescent with long standing FSGS who presented with NS relapses that are characterized by unusual severe life-threatening hypotensive crisis that was successfully treated with IVIG therapy.
A 21-year-old AA male with known history of infrequent relapses of steroid resistant NS since the age of 3 yrs. At the age 18 years, he started having frequent relapses (9 events per year) and a repeat renal biopsy showed FSGS with 40% tubular atrophy and scarring. Relapses were characterized by precipitous events of heavy proteinuria (10-20 gm/day), severe hypoalbuminemia (< 1 gm /dL), and a peripheral circulatory failure. The latter was complicated by cerebral hypoperfusion with a syncope and atrial fibrillation. He was not responsive to Mycophenolate Mofetil and Rituximab. There was an interval event of COVID-19 infection that warranted monoclonal antibody. Patient was reluctant to undergo a proposed medical and surgical nephrectomy. Given a concurrent severe hypoalbuminemia and hypogammaglobinemia, an empirical treatment with a sequential IVIG was commenced. Consequently, frequency and severity of relapses had reduced for the past 10 months with no subsequent ICU admissions.
His serum creatinine has always been normal (0.4-0.7 mg/dl). Genetic studies showed heterozygous APOL1 haplotypes of G1 and G2 variants.
We present a rare case of life-threatening episodes of severe NS relapses in a patient with FSGS and G1/G2 APOL1 gene mutation who had a superimposed COVID-19 infection that responded to serial doses of IVIG therapy. We believe the beneficial effect of IVIG might be related to an increase in intravascular oncotic pressure and immunologic effects. IVIG therapy may be considered in patients with staroid resistant NS that failed to respond to conventional immunosuppressive modalities.