Abstract: PO1826
Neurorenal Syndrome: Two Cases of Tip-Variant Focal Segmental Glomerulosclerosis Associated with Guillain-Barré Syndrome
Session Information
- Glomerular Diseases: IgA, C3G, and FSGS
October 22, 2020 | Location: On-Demand
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Report
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Hager, Drew, University of Manitoba College of Medicine, Winnipeg, Manitoba, Canada
- Zacharias, James M., University of Manitoba College of Medicine, Winnipeg, Manitoba, Canada
- Gibson, Ian W., University of Manitoba College of Medicine, Winnipeg, Manitoba, Canada
- Hingwala, Jay P., University of Manitoba College of Medicine, Winnipeg, Manitoba, Canada
Introduction
Glomerular disorders have been associated with immune-mediated polyneuropathies in previous case reports. We present two cases of tip-variant focal segmental glomerulosclerosis (FSGS) associated with a variant of Guillain-Barré syndrome (GBS) in Winnipeg, Manitoba.
Case Description
Two previously healthy males aged 62- and 55-years old presented to our hospital with extremity weakness and paresthesias. They each progressed to flaccid paralysis and respiratory failure despite IV immunoglobulin (IVIG) and plasma exchange therapy (PLEX). Initial investigations were consistent with Acute Motor-Sensory Axonal Polyneuropathy (AMSAN), a variant of GBS. Nephrotic syndrome was identified after four months in case one and immediately in case two. Each patient had 20 g/day of proteinuria, preserved renal function, and histologic diagnosis of tip-variant FSGS on renal biopsy. Both cases responded to high-dose corticosteroids initially. Case one relapsed during his taper requiring re-initiation of steroids and addition of mycophenolate mofetil (MMF). He was discharged following thirteen months in hospital with complete remission of proteinuria and ongoing neurologic recovery. Case two achieved complete remission of proteinuria and was discharged after six months with ongoing neurologic recovery.
Discussion
Our cases have similar presentations and responses to therapy suggesting they may share a common circulating autoantibody reacting against shared neural and glomerular podocyte antigens. Circulating autoantibodies including anti-contactin-1 and neurofascin have previously been implicated in chronic inflammatory demyelinating polyneuropathy (CIDP), a chronic variant of GBS. Identifying the culprit immune target in primary FSGS is limited due to the absence of immune complex deposition.
The timing of podocytopathy development compared to GBS is highly variable in cases reported throughout the literature. The onset and diagnosis of FSGS in Case 1 was either delayed or unrecognized illustrating the importance of educating clinicians about this neuro-renal syndrome.
Although not routinely used in GBS, corticosteroids have led to favorable outcomes in our cases and those reported throughout the literature. Recognition of a co-existing nephrotic syndrome with GBS could significantly change management and impact treatment outcomes.