Abstract: SA-PO0709
Steroid-Responsive Cryofibrinogen-Associated Kidney Disease: A Case of Relapse and Remission
Session Information
- Glomerular Diseases: Complement-Mediated Glomerulopathies and Infection-Related GN
October 24, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Author
- Gulden, Erin Kayleigh, San Antonio Uniformed Services Health Education Consortium, Joint Base San Antonio Fort Sam Houston, Texas, United States
Introduction
Cryofibrinogen associated kidney disease is a rare cause of nephrotic syndrome characterized by glomerular deposition of fibrinogen containing complexes, typically producing a membranoproliferative pattern of injury. Its clinical course and management remain poorly understood, with available evidence limited to case reports. This is a case of cryofibrinogenic glomerulopathy that underwent spontaneous remission followed by relapse, ultimately demonstrating a therapeutic response to corticosteroids.
Case Description
A 72 year old man presented with edema and nephrotic range proteinuria (UPCR 10 g/g). Kidney biopsy revealed a membranoproliferative pattern with organized electron dense deposits and immunofluorescence, findings consistent with cryofibrinogenic glomerulopathy. Evaluation for secondary causes, including malignancy, autoimmune disease, infection, and cryoglobulins, was unrevealing. Initial management with renin angiotensin aldosterone system (RAAS) blockade led to partial improvement followed by spontaneous remission. Five months later, he relapsed with recurrent edema, hypoalbuminemia, and proteinuria >7 g/g.
In the absence of established treatment guidelines, high dose prednisone (60 mg daily) was initiated. Within four weeks, proteinuria declined by >50%, serum albumin normalized, and edema resolved. Telmisartan uptitration further reduced proteinuria to 1.28 g/g, and empagliflozin was added for additional antiproteinuric benefit. Kidney function remained stable (creatinine 1.2 mg/dL), and no systemic features of cryofibrinogenemia emerged over nine months of follow-up.
Discussion
This case highlights the rarity of cryofibrinogen associated kidney disease, the frequent absence of identifiable secondary triggers, and the uncertainty surrounding optimal management. The patient’s spontaneous remission followed by relapse also underscores the need for continued surveillance. Although prior reports describe variable responses to immunosuppression, the improvement with glucocorticoids in this case suggests that select patients may benefit from steroid therapy. Adjunctive measures, including RAAS blockade and SGLT2 inhibition, contributed to sustained proteinuria reduction and preservation of kidney function. This case adds to the limited literature and reinforces the need for further research into treatment options.