Clinicopathological Spectrum and Treatment Outcomes of Cryofibrinogen-Associated Nephropathies.

ABSTRACT:

Introduction

Cryofibrinogen-associated nephropathy (CFN) is a very rare disease. Only few data are available about the clinicopathological presentation and treatment outcomes.

Methods

Patients with cryofibrinogenemia (CF) diagnosed in French expert laboratories, and kidney biopsy findings suggestive of CFN (pauci-immune membranoproliferative glomerulonephritis [MPGN], thrombotic microangiopathy [TMA] and/or indirect signs of ischemia) were retrospectively included. Estimated glomerular filtration rate (eGFR), urinary protein-to-creatinine ratio (UPCR), and specific treatments were collected. Renal response (RR) was defined as a reduction of UPCR to < 0.5 g/g (or decrease > 50% if > 3 g/g at baseline) and an improvement of eGFR > 30% (if < 60 ml/min per 1.73 m² and

SUBMITTER: Dang J 

PROVIDER: S-EPMC12799572 | biostudies-literature | 2026 Jan

REPOSITORIES: biostudies-literature