Plasma-negative, renal-limited cryofibrinogen associated glomerulonephritis – A unique case report

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Plasma-negative, renal-limited cryofibrinogen associated glomerulonephritis – A unique case report

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Co-author 1

Jarrad Hopkins jarrad.hopkins@sa.gov.au Faculty of Health and Medical Sciences The University of Adelaide Adelaide Australia *

Co-author 2

Ann Nguyen-Hoang Ann.Nguyen-Hoang@sa.gov.a Department of Anatomical Pathology SA pathology Adelaide Australia -

Co-author 3

John Brealey John.Brealey@Sa.gov.a Department of Anatomical Pathology SA pathology Adelaide Australia -

Co-author 4

Pravin Hissaria Pravin.Hissaria@sa.gov.a Faculty of Health and Medical Sciences The University of Adelaide Adelaide Australia -

Co-author 5

Jola Kapojos Jola.Kapojos@sa.gov.a Renal Unit Lyell McEwin Hospital Adelaide Australia -

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Introduction

Cryofibrinogen-associated glomerulonephritis is characterised by membranoproliferative glomerulonephritis without immunoglobulin deposition and unique ultrastructural features. This case report presents a 63-year-old male with renal-limited cryofibrinogen-associated glomerulonephritis, with negative plasma cryofibrinogen levels.

Methods

Patient background: His medical history included metallic aortic valve replacement and long-term anticoagulation therapy. Clinical examination revealed no cutaneous manifestations or thrombotic events. Initial laboratory investigations showed severe renal dysfunction, but multiple negative results for plasma cryofibrinogen, serum cryoglobulin and a comprehensive autoimmune, infective and malignancy panel

Results

Renal biopsy revealed mesangiocapillary glomerulonephritis with focal vasculitis and significant interstitial fibrosis, and electron microscopy identified double-walled microtubules consistent with cryofibrinogen. Our patient was managed without immunosuppressive therapy due to significant renal scarring and absence of extra-renal manifestations.

Conclusion

This case describes the first report of cryofibrinogen-associated glomerulonephritis in the absence of detectable cryofibrinogen in serum, with diagnosis relying on ultrastructural findings. Differential diagnoses such as immunotactoid glomerulonephritis were considered but ruled out based on morphological characteristics. This case adds to the limited literature on renal-limited cryofibrinogen and emphasises the necessity for thorough investigation including electron microscopy assessment of renal biopsies to ascertain the diagnosis.

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