Cryoglobulinemic glomerulonephritis: clinical presentation and histological features, diagnostic pitfalls and controversies in the management. State of the art and the experience on a large monocentric cohort treated with B cell depletion therapy.

Abstract

Cryoglobulinemia is defined by the presence of immunoglobulins having the following characteristics: forming a gel when temperature is <37 °C, precipitate in a reversible manner in the serum, and redissolve after rewarming. The presence of both polyclonal IgG and monoclonal IgM (type II), or of polyclonal IgG and polyclonal IgM (type III) identifies the mixed cryoglobulinemia (MC). The identification of the Hepatitis C virus (HCV) infection in most of the cases previously defined as "essential" represented a cornerstone in the understanding the pathogenesis of this condition. The picture of MC comprehends heterogeneous clinical presentations: from arthralgias, mild palpable purpura, fatigue to severe vasculitis features with skin necrotic pattern, peripheral neuropathy and, less commonly, lungs, central nervous system, gastrointestinal tract, and heart involvement. The kidney represents the most common organ presentation, and the presence of glomerulonephritis is a key element when considering prognosis. We discuss the clinical presentation and histological features, diagnostic pitfalls, and controversies in the management of patients with cryoglobulinemic glomerulonephritis, with a special focus on reporting our experience in treating patients with B cell depletion therapy.

• Authors: Daniela Rossi, Savino Sciascia, Roberta Fenoglio, Michela Ferro, Simone Baldovino, Joelle Kamgaing, Federica Ventrella, Ileana Kalikatzaros, Lucia Viziello, Laura Solfietti, Antonella Barreca, Dario Roccatello
• Journal: Minerva medica (Minerva Med) Vol. 112 Issue 2 Pg. 162-174 (Apr 2021) ISSN: 1827-1669 [Electronic] Italy
• PMID: 33198442 (Publication Type: Case Reports, Journal Article, Review)

Chemical References

• Antiviral Agents
• Cryoglobulins
• Immunoglobulin G
• Immunoglobulin M
• Immunologic Factors
• Immunosuppressive Agents
• Rituximab

Topics

• Antiviral Agents (adverse effects, therapeutic use)
• Arthralgia (etiology)
• B-Lymphocytes
• Biopsy
• Cohort Studies
• Cryoglobulinemia (complications, diagnosis, pathology, therapy)
• Cryoglobulins (isolation & purification)
• Diagnostic Errors
• Fatigue (etiology)
• Glomerulonephritis (diagnosis, etiology, pathology, therapy)
• Glomerulonephritis, Membranoproliferative (pathology)
• Glomerulosclerosis, Focal Segmental (pathology)
• Hepatitis C, Chronic (complications, diagnosis, drug therapy)
• Humans
• Immunoglobulin G (blood)
• Immunoglobulin M (blood)
• Immunologic Factors (therapeutic use)
• Immunosuppressive Agents (therapeutic use)
• Kidney (pathology)
• Lymphocyte Depletion (methods)
• Male
• Middle Aged
• Plasma Exchange
• Prognosis
• Prospective Studies
• Purpura (drug therapy, etiology, pathology)
• Rituximab (therapeutic use)
• Vasculitis (etiology)