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BMC Nephrol ; 21(1): 293, 2020 07 23.
Artículo en Inglés | MEDLINE | ID: mdl-32703171

RESUMEN

BACKGROUND: Cryoglobulins are cold-precipitable immunoglobulins that may cause systemic vasculitis including cryoglobulinaemic glomerulonephritis (CGN). Type 1 cryoglobulins consist of isolated monoclonal immunoglobulin (mIg), whereas mixed cryoglobulins are typically immune complexes comprising either monoclonal (type 2) or polyclonal (type 3) Ig with rheumatoid activity against polyclonal IgG. Only CGN related to type 1 cryoglobulins has been clearly associated with monoclonal gammopathy of undetermined significance (MGUS) using the conventional serum-, urine- or tissue-based methods of paraprotein detection. CASE PRESENTATION: We present four patients with noninfectious mixed (type 2 or 3) CGN and MGUS. Two patients had type 2 cryoglobulinaemia, one had type 3 cryoglobulinaemia, and one lacked definitive typing of the serum cryoprecipitate. The serum monoclonal band was IgM-κ in all four cases. Treatments included corticosteroids, cyclophosphamide, plasma exchange, and rituximab. At median 3.5 years' follow-up, no patient had developed a haematological malignancy or advanced chronic kidney disease. Other potential causes of mixed cryoglobulinaemia were also present in our cohort, notably primary Sjögren's syndrome in three cases. CONCLUSION: Our study raises questions regarding the current designation of type 2 CGN as a monoclonal gammopathy of renal significance, and the role of clonally directed therapies for noninfectious mixed CGN outside the setting of haematological malignancy.


Asunto(s)
Crioglobulinemia/complicaciones , Crioglobulinas , Glomerulonefritis/complicaciones , Inmunoglobulina M/sangre , Cadenas kappa de Inmunoglobulina/sangre , Gammopatía Monoclonal de Relevancia Indeterminada/complicaciones , Síndrome de Sjögren/complicaciones , Anciano , Crioglobulinemia/sangre , Crioglobulinemia/terapia , Ciclofosfamida/uso terapéutico , Femenino , Glomerulonefritis/terapia , Glucocorticoides/uso terapéutico , Humanos , Factores Inmunológicos/uso terapéutico , Inmunosupresores/uso terapéutico , Masculino , Persona de Mediana Edad , Gammopatía Monoclonal de Relevancia Indeterminada/sangre , Gammopatía Monoclonal de Relevancia Indeterminada/terapia , Intercambio Plasmático , Rituximab/uso terapéutico
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