Clinical Spectrum, Treatment, and Outcome of Patients with Type II Mixed Cryoglobulinemia without Evidence of Hepatitis C Infection

Foessel, L; Besancenot, J.-F.; Blaison, G.; Magy-Bertrand, Nadine; Jaussaud, R.; Étienne, Yves; Maurier, F.; Audia, Sylvain; Martin, Thierry

doi:10.3899/jrheum.100898

Cited by 37 publications

(43 citation statements)

References 27 publications

(21 reference statements)

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“…Anti‐HCV antibodies and HCV RNA have been detected in cryocrit, and HCV core proteins are observed in cutaneous and kidney deposits . Although, HCV infection is actually considered to be the main cause of MC‐related vasculitis, 25% to 50% of cases of non‐HCV cryoglobulinemia remain of undetermined aetiology …”

Section: Introductionmentioning

confidence: 99%

Hepatitis E virus–associated cryoglobulinemia in solid‐organ–transplant recipients

Marion

Abravanel

Bello

et al. 2018

Liver International

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Section: Introductionmentioning

confidence: 99%

Hepatitis E virus–associated cryoglobulinemia in solid‐organ–transplant recipients

Marion

Abravanel

Bello

et al. 2018

Liver International

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“…This is partly because of limited biochemical analysis in these studies, which tended to focus only Conditions other than MGUS were present in our cohort potentially accounting for the development of mixed CGN. Thus pSS, which represents the commonest cause of mixed cryoglobulinaemia/CGN after HCV infection (8,9,11,28,29), was present at time of diagnostic renal biopsy in three patients (conforming to current EULAR/ACR criteria (31)). pSS involves a disease process of continuous polyclonal B cell activation, with malignant transformation of B cell clones in some cases, based on the increased incidence of lymphoma in patients with pSS (32, 33) (especially those with mixed cryoglobulinaemia (34,35)).…”

Section: Discussionmentioning

confidence: 96%

“…We report a series of four patients with noninfectious mixed CGN in whom MGUS was diagnosed using the conventional methods for paraprotein detection (16,27). One in every five patients assessed in our hospital cohort of noninfectious mixed CGN was found to have MGUS, although the true incidence of this association remains uncertain owing to a paucity of data in the major published series (11,28,29). This is partly because of limited biochemical analysis in these studies, which tended to focus only Conditions other than MGUS were present in our cohort potentially accounting for the development of mixed CGN.…”

Section: Discussionmentioning

confidence: 99%

Noninfectious mixed cryoglobulinaemic glomerulonephritis associated with monoclonal gammopathy of undetermined significance

Flavell

Fullinfaw

Smith

et al. 2020

Preprint

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Background Cryoglobulins are cold-precipitable immunoglobulins that may be found in association with 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. Methods We retrospectively assessed our patient cohort of CGN related to mixed (type 2 or 3) cryoglobulins for those with a monoclonal band on serum protein electrophoresis (SPEP), with exclusion of infectious cases and those involving malignant clonal plasma cell or B cell disorders. Results We identified four patients with a median age of 54 years, including three women. Two patients had type 2 cryoglobulinaemia, one had type 3 cryoglobulinaemia, and one lacked definitive typing of the serum cryoprecipitate. The serum monoclonal band identified on SPEP 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. Conclusions Our study raises questions regarding the attribution of causation to MGUS, and the role of clonally directed therapies outside the setting of haematological malignancy.

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“…To our knowledge, this is the first case report of a patient with mixed cryoglobulinaemia associated with non-Hodgkin's lymphoma who presented with nephritis and acute renal failure [7, 8]. …”

Section: Discussionmentioning

confidence: 99%

Successful Treatment of Cryoglobulinaemia with Rituximab

Choudhry

Rao

Juneja

2012

Case Rep Nephrol Urol

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Cryoglobulinaemia is a systemic inflammatory condition characterised by immune complex-mediated small-to-medium-sized vasculitis. It has a wide variety of presentations ranging from bruising, neuropathy, and hepatosplenomegaly to acute renal failure. Mixed cryoglobulinaemia is the most common type and is strongly associated with hepatitis C. Management approaches include use of cyclophosphamide, prednisolone, and plasmapheresis, with differing views on alternative treatments in resistant cases. Rituximab has emerged as an attractive option in resistant cases on account of its potent immunosuppressive effects on B cells. We describe a case of type 2 mixed cryoglobulinaemia in association with non-Hodgkin’s lymphoma resistant to standard treatments which responded well to rituximab. This case is remarkable as mixed cryoglobulinaemia associated with non-Hodgkin’s lymphoma presenting with nephritis is unusual, and, contrary to the high rate of recurrence in lymphoma-related cryoglobulinaemia, our patient has not shown any recurrence over 24 months. This highlights an alternative treatment modality which can be used in patients not responsive to existing managements for this condition with benefits of minimal side effects and no oncogenetic potential.

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Clinical Spectrum, Treatment, and Outcome of Patients with Type II Mixed Cryoglobulinemia without Evidence of Hepatitis C Infection

Cited by 37 publications

References 27 publications

Hepatitis E virus–associated cryoglobulinemia in solid‐organ–transplant recipients

Hepatitis E virus–associated cryoglobulinemia in solid‐organ–transplant recipients

Noninfectious mixed cryoglobulinaemic glomerulonephritis associated with monoclonal gammopathy of undetermined significance

Successful Treatment of Cryoglobulinaemia with Rituximab

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