The characteristics of seronegative and seropositive non-hepatitis-associated cryoglobulinemic glomerulonephritis Journal Article
| Authors: | Javaugue, V.; Valeri, A. M.; Jaffer Sathick, I.; Said, S. M.; Erdogan Damgard, S.; Murray, D. L.; Klobucher, T.; Andeen, N. K.; Sethi, S.; Fervenza, F. C.; Leung, N.; Nasr, S. H. |
| Article Title: | The characteristics of seronegative and seropositive non-hepatitis-associated cryoglobulinemic glomerulonephritis |
| Abstract: | The clinicopathologic characteristics and long-term outcome of non-hepatitis-associated cryoglobulinemic glomerulonephritis (CryoGN) are not well-defined and cases with undetectable serum cryoglobulin (seronegative CryoGN) have not been investigated. To resolve this, we retrospectively identified 81 patients with biopsy-proven non-hepatitis CryoGN, including 22 with seronegative CryoGN. The median age was 61 years and 76% presented with nephritic syndrome. A hematologic condition was found in 89% of patients, including monoclonal gammopathy of renal significance (65%) and symptomatic lymphoproliferative disorder (35%). In the seropositive group, 56% had type II, 29% type I, and 8% type III cryoglobulin. Extrarenal manifestations, mostly of skin, were present in 64% and were significantly less common in seronegative CryoGN. Glomerular deposits by immunofluorescence were IgM dominant (84%) and polytypic (70%) in the seropositive group, whereas 52% of seronegative cases had monotypic deposits (i.e., type I cryoglobulin). Ultrastructurally, the deposits were organized in 77% of cases. Substructure appearance significantly differed according to the type of CryoGN, forming most commonly short cylindrical structures in type II and other organized substructures in type I CryoGN. Most patients were treated with clone-directed therapy. On follow up (median 33 months), 77% had partial or complete remission, 10% reached kidney failure and 14% died. Predictors of kidney failure on univariate analysis were AKIN stage 3, positive rheumatoid factor and biclonal gammopathy at diagnosis. We conclude that most CryoGN cases (types I and II) are due to a hematologic condition and are associated with favorable outcome after clone-directed therapy. Seronegative CryoGN accounts for about a quarter of cases and is mostly a kidney-limited disease. Thus, further investigations are needed to unravel the pathophysiology of seronegative CryoGN. © 2022 International Society of Nephrology |
| Keywords: | kidney biopsy; mgrs; cryoglobulinemic glomerulonephritis; mpgn; serum cryoglobulin |
| Journal Title: | Kidney International |
| Volume: | 102 |
| Issue: | 2 |
| ISSN: | 0085-2538 |
| Publisher: | Elsevier Inc. |
| Date Published: | 2022-08-01 |
| Start Page: | 382 |
| End Page: | 394 |
| Language: | English |
| DOI: | 10.1016/j.kint.2022.03.030 |
| PUBMED: | 35513122 |
| PROVIDER: | scopus |
| DOI/URL: | |
| Notes: | Article -- Export Date: 1 August 2022 -- Source: Scopus |
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