IgM Variant of Proliferative Glomerulonephritis With Monoclonal Immunoglobulin Deposits: A Case Series
Notice bibliographique
Résumé
RATIONALE & OBJECTIVE: Most deposits in proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) are of the IgG class. The IgM variant (PGNMID-IgM) is very rare, and data are mostly derived from single case reports or cases associated with B-cell lymphoproliferative disorders. We describe the clinicopathologic characteristics and outcomes among cases of PGNMID-IgM. STUDY DESIGN: Case series. SETTING & PARTICIPANTS: 23 PGNMID-IgM cases identified from kidney pathology archives. PGNMID-IgM was defined by glomerulonephritis with glomerular granular monotypic IgM deposits after excluding cryoglobulinemic glomerulonephritis and intracapillary monoclonal deposits disease. FINDINGS: Seventy-eight percent of the cases were male, they had a median age of 72 years, and they had presented with proteinuria (median, 3.1g/day), hematuria (91%), and reduced estimated glomerular filtration rate (median serum creatinine, 1.9mg/dL). Hypocomplementemia was present in 31% of cases. The underlying hematologic condition for all cases was monoclonal gammopathy of renal significance (MGRS). Serum protein electrophoresis/serum immunofixation (SPEP/SIF) detected the nephropathic monoclonal immunoglobulin (MIg) in 27% of cases whereas matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF) detected the nephropathic IgM in 4 of 7 tested patients. Kidney biopsy revealed membranoproliferative glomerulonephritis (83%) with nonorganized glomerular monotypic IgM (100%) and C3 deposition (96%), but C1q deposition was rare. Seventeen percent received symptomatic treatment alone, 17% received steroids alone, and 65% received other immunosuppressive therapy (mostly rituximab-based therapy). Follow-up (median, 40 months) was available for all patients. The median kidney and patient survivals were 44 and 158 months, respectively. Three patients underwent kidney transplantation, and all had recurrence, in 2 cases within a month. LIMITATIONS: Small sample size, retrospective design, nonstandardized clinical management. CONCLUSIONS: PGNMID-IgM mostly affects elderly men and is associated with MGRS with a low detection rate of the circulating nephropathic MIg on SPEP/SIF, which may be improved by MALDI-TOF. Kidney survival is guarded, with a high rate of early recurrence after transplant although overall survival is favorable. The pathogenesis remains unknown, but it likely involves local activation of alternative or lectin pathways of complement system by the monotypic IgM. PLAIN-LANGUAGE SUMMARY: The IgM variant of proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID-IgM) is very rare, and data on its clinicopathologic and outcome characteristics are scanty. This study of 23 patients with PGNMID-IgM identified in the Mayo Clinic pathology archives revealed that most patients were elderly White males who presented with proteinuria, hematuria, and reduced kidney function. The underlying hematologic condition was monoclonal gammopathy of renal significance in all cases, and the detection rate of the circulating nephropathic monoclonal immunoglobulin with serum protein electrophoresis/serum immunofixation was low but was increased using matrix-assisted laser desorption/ionization time-of-flight mass spectrometry. Pathologically, all cases exhibited nonorganized glomerular monotypic IgM, most with a membranoproliferative glomerulonephritis pattern. Outcome analysis revealed a guarded kidney survival (median, 44 months), a high rate of early recurrence after transplant, and favorable patient survival (158 months).
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Comment cette classification a été obtenuedéplier
Prédiction machine sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Le volet Gemma est une étiquette directe du modèle pour chaque travail de la base, lue sur la notice réduite au titre. Le volet Codex est un classifieur appris des 10 348 étiquettes directes de Codex et calibré sur les taux pondérés de l'échantillon; les champs sans appui suffisant ne portent aucun appel Codex. Le mode candidate est l'union des deux volets; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont pas des étiquettes humaines.
Scores du classifieur distillé par catégorie (deux têtes)
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,001 | 0,003 |
| Méta-épidémiologie (sens strict) | 0,002 | 0,001 |
| Méta-épidémiologie (sens large) | 0,001 | 0,001 |
| Bibliométrie | 0,003 | 0,002 |
| Études des sciences et des technologies | 0,002 | 0,002 |
| Communication savante | 0,002 | 0,002 |
| Science ouverte | 0,002 | 0,002 |
| Intégrité de la recherche | 0,003 | 0,001 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,002 | 0,001 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule source (Gemma direct ou Codex distillé), pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».