Many challenges remain in diagnosing monoclonal immunoglobulin-associated renal disease, despite widespread application of immunofluorescence (IF) and immunohistochemistry. Here, we report a newly diagnosed case of multiple myeloma with clinical suspicion of renal amyloidosis, which had negative IF staining for kappa and lambda light chains in the glomeruli. Although laser microdissection and mass spectrometry-based proteomic analysis have emerged as important tools for amyloid typing in the literature, such facilities are still not widely available in Asia. We propose that a clinicopathological algorithm for the evaluation of organized monoclonal renal deposits, together with a combined nephrological-haematological approach, will still be adequate to generate an unequivocal diagnosis in the majority of cases.
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http://dx.doi.org/10.1159/000508785 | DOI Listing |
Glomerular Dis
October 2024
Division of Nephrology, Department of Internal Medicine, University of Nebraska Medical Center, Omaha, NE, USA.
Background: Complement 3 (C3) glomerulopathy (C3G) is a heterogenous disease characterized by dysregulation of the complement alternative pathway. Within 10 years of a diagnosis, roughly 50% of patients with C3G will progress to end-stage kidney disease. Historically, treatment options have been limited to nonspecific immune suppression with suboptimal response rates to recommended therapies.
View Article and Find Full Text PDFClin Exp Nephrol
November 2024
Department of Nephrology, Fujita Health University School of Medicine, Toyoake, Aichi, Japan.
Introduction: Clinical epidemiological data on monoclonal gammopathy of renal significance (MGRS) are lacking. In this retrospective observational study, MGRS was compared with B-cell or plasma cell malignancies (BCM/PCM) with renal involvement to clarify differences in their clinical features.
Methods: Among the 1408 renal biopsies performed at our hospital, 25 MGRS and 18 BCM/PCM patients were identified.
Nephrol Dial Transplant
November 2024
Department of Immunology, Assistance Publique- Hôpitaux de Paris (AP-HP), Georges Pompidou European Hospital, Paris, France.
Dysregulation of the alternative pathway of complement plays a central role in the pathophysiology of C3 Glomerulopathy (C3G). Various autoimmune and genetic factors targeting the alternative pathway have been associated to both C3G and primary Immunoglobulin-associated Membranoproliferative Glomerulonephritis (Ig-MPGN), suggesting shared pathophysiological mechanisms. This review highlights the wide range of disease drivers identified that mainly target components or protein complexes of the alternative pathway, both in C3G and Ig-MPGN.
View Article and Find Full Text PDFNephrol Dial Transplant
November 2024
Centre de Recherche des Cordeliers, Sorbonne Université, Inserm, Université Paris Cité, Inflammation, Complement and Cancer team, Paris, France.
C3 glomerulopathy (C3G), a prototype of complement mediated disease, is characterized by significant heterogeneity, not only in terms of clinical, histological and biological presentation but also of prognosis, and response to existing therapies. Recent advancements in understanding the factors responsible for alternative pathway dysregulation in the disease have highlighted its even more complex nature. Here, we propose a reexamination of the diversity of C3G presentations in light of the drivers of complement activation.
View Article and Find Full Text PDFBlood Res
August 2024
Division of Nephrology, Hyonam Kidney Laboratory, Soonchunhyang University Hospital, 59 Daesagwan-Ro, Yongsan-Gu, Seoul, South Korea.
Kidney disease is a frequent complication of multiple myeloma and other malignancies associated with monoclonal gammopathies. Additionally, dysproteinemia-related kidney disease can occur independently of overt multiple myeloma or hematologic malignancies. Monoclonal gammopathy of renal significance (MGRS) is a spectrum of disorders in which a monoclonal immunoglobulin produced by a benign or premalignant B-cell or plasma cell clone causes kidney damage.
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