Objectives: With the in-depth study of complement dysregulation, glomerulonephritis with dominant C3 has received increasing attention, with a variety of pathologic types and large differences in symptoms and prognosis between pathologic types. This study analyzes the clinical, pathological, and prognostic characteristics of different pathological types of glomerulonephritis with dominant C3, aiming to avoid misdiagnosis and missed diagnoses.
Methods: The clinical, pathological, and follow-up data of 52 patients diagnosed as glomerulonephritis with dominant C3 by renal biopsy from June 2013 to October 2022 were retrospectively analyzed. According to the clinical feature and results of pathology, 15 patients with post-infectious glomerulonephritis (PIGN) and 37 patients with of non-infectious glomerulonephritis (N-PIGN) were classified. N-PIGN subgroup analysis was performed, and 16 patients were assigned into a C3-alone-deposition group and 21 in a C3-dominant-deposition group, or 27 in a C3 glomerulopathy (C3G) group and 10 in a non-C3 nephropathy (N-C3G) group.
Results: The PIGN group had lower creatinine values (84.60 μmol/L vs179.62 μmol/L, =0.001), lower complement C3 values (0.36 g/L vs0.74 g/L, <0.001) at biopsy, and less severe pathological chronic lesions compared with the N-PIGN group. In the N-PIGN subgroup analysis, the C3-dominant-deposition group had higher creatinine values (235.30 μmol/L vs106.70 μmol/L, =0.004) and higher 24-hour urine protein values (4 025.62 mg vs1 981.11 mg, =0.037) than the C3-alone-deposition group. The prognosis of kidney in the PIGN group (=0.049), the C3-alone-deposition group (=0.017), and the C3G group (=0.018) was better than that in the N-PIGN group, the C3-dominant-deposition group, and the N-C3G group, respectively.
Conclusions: Glomerulonephritis with dominant C3 covers a variety of pathological types, and PIGN needs to be excluded before diagnosing C3G because of considerable overlap with atypical PIGN and C3G; in addition, the deposition of C1q complement under fluorescence microscope may indicate poor renal prognosis, and relevant diagnosis, treatment, and follow-up should be strengthened.
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http://dx.doi.org/10.11817/j.issn.1672-7347.2024.230328 | DOI Listing |
Diagnostics (Basel)
December 2024
Department of Internal Medicine and Rheumatology, "Dr. Ion Cantacuzino" Clinical Hospital, 011437 Bucharest, Romania.
Coatomer subunit α (COPA) syndrome is a mendelian autosomal dominant immune dysregulation disease characterized by early onset lung disease in the form of diffuse alveolar hemorrhaging or interstitial lung disease, frequently associated with arthritis, glomerulonephritis, and high titer autoantibodies usually mimicking other autoimmune diseases. While immunosuppressive medication has been effective in controlling arthritis, data on long-term lung disease control remains scarce, which poses a real challenge as the progression of lung disease is the main cause of poor life expectancy in COPA patients. Nevertheless, JAK inhibitor therapy seems to be the most promising therapeutic choice now.
View Article and Find Full Text PDFFront Immunol
January 2025
Department of Rheumatology, University of Texas Southwestern Medical Center, Dallas, TX, United States.
Background: Both intrinsic renal cells and immune cells contribute to driving renal inflammation and damage. However, the respective roles of intrinsic renal cells and immune cells in crescentic glomerulonephritis, and the key molecular factors driving pathogenesis are still unclear.
Methods: The roles of intrinsic renal cells and renal infiltrating immune cells in crescent formation were explored using renal transplantation after experimental anti-GBM disease induction in 129x1/svJ and C57BL/6J mice.
Diagnostics (Basel)
November 2024
Division of Nephrology, Hospital das Clínicas, University of São Paulo School of Medicine, São Paulo 05403-010, SP, Brazil.
Background: Since the introduction of the SLICC criteria in 2012, biopsy-proven lupus nephritis (LN) has been the only independent diagnostic criterion for systemic lupus erythematosus (SLE). This was reaffirmed by the EULAR/ACR in 2019, emphasizing the importance of renal biopsy in LN. However, the current classification lacks specific histopathological criteria for defining LN.
View Article and Find Full Text PDFKidney Res Clin Pract
November 2024
Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Republic of Korea.
Background: The clinical significance and renal outcomes of C1q nephropathy (C1qN) are unclear; therefore, the implications of C1qN as a new pathological entity are uncertain. We compared the clinical characteristics of glomerulonephritis reclassified into cases that meet the definition of C1qN and glomerulonephritis not included in the definition of C1qN.
Methods: In total, 21,697 patients who underwent native kidney biopsy at 18 hospitals throughout Korea between 1979 and 2018 were retrospectively enrolled.
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