IllnessGlomerulonephritis, membranoproliferative; differential diagnosis

NGS +

Membranoproliferative or mesangiocapillary glomerulonephritis (MPGN) was primarily a (light or electron) microscopic diagnosis, which is now supplemented by integrated information from clinic, serology and genetics. MPGN I is characterized by immune complexes with poly- or monoclonal IgG antibodies; MPGN II is complement-mediated glomerulonephritis. Type II is now referred to as C3 glomerulopathy (C3GP). The onset od C3GP is characterized by micro- or macroscopic hematuria, proteinuria and progressive renal function impairment. Renal function impairment is more common in adults and the elderly. These clinical manifestations are common to all three subtypes of C3GP ("dense deposits disease" DDD; C3 glomerulonephritis, CFHR5 glomerulopathy) based on aberrant control of the alternative complement pathway and sequential deposition of C3 in the glomeruli. DDD onset is characterized by either nephrotic or acute nephritic syndrome. Hypertension may be present at onset or later. In a few cases, DDD shows drusen at the fundus oculi, and some patients may have partial lipodystrophy as in MPGN. Diseases from this group are inherited either autosomal recessively or dominantly. The molecular genetic yield is little over 20% in these very rare conditions. A negative molecular genetic result does not constitute exclusion of the clinical diagnosis.

References: https://www.ncbi.nlm.nih.gov/books/NBK1425/

• Alias: Mesangiocapillary glomerulonephritis; MPGN
• Allelic: Basal laminar drusen (CFH)
• Allelic: C3 deficiency (C3)
• Allelic: Complement factor B deficiency (CFB)
• Allelic: Complement factor H deficiency (CFH)
• Allelic: Fish-eye disease (LCAT)
• Allelic: Macular degeneration, age-related, 14, reduced risk of (CFB)
• Allelic: Macular degeneration, age-related, 4 (CFH)
• Allelic: Macular degeneration, age-related, 9 (C3)
• Allelic: Macular degeneration, age-related, reduced risk of (CFHR1, CFHR2, CFHR3, CFHR4)
• Allelic: Macular degeneration, age-related, reduced risk of (CFHR3)
• Allelic: Thrombophilia due to thrombomodulin defect (THBD)
• C3 glomerulopathy (CFHR2, CFI)
• Complement factor D deficiency (CFD)
• Hemolytic uremic syndrome, atypical, susceptibility to (CFHR1, CFHR2, CFHR3, CFHR4)
• Hemolytic uremic syndrome, atypical, susceptibility to, 1 (CFH)
• Hemolytic uremic syndrome, atypical, susceptibility to, 2 (CD46)
• Hemolytic uremic syndrome, atypical, susceptibility to, 3 (CFI)
• Hemolytic uremic syndrome, atypical, susceptibility to, 4 (CFB)
• Hemolytic uremic syndrome, atypical, susceptibility to, 5 (C3)
• Hemolytic uremic syndrome, atypical, susceptibility to, 6 (THBD)
• Hemolytic uremic syndrome, atypical, susceptibility to, 7 (DGKE)
• Immune-complex-mediated MPGN (CFHR2, CFI)
• Methylmalonic aciduria and homocystinuria, cblC type (MMACHC)
• Nephropathy due to CFHR5 deficiency (CFHR5)
• Nephrotic syndrome, type 7 (DGKE)
• Norum disease (LCAT)
• Thrombotic thrombocytopenic purpura, hereditary (ADAMTS13)