Not all cases of TBMN can be linked to mutations in COL4A3 and COL4A4. Research to better understand the spectrum of mutations that cause TBMN is ongoing. Extrarenal manifestations in…
ANTI-GBM DISEASE Anti-GBM disease occurs when autoantibodies directly target the glomerular basement membrane, causing inflammation that almost always leads to RPGN. Because anti-GBM disease (either isolated or as part of…
PROGNOSIS Outcomes in idiopathic disease are poor. More than 60% of patients with type I MPGN will progress to ESRD within 10 years of diagnosis. For DDD, the picture is…
On immunofluorescence, types I and III MPGN usually show prominent granular capillary wall staining with C3, variable amounts of IgG and IgM, and sometimes C1q and C4. In contrast, in…
The pathogenesis of type III MPGN appears similar to that of type I MPGN. Indeed, many consider type III MPGN to be a morphologic variant of type I, although the…
In adults, the prognosis is much worse, likely in large part due to the underlying comorbidities and chronic infections that predispose to PIGN. Only about half of patients will achieve…
Several different histologic findings may be present in PIGN, and the diagnosis can usually be made using light microscopy and immunofluorescence. On light microscopy, PIGN classically causes diffuse hypercellularity involving…
At normal body temperature, the concentration of dissolved carbon dioxide is equal to 3% of its partial pressure. By substitution, and slight further rearrangement: This formula, an expression of the…
The clinical features associated with worse outcomes include persistent proteinuria (>1 g/day), elevated serum creatinine concentration at diagnosis, and poorly controlled hypertension. The pathologic features associated with worse outcomes include…
Mild to moderate mesangial hypercellularity is typically seen using light microscopy. A subset of cases feature endocapillary hypercellularity, which can be segmental or global, focal or diffuse, and with or…
