Membranoproliferative glomerulonephritis

Abstract

Membranoproliferative glomerulonephritis is an uncommon kidney disorder characterized by mesangial cell proliferation and structural changes in glomerular capillary walls. It can be subdivided into idiopathic and secondary forms, which are differentially diagnosed by a review of clinical features, laboratory data, and renal histopathology. Three types—I, II, and III—have been defined by pathologic features. All three types are associated with hypocomplementemia, but they manifest somewhat different mechanisms of complement activation. Type II, also known as “dense deposit disease”, is associated with the presence of C3-nephritic factor. Membranoproliferative glomerulonephritis primarily affects children and young adults, with patients presenting with nephrotic or nephritic syndrome or with asymptomatic renal disease. This type of glomerulonephritis often progresses slowly to end-stage renal disease, and it tends to recur after renal transplantation, especially type II. The efficacy of various forms of treatment remains controversial; however, long-term steroid treatment seems to be effective in children with nephrotic-range proteinuria. Improvement in renal outcomes largely relies on the evaluation of more selective agents in carefully controlled studies.

Eculizumab in Pediatric Dense Deposit Disease

“…To our knowledge, the effect of eculizumab on leukocyturia in DDD GN has previously not been described. Although leukocyturia is not commonly reported as a presenting symptom in DDD (8,9,28), it was a consistent finding in all patients with urinary leukocyte counts available. In the absence of other causes of leukocyturia, the fact that leukocyturia disappeared almost completely and within 1 week after the first dose in all episodes of GN with leukocyturia strongly indicates a direct effect of eculizumab.…”

“…However, to date, no evidence exists that any of these treatments indeed influences the course of the disease (3,7,8) and, consequently, the prognosis remains dismal (9,10). In recent years, attention has shifted to eculizumab, a humanized monoclonal antibody that binds complement factor 5 (C5) with high affinity and thus prevents generation of the terminal complement complex C5bC9.…”

Original paper Renal interstitial mast cell count is significantly higher in membranoproliferative glomerulonephritis than in class IV lupus nephritis

“…Membranoproliferative glomerulonephritis (MPGN) is a rare renal disease characterized by diffuse mesangial cell proliferation, along with structural changes in glomerular capillary walls, specifically subendothelial and subepithelial deposits [3,4]. This pattern is similar to that seen in lupus nephritis ISN/RPS class IV.…”

Treatment of Steroid-Resistant Nephrotic Syndrome

“…It typically presents with haema turia (macroscopic or microscopic), non-selective proteinuria which is usually, but not always in the nephrotic range, and frequently hypertension and deteriorating renal function. Hypocomplementemia (persistently reduced plasma concentration of C3) is commonly seen associated with MPG [52] Depositions of immunocomplexes can be observed in Immune complex-mediated MPGN. Frequent infections that result in this type are Hepatitis C and B.…”