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Membranoproliferative Glomerulonephritis

The case that we will present it is that of a 10-year-old girl who wakes up in the morning with swollen eyes and who is pale. Examined by the pediatrician, was found to have a blood pressure that’s elevated, pretibial pitting, edema, bilateral decreased breath sounds - that we already know is pleural effusions - urinalysis with 4+ proteinuria and large blood, 50-100 RBC’s and blood tests with a creatinine that is already elevated for age and size, chronic renal failure, albumin is low, there is a nephrotic syndrome and the C-3 is very very depressed complement, and anemia. So this person could also have acute glomerulonephritis. Everything fits, however there will be the low complement that does not go back up to normal. It is chronically hypo-complementemia. And this person will need a biopsy and the biopsy shows dense deposits in the basement membrane. I’m not going to show you renal pathology. You are not going to have renal pathology, but if there is a description of a pathology, if you hear the words, "Double-contoured basement membrane" or "Dense deposits" that usually indicates membranoproliferative glomerulonephritis.

In terms of the findings in urine, other than what we already talked about, it’s urine that’s called nephritic/nephrotic because it’s got both; it can have red blood cell casts, red blood cells and proteinuria to a nephrotic range. Again, the most important thing in MPGN is the hypo-complementemia, which is persistent. I said already, if you hear these words described in some question, tram-track appearance, which is double-contoured basement membrane.

The natural history, unfortunately, is that of 50% going to end-stage within ten years and the rest, almost the rest, going into end-stage.

So it’s a progressive disease, leading to renal failure, end-stage renal disease and there is a specific known one treatment that actually treats it. We do use immune suppression but it’s not that we manage to delay or even prevent the progression to renal failure. So most of them do end up in end-stage on dialysis and then receive a transplant. There is a high incidence of recurrence of the disease in transplants.

IgA nephropathy. As I’ve mentioned already several times today, it’s the most common chronic glomerulonephritis and the typical picture will be that of a … there is no typical age, so 16-years-old is just an age I chose, but it is older children and young adults. But no age has been spared. There have been descriptions of babies with IgA nephropathy and old people with IgA nephropathy, but in our population it will be most likely an older child or young adult. Sixteen-year-old boy with a runny nose and a cough told his mother that when he went to the bathroom he saw that his urine was red. This was apparently ignored, and a few months later the boy was seen by his pediatrician because of a sore throat. During the exam he told the doctor that now he has red urine again. The blood pressure was 138/83, no edema, no rash or joint findings. The urine was tea-colored with too numerous to count red blood cells, and 3+ protein. On blood test, complements were normal, ANA was negative, creatinine 1, and BUN 25.

The biopsy; it is more prevalent in males, but not … some studies show 15:1, but really it’s not that high in most studies. The most important feature is the synpharyngitic hematuria; the finding of hematuria during the time of the upper respiratory infection.

The diagnosis is by finding of mesangial deposition of IgA in the biopsy. So the biopsy is the sine quo non of this disease. You cannot make it by serological testing or any other way, other than elimination of other things and definitively by a biopsy. There is no known treatment that we know will treat this, and there is a big study ongoing right now trying to determine which is better.

In terms of predictive features that predict a worse outcome, are males, older age at onset, heavy proteinuria at the time of diagnosis, hypertension, and obviously the worse the biopsy looks.

Systemic Lupus Erythematosus. In terms of renal manifestations, I already mentioned that again it is nephrotic/nephritic urine. Both nephrotic range proteinuria or non-nephrotic range proteinuria, but also hematuria with red blood cells and red blood cell casts.