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Systemic Lupus Erythematosus of the Kidney

Lupus of the kidney is associated with both nephrotic range proteinuria or non-nephrotic range proteinuria, and also hematuria with red blood cells and red blood cell casts. That’s called nephrotic/nephritic or nephritic/nephrotic. They have hypertension and decreased renal function because it’s a chronic progressive glomerulonephritis as well. The pathology ranges between nothing, normal pathology - either with already renal involvement manifested by maybe some proteinuria, not heavy proteinuria - up to the worst pathology, that is diffuse proliferative glomerulonephritis.

Going from the normal pathology, the next one is mesangial expansion and mesangial hypercellularity. Next is focal proliferative; that’s when the proliferation is in focal areas in the glomerulus. And diffuse; that is the worst kind of pathology.

The treatments are different, and I won’t go into details about treatment, but the findings in urine and blood are pretty self-evident. We’ve already mentioned them several times. In blood tests there will be the findings of lupus, anemia, leukopenia and thrombocytopenia with a positive VDRL, which is really a false positive. The most important findings, lab-wise, are the serological findings which are specifically anti double-stranded DNA antibodies and anti-Smith antibodies are the most specific.

In terms of treatment, again, there are different treatments to the different pathologies. The worst pathology, diffuse proliferative glomerulonephritis, requires high dose IV prednisone or Solu-Medrol and Cytoxan and lots of intense immune suppression.

In terms of prognosis, before we even had steroids, which none of us I think were in that era, there was a dismal prognosis with a five-year survival less than 10%. Nowadays, thankfully, the lupus patients don’t die of renal failure.

Alport’s has an association with deafness and the familial hematuria, leading to renal failure. And remember the HSP, which is what we see in kids, as another spectrum. The HSP, which is IgA in terms of pathology, but with a rash and the joint involvement. So those would be things that are more … that would be reasonable to be in the test.

Urinary Tract Infections. In the neonatal age, the predominance of males have UTI’s. That’s an important feature that then changes when they get older, to much much higher predominance in girls. But in the neonatal age, more boys than girls have UTI’s. For instance, the most common cause - no matter what age - is by far E. coli. So there is no hesitation about the answer to what’s the most common etiology. It doesn’t matter the age or the gender.

In terms of pathogenesis, the main thing is that in the neonatal period, the spread is hematogenous whereas anywhere after that in age, it is ascending through contamination. Ascending through the urethra. So that’s an important feature that neonates usually have urosepsis.

Next is in clinical presentation. The important thing is the presentation by age groups. In the neonate, UTI presents as any other sepsis. So it has to be thought of as a possible cause and urine has to be checked. As a general sepsis workup is done, urine culture is part of that workup and it mustn’t be forgotten, because that’s a very very important cause of sepsis. The findings, the clinical presentation is not necessarily any different than any other sepsis. They don’t complain of flank pain or abdominal pain. So that’s important to remember. In older infants, also, looks like any sepsis. It will be more associated with not wanting to eat so well. Starting to be FTT if it goes on for awhile. They might start crying on urination if there is a very perceptive parent, but again they don’t complain of the things we are used to asking about for UTI symptoms in all the kids; abdominal pain, flank pain, dysuria, frequency, etc. So the presentation in different age groups are different. The important thing in examination, history and examination, perinatal history such as oliguria, for instance. Indicating some problem with making of amniotic fluid, such as posterior urethral valves for instance, an anatomical abnormality. Or the finding of hydronephrosis on a prenatal ultrasound. Then voiding habits or voiding patterns. Boys, the moms can say, or the parents can say, that they have never seen a stream except for dribbling. That’s suspicious. Most mothers, or parents - sorry, nowadays fathers are just as involved - so most people get a spritz in their eye once in awhile. Some moms don’t know that there is a stream that goes straight up, and that indicates that there is not a good urine stream and that can lead to the suspicion of posterior urethral valves, especially.