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Disease Progression of LN from SLE


Jorge Larranaga, MD, and Alvin Wells, MD, PhD, describe the progression of SLE to lupus nephritis.

Alvin Wells, MD, PhD: Lupus can affect many aspects of the body, including the scalp, skin, kidneys, heart, and lungs. Some patients have kidney involvement at the time of diagnosis of lupus. One way we tell is by doing a blood test to look for elevation of the creatinine, and then we do a urine test to look for protein in the urine, or proteinuria. The data show that of every 3 patients with lupus who present for the first time to a rheumatology clinic, 1 will have lupus nephritis. That triggers us to start aggressive therapy so that we don’t let that patient progress to end-stage renal disease, which essentially means dialysis. Unfortunately, if patients have lupus nephritis, they’re 45 times more likely to get kidney disease than a patient without lupus nephritis. That’s a bad sign.

Jorge Larranaga, MD: With respect to the progressive nature of lupus nephritis over time, the disease tends to progress to transplant and dialysis. Depending on what you read, between 45% and 55% of patients with SLE [systemic lupus erythematosus] develop lupus nephritis. There are multiple histologic phases of lupus nephritis. Some remain stagnant, and others proceed in aggressive fashion to end-stage renal disease. Seventy percent of patients with lupus nephritis have stage III, IV, or V [disease], which tend to be the most aggressive [stages] with respect to the early intervention and management of the disease state. It can fluctuate. Many years in the past, we probably weren’t as aggressive with stage III. But now we recognize that the fluctuations and aggressiveness of the insult of the autoimmune disease can lead to significant deposition and rapid progression to the end stage, causing renal demise and ultimately patient survival and death.

With respect to a renal flare, we recognize that a single episode of lupus nephritis can lead to irreversible and permanent damage of nephron loss. Subsequent renal insults lead to end-stage nephron survival and the need of renal replacement therapy. This has shown that lupus flare-ups and nephritis worsen the outcome of the patient’s end-stage renal disease and increase the patient’s morbidity and mortality. Ultimately, patient survival is decreased and death ensues.

We have to be vigilant to the end-organ target damage, which tends to be unpredictable with autoimmune disease. Potential nephron and human demise is what we’re dealing with. That’s where the urgency in the management and aggressive interventions of this disease takes place. Unfortunately, the screening surveillance has statistically shown to be quite poor—in particular the lack of academic guidance, socioeconomic status of the patients, and compliance at a very young age. The unpredictable behavior going from subtle to aggressive deterioration is all based on the histologic stages of this disease.

With respect to the renal flare-ups, in addition to the above, we combine patient clinical presentations and findings, laboratory disease serology, urinary sediments, and renal markers to help assist in recognizing the flare-ups of these patients. In particular, hematuria, dysmorphic RBCs [red blood cells], CAS formations, proteinuria, urine PCR [protein-creatinine ratio], and serum creatinine all get taken into [consideration] in sequential fashion, along with the patient’s clinical findings and complaints and the serology activity of lupus nephritis. Depending on the degree of nephritis or nephrosis components, the serum creatinine behavior can predict the histologic stage, along with proteinuria and RBCs recognizing the potential of all those high-risk patients, which will lead us to the guidance of a renal biopsy, of which indications have changed over the years. With a renal biopsy, histologic tissue is of major importance in the diagnosis, prognosis, and therapeutic intervention.

Transcript edited for clarity.

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