The Intersection of Lupus and End-Stage Renal Disease

It’s a question that I have considered from time to time in my nursing career—in a patient with severe systemic lupus erythematosus (SLE) who also develops end-stage renal disease (ESRD), can the patient’s SLE ever go into remission? As with many clinical questions, experience gave me my answer.

In 2009, I met LW, a 37-year-old patient who had been diagnosed with SLE about a decade ago. Upon his initial diagnosis, he had a positive anti-ribosomal P titer (165 U; normal 1-20, borderline between 20 and 25), mildly elevated anti-chromatin antibodies (119 U; normal 0-99), and a mildly elevated serum creatinine level (1.58 mg/dl, normal 0-1.3). A renal biopsy showed grade V glomerulonephritis, which unfortunately is indicative of near-certain future ESRD requiring eventual dialysis and likely renal transplant.1

LW was started on daily prednisone 60 mg, which helped control his SLE symptoms, along with furosemide 40 mg, potassium chloride 20 mEq, daily isosorbide mononitrate ER 30 mg, daily labetalol hydrochloride 200 mg QD, daily lisinopril 20 mg, and daily mycophenolate mofetil 1 g.
Not long after his diagnosis, LW’s begin spilling 3-4 g of protein in urine each day, which led to him being placed on cyclophosphamide. He received 7 doses over the subsequent 10 months, which unfortunately was ineffective in alleviating his lupus-related symptoms. Even more significantly, he soon developed lupus cerebritis, possibly caused by the prolonged use of prednisone and uremia (his serum creatinine was now up to 6.82 mg/dL).

LW was hospitalized on several occasions with many of the more common symptoms associated with lupus cerebritis, including polyneuropathy, severe headaches, hallucinations, psychosis, and seizures.2 He was treated with additional prednisone and cyclophosphamide, which led to the common push/pull we often see with our patients. His cerebritis-related symptoms became better controlled, but LW suffered numerous side effects of prednisone, including weight gain, agitation, and large swings in his blood sugar levels.

LW would come into our office wearing beach sandals, the only footwear that would accommodate his grossly swollen and cumbersome feet. Trained as an engineer, LW was totally disabled when he first started coming to our office, barely surviving between frequent hospitalizations.

There were, not surprisingly, good and bad days. LW began to suffer from colitis, again possibly due to the prednisone but also potentially due to his SLE. Thankfully, he did not develop a pleural or pericardial effusion.

Due to his renal disease and loss of erythropoietin, LW’s hemoglobin was perpetually low, regularly hovering around 7.7 g/dL, and he had a hematocrit of 23.5%. After many months of coaxing, LW finally agreed to see a nephrologist, who added epoetin alfa to his medication regimen in an effort to stabilize his blood counts. This led to resolution of his proteinuria as his renal output was reduced to <400 cc over a 24-hour span, which is considered oliguric renal failure.3 In addition, his serum creatinine spiked to 13 mg/dL.

We felt that the root cause of many of LW’s issues was renal failure and so, during visits when he seemed most mentally alert, we suggested to him that dialysis would likely have a positive impact on many of his symptoms. Knowing how difficult it would be for him to accept being dependent on dialysis for the rest of his life, I emphasized the very real danger of succumbing to his medical issues if they were not addressed soon.

Perhaps because of his engineering background and the science behind the technology, LW finally agreed to consider ambulatory peritoneal dialysis. I made sure that he understood that this would require daily treatment, but that, if all went well, he might be able to return to work and be in better control of his disease.

“While the reasons for their improvements are not well understood, I am always grateful when we can help patients get their lives back.”

A few weeks later, LW came into our office wearing a dress shirt and tie. He was beaming as he told us, “I am working again!” While his lab results were still concerning—his serum creatinine was down, albeit slightly, to 9.0 mg/dL—his enthusiasm was something we had never before seen. While LW admitted needing to nap at lunch everyday due to fatigue, that was still a vast improvement from monthly hospitalizations, seizures, and psychoses.

So is LW’s SLE truly gone? The medical literature shows it is not uncommon for patients on extended dialysis to see their lupus symptoms improve significantly. One recent review found that the percentage of lupus patients with clinical activity after the initiation of dialysis decreased to 55% after 1 year of dialysis, to 6.5% after 5 years, and to 0% after 10 years. There was a corresponding decrease in serologic activity and disease activity scores. There is even a term—lupus burn-out—to describe this phenomenon.4 The underlying mechanisms behind these remarkable improvements in SLE-related symptoms is not entirely clear.

Of course, providers should note the treatment of ESRD does not always result in complete or even partial resolution of lupus-related manifestations as seen with LW. I have, however, had a second patient with a similar story after a renal transplant.

While the reasons for their improvements are not well understood, I am always grateful when we can help patients like LW get their lives back. Patients with severe lupus often suffer on the precipice of health-related calamities, and our options for treatment, while improving, still offer only moderate hope for some of our patients. All we can do is try our best for our patients and to maintain our optimism on the darkest of days with the hope that something will work as far down the line as our patients need to travel.

AUTHOR PROFILE:
Jacqueline Fritz, RN, MSN, CNS, RN-BC, is Owner and Coordinator of Education at the Medical Advancement Center in Cypress, CA. Her primary responsibility is working as an advanced practice nurse for a large rheumatology practice where she is involved in patient visits, research programs, and infusion center coordination. In addition, she enjoys speaking, teaching, and learning about immunology.

 

References

1. Bhinder S, Singh A, Majithia V. Membranous (class V) renal disease in systemic lupus erythematosus may be more common than previously reported: results of a 6-year retrospective analysis. Am J Med Sci. 2010;339(3):230-2.

2.Magro-Checa C, Zirkzee EJ, Huizinga TW, Steup-Beekman GM. Management of neuropsychiatric systemic lupus erythematosus: current approaches and future perspectives. Drugs. 2016;76:459-483.

3.Klahr S, Miller SB. Acute oliguria. N Engl J Med. 1998;338(10):671-5.

4.Lionaki S, Skalioti C, Boletis JN. Kidney transplantation in patients with systemic lupus erythematosus. World J Transplant. 2014;4(3):176-82.