Lupus Nephritis May Be ‘Very Delayed’ After Initial SLE Diagnosis, Case Studies Show

While lupus nephritis usually develops within five years of a systemic lupus erythematosus (SLE) diagnosis, a case study of three patients shows that the condition may rarely appear 15 years or more after diagnosis.

In the report, researchers call it “very delayed lupus nephritis,” pointing out it is to be distinguished from late-onset lupus nephritis, which occurs only in women older than 50. In all three cases examined in “Very delayed lupus nephritis: a report of three cases and literature review,” the women developed lupus nephritis before they were 50 years old.

Lupus nephritis (LN) is a frequent condition arising from lupus that develops in nearly half of patients. But while the disease develops within five years in the vast majority of patients, 5 to 15 percent may develop it subsequently. To date, the longest interval reported between an SLE and a LN diagnosis is 34 years.

Researchers at Hospital da Luz, in Portugal, described the cases of three patients who developed lupus nephritis 15 years or more after their SLE diagnosis – the only three patients in their group of almost 700 patients seen over 40 years. Their findings were published in the journal Lupus Science & Medicine.

The first patient was an African woman diagnosed with lupus at age 28, and with lupus nephritis 19 years later, at age 47. The second case was of an Indian woman who developed lupus nephritis at 32 years of age, 17 years after her lupus declared itself at age 15. Finally, the third case was an Asian woman diagnosed with lupus at age 32, and whose lupus nephritis was confirmed at age 47, 15 years later.

All patients had multiple SLE flares during the course of disease before LN onset. Hydroxychloroquine, known to prevent kidney disease and SLE flares, was prescribed to the three patients for most of their disease course. Rituxan (rituximab) successfully treated two patients long before they showed any signs of lupus nephritis.

Lupus nephritis was detected by the presence of microscopic blood in urine and/or high levels of proteins in urine, suggesting kidney abnormalities. For all three patients, the condition was identified after an arthritis flare.

All patients received mycophenolate mofetil and steroids for their lupus nephritis, but two needed Rituxan as an alternative treatment. Two patients were only followed for 2-4 months after treatment, and their disease course and treatment outcomes are still unknown.

The third case was followed for five years. She had a lupus nephritis flare that was treated with mycophenolate mofetil, but went on to develop chronic kidney disease with only partial remission.

Researchers point that very delayed lupus nephritis is a rare condition, but believe that more cases might emerge in the near future as a consequence of increased life expectancy.

“With the increasing life expectancy of patients with SLE, it is likely that more cases of DLN [delayed lupus nephritis] will emerge… Renal biopsy to search for silent LN, or SLE therapy optimization to prevent renal damage might be reasonable management options,” the researchers concluded.