Protecting lupus patients from organ damage
Isha struggled to fit her swollen feet into shoes. She was so physically weak that simply walking and talking was a major accomplishment, and most of her thick, wavy dark hair had fallen out.
After being diagnosed with lupus a year earlier at the age of 19, her new symptoms concerned her parents enough to take her straight to the hospital. She was quickly diagnosed with lupus nephritis, which had already caused significant damage to her kidneys, and she was started on an immunosuppressant.
“I had no idea what nephritis was. And I was shocked to find out that 50% of lupus patients have a chance of having organ involvement,” Isha says. “But nobody told me that.”
Her life changed overnight. She went from being a typical young adult and college student to fighting a daily battle of managing her new disease. She now had to be mindful of the ingredients and potential triggers in certain foods, she could no longer stand for long periods of time, and she had to be careful of exposure to illnesses in crowded places; something that Isha has had to worry about more recently.
COVID-19 and the disruption of routine medical care
As the COVID-19 pandemic disrupted the lives of millions around the world, people living with systemic lupus erythematosus (SLE)—the most common form of lupus, a chronic, incurable autoimmune disease in which the immune system attacks its own tissues—were no exception. The pandemic led to missed appointments and delayed treatments for people living with lupus, and healthcare providers reported more complications with the disease than before the pandemic.
According to a recent GSK global survey of 648 rheumatologists, nephrologists and internists, more than half of these physicians (57%) reported that some of their lupus patients did not schedule in-person visits due to fear of contracting COVID-19. Missed visits may have had significant consequences for these patients, with the survey also revealing responder reports that nearly a quarter (23%) of their patients experienced an increase in lupus flares or more severe symptoms during the pandemic. Lupus flares can nearly double the risk of organ damage.(i)
“Understandably, many people living with lupus put off in-person appointments out of fear of getting COVID-19,” explains Dr. Jaime Calvo Alén, Ph.D., a rheumatologist at Araba University Hospital in Vitoria-Gasteiz, Spain. “While more data will have to be gathered on the full impact of COVID-19, in my experience delaying care likely contributed to more flares or more uncontrolled symptoms, potentially leading to a higher risk of disease progression and organ damage that otherwise may have been avoided.”
According to the GSK survey, some healthcare providers are not starting the conversation about lupus-related organ damage risks until a year after diagnosis, or only when there are clinical signs of organ damage—which means irreversible organ damage may have already occurred. This report is supported by a 2022 World Lupus Federation survey of nearly 7,000 lupus patients in more than 100 countries, in which 56% of patients reported that their physician didn’t discuss the topic of organ damage at the time of their diagnosis.
Isha says that one of the biggest lessons she’s learned is to be more involved in her own healthcare and be an active receiver of any treatment.
“If you don’t understand a term or a concept, ask,” she says. “Be curious about any medication that you are taking. Why are you taking it? What happens? What does this medication do?”
Despite the prevalence of organ damage in lupus patients, communication gaps persist
Damage to the kidneys, heart, eyes and nervous system can be a major complication of lupus and carry significant quality-of-life and healthcare costs. However, lack of communication about organ damage makes it more difficult for providers and patients to work together to prevent damage before it starts. In addition, “some physicians may underestimate the risk to lupus patients who don’t have active symptoms,” says Dr. Calvo Alén, noting that “patients need to know that underlying inflammation with lupus can cause damage to internal organs, even without obvious symptoms, and because of this, some medications must be sustained regardless of having specific symptoms or not.”
According to the World Lupus Federation (WLF) survey of people living with lupus, 87% of patients reported one or more organs or organ systems affected by the disease, and more than half (53%) of these patients were hospitalized due to organ damage from lupus. Importantly, 42% of these patients had irreversible organ damage, including 17% of patients who reported irreversible damage to their kidneys. Inflammation of the kidneys, or lupus nephritis, affects half of all lupus patients within five years of diagnosis, but in many cases, the disease is treatable and potentially stabilized if caught early enough, says Dr. Calvo Alén.
Mike Donnelly, vice president of communications for the World Lupus Federation, underscores the need for early conversations. “Many providers don’t bring up the possibility of organ damage until it’s too late,” he says. “Even when a lupus patient does not ‘look sick,’ the disease may be damaging their organs, causing lasting complications and effects.”
Being proactive to address organ damage
Lupus remains somewhat of a mystery, and symptoms can vary widely from patient to patient. Further, many physicians in the GSK survey said that it is difficult to determine which patients are at the highest risk for developing organ damage, and this complicates treatment. Most physicians (72%) say that the current standard of care regimen, which includes anti-malarial medicines, steroids and immunosuppressants, can sufficiently reduce the risk of long-term organ damage for most lupus patients. However, research shows that this does not prevent organ damage in a significant number of patients; in fact, steroids may contribute to it.(ii),(iii),(iv),(v) In the WLF survey, some patients reported that their physician had never told them that steroids can cause organ damage.
The findings of the GSK survey suggest that more information about available treatments might help healthcare providers and patients balance the need for rapid symptom relief with long-term treatment goals. Most physicians (79%) in the study said that a lack of disease-modifying therapies—or therapies that can stop or slow disease progression—makes it difficult to treat lupus, although other research shows that symptoms of lupus can be managed by disease-modifying treatments, like biologics, that disrupt the source of the inflammatory process.(vi)
Because some healthcare providers may wait to discuss preventing organ damage, Dr. Calvo Alen suggests that people living with lupus take the lead and bring up the topic during appointments, and that the best time to discuss organ damage is when a patient is starting a new medication.
“My biggest advice is to take control of your own health,” Isha says. “You must be in charge of your own healthcare journey.”
Isha Vedantam is a real person living with lupus.
(i) Ugarte-Gil MF, Acevedo-Vasquez E, Alarcon GS, Pastor-Asurza CA, Alfaro-Lozano JL, Cucho-Venegas JM, et al. The number of flares patients experience impacts on damage accrual in systemic lupus erythematosus: data from a multiethnic Latin American cohort. Ann Rheum Dis 2015;74:1019-23.
(ii) Gladman DD, Urowitz MB, Rahman P, et al. Accrual of organ damage over time in patients with systemic lupus erythematosus. J Rheumatol 2003;30(9):1955-1959.
(iii) Sung YK, Hur NW, Sinskey JL, et al. Assessment of damage in Korean patients with systemic lupus erythematosus. J Rheumatol 2007;34(5):987-991.
(iv) Segura BT, Bernstein BS, McDonnell T, et al. Damage accrual and mortality over long-term follow-up in 300 patients with systemic lupus erythematosus in a multiethnic British cohort. Rheumatology (Oxford) 2020;59(3):524-533.
(v) Lopez R, Davidson JE, Beeby MD, et al. Lupus disease activity and the risk of subsequent organ damage and mortality in a large lupus cohort. Rheumatology (Oxford) 2012;51(3):491-498.
(vi) van Vollenhoven R, Askanase AD, Bomback AS, et al. Conceptual framework for defining disease modification in systemic lupus erythematosus: a call for formal criteria. Lupus Science & Medicine 2022;9:e000634. doi: 10.1136/lupus-2021-000634.