People with childhood-onset systemic lupus erythematosus (cSLE) develop significant disease-associated damage early in life and have lower quality of life in their adulthood, without achieving medication-free remission, according to a recent Dutch study.
The study, “Long‐term clinical outcomes in a cohort of adults with childhood‐onset Systemic Lupus Erythematosus,” was published in the journal Arthritis and Rheumatology.
SLE is an inflammatory autoimmune condition that can develop in both children and adults, and it mainly affects women and non-white people. cSLE, accounting for 10-20% of all SLE cases, is defined by symptom onset before 18 years of age and a mean age of 11-12 years at onset.
People with cSLE usually have more severe clinical course than those with adult-onset SLE, with higher disease activity and fast accumulation of disease-associated damage. Children with lupus may develop kidney and or brain disease early and have increased physical disability, as well as reduced quality of life.
However, long-term follow-up data of cSLE patients is limited, and information regarding disease damage over time and patients’ quality of life in adulthood is lacking.
Researchers in the Netherlands investigated the medical data and health-related quality of life (HRQOL) of adults with cSLE. This was part of a study called Childhood-onset SLE in the Netherlands (CHILL -NL), which aims to assess the burden of cSLE in the Netherlands.
They recruited 111 cSLE adult patients (101 women and 10 men) from November 2013 to April 2016. Eighty patients (72%) were white, and patients had the disease for a median of 20 years.
A physical examination was conducted to all patients at a single study visit, and patients’ mean age at the visit was 33 years. Disease activity was assessed through the SLE Disease Activity Index 2000 (SLEDAI-2K), accumulated disease-associated damage through the SLE International Collaborating Clinics (SLICC)/American College of Rheumatology Damage Index (SDI), and HRQOL using the 36-Item Short Form Health Survey (SF-36).
Twenty years after diagnosis, most patients had low disease activity at study visit, but 71% of them still used prednisone, hydroxychloroquine and/or other disease-modifying anti-rheumatic drugs (DMARDs).
“Clearly drug-free remission remains difficult to achieve, and current DMARDs are not effective enough to be used without corticosteroids [such as prednisone] in many patients,” the researchers wrote.
According to the patients, these medications had a negative impact on their physical appearance (89%), physical health (36%), or mental health (28%). Patients associated the use of prednisone with a greater impact in their physical appearance (such as weight gain) and mental health (such as mood swings), and DMARDs other than hydroxychloroquine as having the greater impact on their physical health (such as nausea).
cSLE-related symptoms — mainly in the skin, joints, blood, and kidney — developed mostly within two years after diagnosis, and disease-associated damage (such as stroke and heart attack) five years post-diagnosis.
More than half of the patients (62%) had disease-associated damage, mainly in the musculoskeletal system, central nervous system (brain and spinal cord) and kidneys. Major medical complications — including kidney transplant, stroke, heart attack, and joint replacement — occurred when the patients were in their early 20s and 30s.
The team found that disease duration was the main variable associated with the development of damage in these patients.
They noted that these findings suggest that most adults with cSLE develop significant disease-associated damage at a young age and that preventive screenings to control disease activity and heart involvement before the age of 30 should be conducted to improve the prediction of disease course.
The cSLE patients’ quality of life was impaired in most domains, compared to the general Dutch population, and was found to be significantly affected not only by disease activity, but also by factors affecting physical appearance. The researchers believe that the identification and management of these other factors may potentially improve the quality of life for these patients.
The team concluded that this data highlights the great impact of cSLE in the future life of patients and the need to follow these patients throughout their life to better understand their disease course and what can be done to improve their quality of life.