From the Journals
Macrophage activation syndrome’s impact in childhood SLE felt mostly early
MAS remains a concern for children with SLE, but many outcomes were not significantly different from those without the complication.
The majority of adults with childhood-onset systemic lupus erythematosus in a longitudinal Dutch study developed significant damage at a young age, remain on corticosteroids in adulthood, and have an impaired health-related quality of life.
Courtesy Wikimedia Commons/doktorinternet/Creative Commons License
The findings in the study, dubbed Childhood-Onset SLE in the Netherlands (CHILL-NL), highlighted the need for preventive screening measures to be put in place before the age of 30 to facilitate a better outcome for patients, who still face high morbidity from childhood-onset systemic lupus erythematosus (cSLE) despite improved survival. Such information is helpful to “answer questions from children and parents regarding the future course of the disease,” wrote Noortje Groot, PhD, of the department of pediatric rheumatology at Erasmus Medical Center–Sophia Children’s Hospital in Rotterdam, the Netherlands, and her colleagues. The report is in Arthritis & Rheumatology.
The current study included all adult SLE patients treated in any Dutch public hospital during the period of November 2013 to April 2016 who were diagnosed with the autoimmune disease prior to their 18th birthday.
All 111 patients involved in the study were seen for a 1.5-hour visit at Erasmus University Medical Center or a local hospital of their choice. During the appointment, a medical history was taken, a physical examination was performed, and the patients completed questionnaires on health-related quality of life (HRQOL).
The average age of patients at the study visit was 33 years, 91% were female, and 72% were white. Median disease duration was 20 years and disease activity was low (median SLE Disease Activity Index 2000 [SLEDAI-2k] = 4). Low complement (32%), skin rashes (14%), and proteinuria (13%) were the most common SLEDAI items reported.
Overall, 68% of the cohort (n = 76) were taking hydroxychloroquine (HCQ), 29% of whom (n = 22) were taking it as monotherapy.
Furthermore, 68% of patients at the study visit were taking corticosteroids and/or non-HCQ disease-modifying antirheumatic drugs (DMARDs), and just over half (51%) of patients (n = 56) were taking corticosteroids either alone or with a non-HCQ DMARD.
“This [finding] is worrying as corticosteroids are associated with the development of damage. Patients are certainly eager to limit corticosteroid use, as almost all patients in the CHILL-NL cohort reported to have negative experiences with prednisone regarding their physical appearance and or mental well-being,” the study authors wrote.
Results also showed that 62% of the patients had damage, predominantly in the musculoskeletal, neuropsychiatric, and renal systems.
Most organ systems became involved within the first 2 years of diagnosis, but after 5 years of disease the nature of disease manifestations tended to shift to damage such as myocardial infarctions.
Notably, after 10-20 years, when cSLE patients were in their early 20s and 30s, significant damage had occurred in more than half of the patients, the authors noted.
“This shift to damage has also been observed in adult-onset SLE patients and urges for preventative screening measures of such (cardiovascular) damage and healthy lifestyle advice (healthy diet, regular exercise, abstinence from smoking),” they wrote.
Multivariate logistic regression showed that damage accrual was associated with disease duration (odds ratio, 1.15; P less than .001), antiphospholipid‐antibody positivity (OR, 3.56; P = .026), and hypertension (OR, 3.21; P = .043). On the other hand, current HCQ monotherapy was associated with an SLICC-Damage Index score of 0 (OR, 0.16; P = .009).
The HRQOL of the cohort, assessed via the Short Form–36, was also impaired compared with the general population, the researchers discovered. For example, the presence of damage reduced HRQOL in one domain, and high disease activity, defined as SLEDAI-2k of 8 or more, strongly reduced HRQOL in four of eight domains. Changes in physical appearance lowered HRQOL in seven of eight domains.
“HRQOL of adults with cSLE is impaired and affected by other factors than disease activity or damage alone. By identifying and addressing these factors, like physical appearance and potentially coping styles, HRQOL may be improved,” they advised.
The study was supported financially by the Dutch Arthritis Foundation and the Dutch national patient association for lupus, antiphospholipid syndrome, scleroderma, and mixed connective tissue diseases.
SOURCE: Groot N et al. Arthritis Rheumatol. 2018 Aug 27. doi: 10.1002/art.40697