Childhood-onset Sjögren’s can cause lasting damage to organs: Study
Damage seen by early adulthood, regardless of symptom severity
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Childhood-onset Sjögren’s disease can cause lasting damage to the body’s organs by early adulthood, regardless of how active the disease has been or how severely the symptoms have manifested.
Those are the findings of a single-center study in the U.K. that investigated the “long-term disease trajectories and complications of childhood-onset Sjögren’s.” The researchers had sought to determine the most effective management strategies for individuals with the chronic autoimmune disorder whose symptoms begin in childhood.
“A high proportion of children and young people develop damage in early adulthood,” the researchers wrote, noting that the study revealed “distinct patterns of disease” among patients.
“These findings highlight the need for improved research quality and evidence-based management strategies for better outcomes,” the team wrote.
The study, “Clinical phenotypes, classification, and long-term outcomes of childhood-onset Sjögren’s disease into adulthood: a single-centre cohort study,” was published in The Lancet Rheumatology.
In people with Sjögren’s, the immune system mistakenly attacks healthy tissues, primarily the glands that produce tears and saliva. This causes inflammation and symptoms such as dry eyes and a dry mouth.
Little info on how Sjögren’s develops when onset occurs in childhood
Because Sjögren’s usually affects adults, little is known about its course when the disease begins in childhood.
“The diagnosis and management of children and young people [with Sjögren’s] is based on expert opinion alone or informed by extrapolation of the management strategies available for adult Sjögren’s disease since good quality research in paediatric populations is scarce,” the researchers wrote.
To learn more, a research team in the U.K. set out to evaluate how childhood-onset Sjögren’s develops until adulthood. The team followed 30 pediatric patients — 28 girls and two boys — for a mean of 10 years and as long as 25 years. The participants had first shown symptoms at a mean age of 12.7 years.
Some patients were followed from the time of diagnosis, while others had their earlier medical records reviewed. All were treated at a specialist young adult rheumatology clinic in London.
“To our knowledge, this is the first study evaluating the natural course of childhood-onset Sjögren’s disease into adulthood,” the researchers wrote.
Initial symptoms were often nonspecific, and included fatigue, experienced by 73%, and joint pain, noted by 70%. Dry eyes or mouth affected 57%, with 50% experiencing swollen glands and 33% having skin rashes. As these symptoms can be mistaken for those of many other diseases, that may explain delays in diagnosis, the researchers noted.
A long diagnostic delay — more than three years — was linked to higher rates of dryness. All nine patients with such a diagnostic delay reported dryness, compared with about one-third (38%) of those diagnosed sooner.
Before their Sjögren’s diagnosis, seven children were diagnosed with the autoimmune disease systemic lupus erythematosus, the most common form of lupus, and two with juvenile idiopathic arthritis, another autoimmune condition.
The team identified two long-term patterns on the EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI), a validated measure of Sjögren’s activity. ESSDAI scores range from zero to 123, with higher scores indicating more active disease.
Some patients had high disease activity over time, with a mean ESSDAI score of 3.9, while others had low activity, with a mean score of 0.8.
Study uncovers 2 distinct symptom trajectories among patients
The ESSPRI — fully, the EULAR Sjögren’s Syndrome Patient-Reported Index — measures patient symptoms, with scores ranging from zero to 10. Higher scores on this index also indicate worse symptoms.
The study revealed two distinct trajectories during adolescence and young adulthood, when patients ranged in age from 13 to 36 years.
Some of these young patients experienced more severe dryness, fatigue, and pain, reflected by a mean ESSPRI score of 5.6. Others, meanwhile, reported milder symptoms, with scores averaging 3.1. These different patterns in ESSDAI and ESSPRI were not explained by sex, age at onset, disease duration, or length of follow-up, however.
At the last assessment, the median ESSDAI score was 2, indicating low disease activity, while the median ESSPRI score was 5.3, indicating the presence of substantial symptoms. The researchers noted that 17 individuals (57%) “already had damage associated with an objective decrease in saliva or tear secretion.”
Interestingly, the amount of long-term damage to the body’s organs — measured with Sjögren’s Syndrome Disease Damage Index (SSDDI), which records complications that cannot be reversed — did not differ between the high- and low-activity groups, the team noted.
This analysis … highlights an important unmet need for improved tools to facilitate early diagnosis … of childhood-onset Sjögren’s disease to enable adequate management.
Among the 17 patients who had accumulated damage at the last assessment, three (18%) had already lost teeth. According to the researchers, this “might suggest a lost window of opportunity to halt the autoimmune glandular insult with the current therapeutic approaches.”
Four patients (29%) had developed lymphoma, a type of blood cancer known to be associated with Sjögren’s, even at young ages.
Overall, these data show that childhood-onset Sjögren’s can cause long-term damage by early adulthood, according to the team.
“This analysis of the largest childhood-onset Sjögren’s disease [group] in the UK with long-term follow-up highlights an important unmet need for improved tools to facilitate early diagnosis and [clinical profile] characterisation of childhood-onset Sjögren’s disease to enable adequate management,” the researchers wrote. The team added that more study is needed “to ascertain whether the early damage accrual, high rate of lymphoma, and disease activity and symptom burden [profiles] observed can be widely validated.”
