Managing paediatric rheumatologic conditions in primary care

Paediatric (childhood-onset) rheumatologic conditions may be a significant driver of disability in children that might progress into adulthood. Audrey Abella speaks with Associate Professor Thaschawee Arkachaisri, Head and Senior Consultant, Rheumatology and Immunology Service, KK Women’s and Children’s Hospital in Singapore, to discuss how such conditions can be managed in primary care.

 

Paediatric rheumatologic conditions involve chronic, noninfectious inflammatory processes affecting structures of the musculoskeletal system, blood vessels, and/or associated organs/tissues secondary to dysregulation of the immune system (adaptive, innate, or complement systems). At the onset of inflammation, various degrees of fibrosis ensue, some of which may be irreversible. Early identification of signs and symptoms is thus imperative for timely diagnosis and early management and, consequently, reduction or even prevention of complications.

There is no current data on the prevalence of paediatric rheumatologic conditions in Southeast Asia given the lack of national registries for such conditions in this region. However, data from surrounding regions reflect an estimated prevalence of around one to four per 1,000 children aged <16 years. [Pediatr Rheumatol Online J 2007;5:21; Curr Rheumatol Rep 2011;13:117-122; Rheumatology (Oxford) 2017;56:390-398] In Singapore, these conditions are estimated to affect ≥3,000 children.

 

Diagnosing paediatric rheumatologic conditions

According to the paediatric rheumatology referral guidelines put forth by the American College of Rheumatology (ACR) in 2010, healthcare professionals should look out for the following signs and symptoms to facilitate an early and accurate diagnosis:

1. Prolonged fever

2. Loss of function (eg, regression in physical skills, inability to attend school)

3. Complaints that are inconsistent with laboratory findings or physical examination

4. Unexplained physical findings (eg, rash, arthritis, anaemia, weakness, weight loss, fatigue, anorexia)

5. Unexplained musculoskeletal pain

6. Undefined autoimmune disease

The ACR guidelines may also help in the diagnostic evaluation and long-term management of other paediatric rheumatologic conditions such as spondyloarthropathies, other arthritides, connective tissue diseases and related syndromes, chronic vasculitides, and other inflammatory diseases.

General practitioners are encouraged to refer to the ACR guidelines or refer paediatric patients with clinical manifestations of rheumatologic conditions to paediatric rheumatology centres, especially for cases requiring second opinion or confirmatory evaluation from specialists. This will help educate families and provide them with measures on how to cope with the disease process affecting their children and manage their expectations.

There are no official local clinical practice guidelines for managing paediatric rheumatologic conditions or a specific test that can confirm the diagnosis. Nonetheless, blood and/or urine tests along with imaging studies may aid in establishing the diagnosis.

It is also important to evaluate for autoimmune disorders associated with other primary diseases such as immunodeficiency, neoplasms, infectious diseases, endocrine disorders, genetic and metabolic diseases, post-transplantation, cystic fibrosis, and arthritis associated with birth defects.

 

Treating paediatric rheumatologic conditions

Treatment will largely depend on patients’ symptoms. Musculoskeletal pain would require non-steroidal anti-inflammatory drugs and re-evaluation within 48–72 hours. The presence of other symptoms merits earlier referral. Unremitting pain would warrant immediate tertiary referral.

Patient perception and treatment adherence are the main challenges in treatment. Some parents may consider alternative remedies to manage their children’s rheumatologic conditions, which could lead to a delay in treatment and/or compromise outcomes.

 

Conclusion

Proper diagnosis and early intervention are advised to minimize short and long-term morbidities and improve overall health outcomes in children with rheumatologic issues. Majority of complications may be prevented with currently available therapies with minimal adverse reactions. Early referral to a paediatric specialist is highly recommended for early diagnosis and treatment.