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Systematic Literature Review of the Presentation and Management of Pediatric Relapsing Polychondritis
Mackenzie L Montero1, Roshan M Panda1, Victoria J Palacios1
1University of Nevada, Reno School of Medicine, USA.
Insights
Pediatric Relapsing Polychondritis (RP) is a rare autoimmune cartilage disorder. Corticosteroids and monoclonal antibodies like infliximab show efficacy in treating pediatric RP, improving patient remission rates.
Area of Science:
- Rheumatology
- Pediatric Autoimmune Diseases
- Cartilage Disorders
Background:
- Pediatric Relapsing Polychondritis (RP) is a rare autoimmune condition affecting cartilage in children.
- Limited research necessitates a review of existing pediatric RP literature for presentation, management, and treatment patterns.
Approach:
- A systematic review of PubMed and Embase (1947-April 2023) was performed.
- Studies included patients under 21 meeting McAdam criteria for RP with management details, adhering to PRISMA guidelines.
Key Points:
- The review analyzed 54 studies with 68 pediatric RP patients, predominantly female, with a median diagnostic delay of 1 year and mean age of onset at 12 years.
- Common symptoms included auricular, nasal, and respiratory tract chondritis. Workup often involved imaging (CT/MRI), bronchoscopy, biopsy, and elevated ESR.
- Corticosteroids (91%) and methotrexate (35%) were common medications; tracheostomy (38%) was the most frequent procedure. Monoclonal antibodies (87%) and corticosteroids (66%) demonstrated high efficacy.
Conclusions:
- Pediatric RP commonly presents with chondritis of the ear, nose, and respiratory tract.
- Corticosteroids and monoclonal antibody therapy, particularly infliximab, are effective treatments.
- Anti-rheumatic drugs and monoclonal antibodies, combined with corticosteroids, enhance remission in pediatric RP.
Introduction:
Pediatric Relapsing Polychondritis (RP) is a rare autoimmune disorder that causes inflammation and damage to cartilage in children. Common symptoms include pain, swelling and deformities in the ears, nose, trachea, joints, and eyes. The lack of research on the pediatric population necessitates further evaluation of the literature on pediatric RP to summarize existing patterns in presentation, management, and treatment.
Methods:
A systematic review was conducted on PubMed and Embase from 1947 to April 2023 on RP in patients under 21 years old abiding by the 2020 PRISMA checklist. Only patient presentations meeting McAdam criteria for RP and including information on management were included.
Results:
From the 304 initial studies, 54 studies were included for final analysis with a total of 68 patients, who were predominantly female (65%). With a median diagnostic delay of 1 year, the mean age of onset was 12 years old. The most common symptoms on presentation included bilateral auricular chondritis (69%), nasal cartilage inflammation (62%), and respiratory tract chondritis (63%). The most commonly reported information in the literature for the initial workup usually included CT/MRI (72%), bronchoscopy (57%), biopsy (51%), and labs (88%), which most commonly displayed elevated ESR (59%). The most common medications were corticosteroids (91%) and methotrexate (35%) and the most common procedural treatment was tracheostomy (38%). The most efficacious treatment options were monoclonal antibodies (87%, n = 15) and corticosteroids (66%, n = 62) used in 22% and 91% of patients, respectively. The most commonly used monoclonal antibody therapy was infliximab (13%, n = 9).
Conclusion:
The most common presentation for pediatric RP includes chondritis of the ear, nose, and respiratory tract. The most effective treatment options include corticosteroids and monoclonal antibody therapy, such as infliximab. Our findings highlight increasing remission achieved with anti-rheumatic drugs and monoclonal antibody treatment, especially alongside corticosteroids.
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