Patient-perceived Burden of Disease in Pediatric Relapsing Polychondritis

Casey A Rimland^{1

2

3}, Marcela A Ferrada^{1

2

3}, Ninet Sinaii^{1

2

3}, Keith A Sikora^{1

2

3}, Robert A Colbert^{1

2

3}, Peter C Grayson^{1

2

3}, James D Katz^{4

5

6}

Affiliations

¹ From the US National Institute of Arthritis and Musculoskeletal and Skin Disorders (NIAMS), National Institutes of Health (NIH), Bethesda, Maryland; University of North Carolina at Chapel Hill School of Medicine, Medical Scientist Training Program, Chapel Hill, North Carolina; NIH Clinical Center, Biostatistics and Clinical Epidemiology Service, NIH, Bethesda, Maryland, USA.
² C.A. Rimland, PhD, NIAMS, NIH, and the University of North Carolina at Chapel Hill School of Medicine, Medical Scientist Training Program; M.A. Ferrada, MD, NIAMS, NIH; N. Sinaii, MPH, PhD, NIH Clinical Center, Biostatistics and Clinical Epidemiology Service, NIH; K.A. Sikora, MD, NIAMS, NIH; R.A. Colbert, MD, PhD, NIAMS, NIH; P.C. Grayson, MSc, MD, NIAMS, NIH; J.D. Katz, MD, NIAMS, NIH.
³ C.A. Rimland and Dr. Ferrada are co-first authors. Drs. Grayson and Katz are co-senior authors.
⁴ From the US National Institute of Arthritis and Musculoskeletal and Skin Disorders (NIAMS), National Institutes of Health (NIH), Bethesda, Maryland; University of North Carolina at Chapel Hill School of Medicine, Medical Scientist Training Program, Chapel Hill, North Carolina; NIH Clinical Center, Biostatistics and Clinical Epidemiology Service, NIH, Bethesda, Maryland, USA. james.katz@nih.gov.
⁵ C.A. Rimland, PhD, NIAMS, NIH, and the University of North Carolina at Chapel Hill School of Medicine, Medical Scientist Training Program; M.A. Ferrada, MD, NIAMS, NIH; N. Sinaii, MPH, PhD, NIH Clinical Center, Biostatistics and Clinical Epidemiology Service, NIH; K.A. Sikora, MD, NIAMS, NIH; R.A. Colbert, MD, PhD, NIAMS, NIH; P.C. Grayson, MSc, MD, NIAMS, NIH; J.D. Katz, MD, NIAMS, NIH. james.katz@nih.gov.
⁶ C.A. Rimland and Dr. Ferrada are co-first authors. Drs. Grayson and Katz are co-senior authors. james.katz@nih.gov.

PMID: 31043550
PMCID: PMC6824962
DOI: 10.3899/jrheum.181456

Patient-perceived Burden of Disease in Pediatric Relapsing Polychondritis

Casey A Rimland et al. J Rheumatol. 2019 Dec.

. 2019 Dec;46(12):1627-1633.

doi: 10.3899/jrheum.181456. Epub 2019 May 1.

Authors

Casey A Rimland^{1

2

3}, Marcela A Ferrada^{1

2

3}, Ninet Sinaii^{1

2

3}, Keith A Sikora^{1

2

3}, Robert A Colbert^{1

2

3}, Peter C Grayson^{1

2

3}, James D Katz^{4

5

6}

Affiliations

¹ From the US National Institute of Arthritis and Musculoskeletal and Skin Disorders (NIAMS), National Institutes of Health (NIH), Bethesda, Maryland; University of North Carolina at Chapel Hill School of Medicine, Medical Scientist Training Program, Chapel Hill, North Carolina; NIH Clinical Center, Biostatistics and Clinical Epidemiology Service, NIH, Bethesda, Maryland, USA.
² C.A. Rimland, PhD, NIAMS, NIH, and the University of North Carolina at Chapel Hill School of Medicine, Medical Scientist Training Program; M.A. Ferrada, MD, NIAMS, NIH; N. Sinaii, MPH, PhD, NIH Clinical Center, Biostatistics and Clinical Epidemiology Service, NIH; K.A. Sikora, MD, NIAMS, NIH; R.A. Colbert, MD, PhD, NIAMS, NIH; P.C. Grayson, MSc, MD, NIAMS, NIH; J.D. Katz, MD, NIAMS, NIH.
³ C.A. Rimland and Dr. Ferrada are co-first authors. Drs. Grayson and Katz are co-senior authors.
⁴ From the US National Institute of Arthritis and Musculoskeletal and Skin Disorders (NIAMS), National Institutes of Health (NIH), Bethesda, Maryland; University of North Carolina at Chapel Hill School of Medicine, Medical Scientist Training Program, Chapel Hill, North Carolina; NIH Clinical Center, Biostatistics and Clinical Epidemiology Service, NIH, Bethesda, Maryland, USA. james.katz@nih.gov.
⁵ C.A. Rimland, PhD, NIAMS, NIH, and the University of North Carolina at Chapel Hill School of Medicine, Medical Scientist Training Program; M.A. Ferrada, MD, NIAMS, NIH; N. Sinaii, MPH, PhD, NIH Clinical Center, Biostatistics and Clinical Epidemiology Service, NIH; K.A. Sikora, MD, NIAMS, NIH; R.A. Colbert, MD, PhD, NIAMS, NIH; P.C. Grayson, MSc, MD, NIAMS, NIH; J.D. Katz, MD, NIAMS, NIH. james.katz@nih.gov.
⁶ C.A. Rimland and Dr. Ferrada are co-first authors. Drs. Grayson and Katz are co-senior authors. james.katz@nih.gov.

PMID: 31043550
PMCID: PMC6824962
DOI: 10.3899/jrheum.181456

Abstract

Objective: To assess patient-reported burden of disease in pediatric patients with relapsing polychondritis (RP) and to compare those findings to adult patients.

Methods: A survey based on known clinical symptoms of RP was developed and administered to patients with a pediatric diagnosis of RP. Adult patients completed a similar survey.

Results: Twenty-one pediatric patients, or their parents, completed surveys. Median age at symptom onset was 6 years (interquartile range 1.8-12). Prior to diagnosis, most pediatric patients went to the emergency room (ER; 61.9%), saw > 3 physicians (57.1%), and took > 1 year to be diagnosed (61.9%). Pediatric patients were often diagnosed with asthma (42.9%), ear infections (42.9%), or sinusitis (33.3%) prior to diagnosis of RP. Symptoms prior to diagnosis included ear pain/redness (85.7%), joint pain/swelling (61.9%), and airway symptoms (38.1%). Four pediatric patients (19%) reported tracheomalacia requiring tracheostomy. Pediatric patients frequently missed school because of their disease (71.4%). Surveys from 290 adult patients were compared to pediatric patients. Pediatric patients were significantly more likely to undergo biopsy (42.9% vs 17.4%; p < 0.01) and be treated with biologics (42.9% vs 19%; p = 0.02). Adults were significantly more likely to be female (87.8% vs 28.6%; p < 0.01) and to report airway symptoms (77.9% vs 47.6%; p = 0.01). Prevalence of disease complications was not significantly different between adult and pediatric patients.

Conclusions: The burden of disease in pediatric patients with RP includes missed school, diagnostic delay, ER visits, and multisystem disease, with resultant damage to cartilaginous structures. Differences in airway involvement and treatment approaches may exist between pediatric and adult patients.

Keywords: CARTILAGE; PEDIATRIC RHEUMATIC DISEASES; SELF-ASSESSMENT.

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Figures

**Figure 1.. Symptoms reported by 21 patients with a pediatric diagnosis of relapsing polychondritis.**
Symptoms reported prior to diagnosis with RP or cumulatively experienced by pediatric patients with relapsing polychondritis.

**Figure 2.. Treatment modalities reported by 21 patients with a pediatric diagnosis of relapsing polychondritis.**
Medications reported by patients being taken currently at time of survey or that were taken in the past. TNF = tumor necrosis factor; NSAID = non-steroidal anti-inflammatory drug

See this image and copyright information in PMC

References

1. Cantarini L, Vitale A, Brizi MG, Caso F, Frediani B, Punzi L, et al. Diagnosis and classification of relapsing polychondritis. J Autoimmun 2014;48–49:53–9. - PubMed
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Patient-perceived Burden of Disease in Pediatric Relapsing Polychondritis

Affiliations

Patient-perceived Burden of Disease in Pediatric Relapsing Polychondritis

Authors

Affiliations

Abstract

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