Casey A Rimland1,2,3, Marcela A Ferrada1,2,3, Ninet Sinaii1,2,3, Keith A Sikora1,2,3, Robert A Colbert1,2,3, Peter C Grayson1,2,3, James D Katz4,5,6. 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.
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.
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.
Authors: Chad E Jacobs; Robert J March; Peter J Hunt; Aksim G Rivera; Sherry Cavanagh; Walter J McCarthy Journal: Vasc Endovascular Surg Date: 2013-05-08 Impact factor: 1.089
Authors: Pablo Arturo Olivo Pallo; Maurício Levy-Neto; Rosa Maria Rodrigues Pereira; Samuel Katsuyuki Shinjo Journal: Rev Bras Reumatol Engl Ed Date: 2017-03-15
Authors: Marcela A Ferrada; Peter C Grayson; Shubhasree Banerjee; Keith A Sikora; Robert A Colbert; Ninet Sinaii; James D Katz Journal: Arthritis Care Res (Hoboken) Date: 2018-07-04 Impact factor: 4.794