Emily Rose1, Marcela A Ferrada1, Kaitlin A Quinn1, Wendy Goodspeed1, Laurent Arnaud2, Aman Sharma3, Hajime Yoshifuji4, Jeff Kim5, Clint Allen5, Arlene Sirajuddin6, Marcus Chen6, Peter C Grayson1. 1. National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, Maryland. 2. Hôpitaux Universitaires de Strasbourg, Strasbourg, France. 3. Postgraduate Institute of Medical Education and Research, Sector-12, Chandigarh, India. 4. Graduate School of Medicine, Kyoto University, Kyoto, Japan. 5. National Institutes on Deafness and Other Communication Disorders, NIH, Bethesda, Maryland. 6. National Heart, Lung, and Blood Institute, NIH, Bethesda, Maryland.
Abstract
OBJECTIVE: Relapsing polychondritis (RP) is a systemic inflammatory disorder of cartilage that lacks validated disease activity measures. Our objective was to test physician global assessment (PhGA), a measure of disease activity commonly used in rheumatic diseases, in a cohort of patients with RP, which has not been done before. METHODS: Adult patients in an observational cohort of RP underwent standardized, comprehensive evaluation at approximately 6-month intervals. PhGA was scored by 3 physicians from the evaluating institution on a scale of 0-10 for each visit. A random subset of 20 visits was scored by 3 independent physicians not affiliated with the evaluating institution. Treatment change between consecutive visits was categorized as increased, decreased, or unchanged. RESULTS: In total, 78 patients were evaluated over 164 visits. The intraclass correlation coefficient (ICC2,1 ) for the 3 raters from the evaluating institution was excellent (0.79 [95% confidence interval (95% CI) 0.73, 0.84]) but was poor in the subset of cases scored by the additional raters (ICC2,1 0.27 [95% CI -0.01, 0.53]). Median PhGA was 3 (range 0-7). PhGA weakly correlated with C-reactive protein level (rs = 0.30, P < 0.01). In response to increased treatment, median PhGA decreased from 3 (interquartile range [IQR] 2, 4) to 2 (IQR 2, 3) (P < 0.01) but rarely went to 0. CONCLUSION: Within a single center, PhGA can be used to quantify disease activity and monitor disease response in RP. Persistent disease activity despite treatment, rather than a relapsing-remitting pattern, is observed for most patients with RP. Reliability of PhGA may not generalize across different institutions. A validated disease-specific activity index is needed in RP.
OBJECTIVE: Relapsing polychondritis (RP) is a systemic inflammatory disorder of cartilage that lacks validated disease activity measures. Our objective was to test physician global assessment (PhGA), a measure of disease activity commonly used in rheumatic diseases, in a cohort of patients with RP, which has not been done before. METHODS: Adult patients in an observational cohort of RP underwent standardized, comprehensive evaluation at approximately 6-month intervals. PhGA was scored by 3 physicians from the evaluating institution on a scale of 0-10 for each visit. A random subset of 20 visits was scored by 3 independent physicians not affiliated with the evaluating institution. Treatment change between consecutive visits was categorized as increased, decreased, or unchanged. RESULTS: In total, 78 patients were evaluated over 164 visits. The intraclass correlation coefficient (ICC2,1 ) for the 3 raters from the evaluating institution was excellent (0.79 [95% confidence interval (95% CI) 0.73, 0.84]) but was poor in the subset of cases scored by the additional raters (ICC2,1 0.27 [95% CI -0.01, 0.53]). Median PhGA was 3 (range 0-7). PhGA weakly correlated with C-reactive protein level (rs = 0.30, P < 0.01). In response to increased treatment, median PhGA decreased from 3 (interquartile range [IQR] 2, 4) to 2 (IQR 2, 3) (P < 0.01) but rarely went to 0. CONCLUSION: Within a single center, PhGA can be used to quantify disease activity and monitor disease response in RP. Persistent disease activity despite treatment, rather than a relapsing-remitting pattern, is observed for most patients with RP. Reliability of PhGA may not generalize across different institutions. A validated disease-specific activity index is needed in RP.
Authors: Shubhasree Banerjee; Kaitlin A Quinn; K Bates Gribbons; Joel S Rosenblum; Ali Cahid Civelek; Elaine Novakovich; Peter A Merkel; Mark A Ahlman; Peter C Grayson Journal: J Rheumatol Date: 2019-03-15 Impact factor: 4.666
Authors: Alberto Cauli; Dafna D Gladman; Alessandro Mathieu; Ignazio Olivieri; Giovanni Porru; Paul P Tak; Claudia Sardu; Raffaele Scarpa; Antonio Marchesoni; William J Taylor; Carlo Salvarani; Joachim Kalden; Ennio Lubrano; Sueli Carneiro; Matteo Piga; Alberto Floris; Francesca Desiati; John A Flynn; Salvatore D'Angelo; Arno W R van Kuijk; Maria Grazia Catanoso; Francesco Caso; Paolo Contu; Ilona Ujfalussy; Philip S Helliwell; Philip J Mease Journal: J Rheumatol Date: 2018-06-15 Impact factor: 4.666
Authors: Cynthia Aranow; Anca Askanase; Shereen Oon; Molla Huq; Alicia Calderone; Eric F Morand; Mandana Nikpour Journal: Ann Rheum Dis Date: 2020-04-02 Impact factor: 19.103
Authors: Casey A Rimland; Kaitlin A Quinn; Joel S Rosenblum; Mollie N Schwartz; K Bates Gribbons; Elaine Novakovich; Antoine G Sreih; Peter A Merkel; Mark A Ahlman; Peter C Grayson Journal: Arthritis Care Res (Hoboken) Date: 2020-09 Impact factor: 5.178