Monoclonal Gammopathy–Associated Scleromyxedema Presenting as Leonine Facies

Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/146383/1/art40530.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/146383/2/art40530_am.pd

Identification of Cysteineâ Rich Angiogenic Inducer 61 as a Potential Antifibrotic and Proangiogenic Mediator in Scleroderma

Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/150582/1/art40890.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/150582/2/art40890_am.pd

Reply

Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/147810/1/art40762.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/147810/2/art40762_am.pd

Management of systemic sclerosis‐associated interstitial lung disease in the current era

Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/154421/1/apl13799_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/154421/2/apl13799.pd

Dyspnea assessment and pulmonary hypertension in patients with systemic sclerosis: Utility of the University of California, San Diego, Shortness of Breath Questionnaire

Objective The University of California in San Diego Shortness of Breath Questionnaire (UCSD SOBQ) has been used to assess dyspnea‐related activity limitation in patients with airway and parenchymal lung disease. We sought to assess the construct validity and responsiveness of the UCSD SOBQ in systemic sclerosis (SSc; scleroderma) patients with incident pulmonary hypertension (PH) and those at high risk of developing PH. Methods We used data from 179 patients enrolled in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry with pre‐PH (defined by criteria on pulmonary function tests and/or echocardiogram) or definite PH with mean pulmonary artery pressure ≥25 mm Hg by right‐sided heart catheterization within 6 months of enrollment. For this analysis, we included those subjects with complete data for self‐reported measures at baseline and at 12 months. Results At baseline, the UCSD SOBQ had strong correlations in the expected direction with the disability index (DI) of the Health Assessment Questionnaire (HAQ) (r = 0.71, P < 0.0001), dyspnea assessment by visual analog scale (r = 0.71, P < 0.0001), and the Short Form 36 (SF‐36) health survey physical component summary (PCS) score (r = −0.77, P < 0.0001), as well as a moderate correlation with the 6‐minute walk test distance (r = −0.33, P < 0.0001), Borg dyspnea score (r = 0.47, P < 0.0001), and diffusing capacity of carbon monoxide (r = −0.33, P < 0.0001). Change in the UCSD SOBQ at 12 months correlated in the expected direction with change in the HAQ DI (r = 0.54, P < 0.0001) and change in the SF‐36 PCS (r = −0.44, P < 0.0001). Multivariate analysis adjusting for age, sex, and race identified male sex as a significant predictor of death (odds ratio [OR] 7.00, 95% confidence interval [95% CI] 1.55–31.76), while the UCSD SOBQ showed a strong trend toward significance (OR 1.82, 95% CI 0.97–3.41). Conclusions The UCSD SOBQ demonstrates good construct validity and responsiveness to change in SSc patients with pulmonary vascular disease.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/96723/1/21827_ftp.pd

Disease modification and other trials in systemic sclerosis have come a long way, but have to go further

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/92019/1/21673_ftp.pd

Fatigue predicts future reduced social participation, not reduced physical function or quality of life in people with systemic sclerosis

Funding: The author(s) disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: This work was supported by a grant from the Patient Centered Outcomes Research Institute (PCORI; Poole/Khanna co-PIs) (Award CER-1310-08323 to J.L.P. and D.K.). The statements presented in this publication are solely the responsibility of the authors and do not necessarily represent the views of PCORI. Dr. Khanna’s work was supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases at National Institutes of Health (K24-AR-063129)Peer reviewedPostprin