This project has provided research infrastructure support for the following clinical protocols:? ? 91-AR-0196 Studies on the Natural History and Pathogenesis of Polymyositis, Dermatomyositis, and Related Diseases? 94-AR-0066 Studies of the Pathogenesis and Natural History of Systemic Lupus Erythematosus (SLE)? 94-AR-0105 Genetics and Pathophysiology of Familial Mediterranean Fever and Related Disorders? 99-D-0070 Natural History of Salivary Gland Dysfunction and Sjogren's Syndrome? 00-AR-0222 Studies of the Pathogenesis and Natural History of Arthritis and Related Conditions? 01-AR-0227 Natural History of Rheumatic Disease in Minority Communities? 02-AR-0156 A Randomized, Double-Blind, Placebo-Controlled Trial of Infliximab in Patients with Dermatomyositis and Polymyositis? 03-AR-0130 Genetic Determinants of Ankylosing Spondylitis Severity - Cross Sectional Study? 03-AR-0131 Genetic Determinants of Ankylosing Spondylitis Severity - Longitudinal Study? 03-AR-0133 Clinically Important Changes in Rheumatoid Arthritis? 03-AR-0173 Studies of the Natural History and Pathogenesis of Neonatal Onset Multisystem Inflammatory Disease (NOMID)? 03-AR-0298 A Pilot Study of the IL-1 Receptor Antagonist Anakinra/Kineret in Patients with Neonatal Onset Multisystem Inflammatory Disease (NOMID/CINCA Syndrome)? 04-AR-0205 Progression of Spinal Fusion in Ankylosing Spondylitis? 05-AR-0014 A Pilot Open-Label Study of IL-1 Trap in Adult Subjects with Autoinflammatory Disease: A Therapeutic Approach to Study Pathogenesis? 07-E-0012 Rituximab in the Treatment of Refractory Adult and Juvenile Dermatomyositis (DM) and Polymyositis (PM)? 08-AR-0008 Insulin Resistance and Atherosclerosis in a Sample of Women with Systemic Lupus Erythematosus? ? In addition to the manuscripts listed in the Publications section, there are a number of other published papers that are based upon clinical support services provided through this project. They include:? ? 1. Hill SC, Namde M, Dwyer A, Poznanski A, Canna S, Goldbach-Mansky R (2007) Arthropathy of neonatal onset multisystem inflammatory disease (NOMID/CINCA). Pediatr Radiol 37:145-52.? 2. Duryea J, Magalnick M, Alli S, Yao L, Wilson M, Goldbach-Mansky R (2008) Semiautomated three-dimensional segmentation software to quantify carpal bone volume changes on wrist CT scans for arthritis assessment. Med Phys 35:2321-30.? 3. Glaser RL, Goldbach-Mansky R (2008) The spectrum of autoinflammatory diseases: recent bench to bedside observations. Curr Opin Rheumatol 20:66-75.? 4. Blackman MR, Muniyappa R, Wilson M, Moquin BE, Baldwin HL, Wong KA, Snyder C, Magalnick M, Alli S, Reynolds J, Steinber SM, Goldbach-Mansky R (2007) Diurnal secretion of growth hormone, cortisol, and dehydroepiandrosterone in pre- and perimenopausal women with active rheumatoid arthritis: a pilot case-control study. Arthritis Res Ther 9:R73.? 5. Lauro CF, Goldbach-Mansky R, Schmidt M, Quezado ZM (2007) The anesthetic management of children with neonatal-onset multi-system inflammatory disease. Anesth Analg 105:351-7.? 6. Louie GH, Chitkara P, Ward MM (2008) Relapse of sarcoidosis upon treatment with etanercept. Ann Rheum Dise 67:896-8.? 7. Ward MM (2007) Association scan of 14,500 nsSNPs in four common diseases identifies variants involved in autoimmunity (2007) Nat Genet 39:1329-37.? 8. Ward MM (2007) Interpreting measurements of physical function in clinical trials. Ann Rheum Dis 66 Suppl 3:iii32-4.? 9. Ward MM, Raveille JD, Learch TJ, Davis JC, Weisman MH (2008) Occupational physical activities and long-term functional and radiographic outcomes in patients with ankylosing spondylitis. Arthritis Rheum 59:822-32.? 10. Ward MM, Reveille JD, Learch TJ, Davis JC, Weisman MH (2008) Impact of ankylosing spondylitis on work and family life: comparisons with the US population. Arthritis Rheum 59:497-503.? 11. Illei GG (2007) The clinical impact of neuropsychiatric manifestations in early systemic lupus erythematosus. Nat Clin Pract Rheumatol 3:428-9.? 12. Illei GG, Yarboro CH, Kuroiwa T, Schlimgen R, Austin HA, Tisdale JF, Chitkara P, Fleisher T, Klippel JH, Balow JE, Boumpas DT (2007) Long-term effects of combination treatment with fludarabine and low-dose pulse cyclophosphamide in patients with lupus nephritis. Rheumatology (Oxford) 46:952-6.

Project Start
Project End
Budget Start
Budget End
Support Year
1
Fiscal Year
2008
Total Cost
$3,389,450
Indirect Cost
Name
National Institute of Arthritis and Musculoskeletal and Skin Diseases
Department
Type
DUNS #
City
State
Country
United States
Zip Code
Kastner, Daniel L; Aksentijevich, Ivona; Goldbach-Mansky, Raphaela (2010) Autoinflammatory disease reloaded: a clinical perspective. Cell 140:784-90
Masters, Seth L; Simon, Anna; Aksentijevich, Ivona et al. (2009) Horror autoinflammaticus: the molecular pathophysiology of autoinflammatory disease (*). Annu Rev Immunol 27:621-68
Goldbach-Mansky, Raphaela; Shroff, Sharukh D; Wilson, Mildred et al. (2008) A pilot study to evaluate the safety and efficacy of the long-acting interleukin-1 inhibitor rilonacept (interleukin-1 Trap) in patients with familial cold autoinflammatory syndrome. Arthritis Rheum 58:2432-42
Chae, Jae Jin; Wood, Geryl; Richard, Katharina et al. (2008) The familial Mediterranean fever protein, pyrin, is cleaved by caspase-1 and activates NF-kappaB through its N-terminal fragment. Blood 112:1794-803
Ryan, John G; de Koning, Heleen D; Beck, Lisa A et al. (2008) IL-1 blockade in Schnitzler syndrome: ex vivo findings correlate with clinical remission. J Allergy Clin Immunol 121:260-2
Ryan, J G; Kastner, D L (2008) Fevers, genes, and innate immunity. Curr Top Microbiol Immunol 321:169-84
Goldbach-Mansky, Raphaela; Pucino, Frank; Kastner, Daniel L (2007) Treatment of patients with neonatal-onset multisystem inflammatory disease/chronic infantile neurologic, cutaneous, articular syndrome: comment on the article by Matsubara et al. Arthritis Rheum 56:2099-101;author reply 2101-2
Wright, Daniel G; Kastner, Daniel L; Pollen, Geraldine B (2007) Challenges and opportunities for systemic amyloidosis research. Summary of an advisory workshop sponsored by the NIH Office of Rare Diseases, Bethesda, Maryland, June 20, 2006. Amyloid 14:103-12
Amos, Christopher I; Chen, Wei Vivien; Remmers, Elaine et al. (2007) Data for Genetic Analysis Workshop (GAW) 15 Problem 2, genetic causes of rheumatoid arthritis and associated traits. BMC Proc 1 Suppl 1:S3
Chitkara, Puja; Stojanov, Silvia; Kastner, Daniel L (2007) The hereditary autoinflammatory syndromes. Pediatr Infect Dis J 26:353-4

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