The Primary Care Physician in the Early Diagnosis of Systemic Sclerosis: The Cornerstone of Recognition and Hope

被引:17
作者
Saketkoo, Lesley Ann [1 ]
Magnus, Jeanette H. [2 ,4 ]
Doyle, Mittie K. [3 ,4 ]
机构
[1] Louisiana State Univ, Hlth Sci Ctr, Dept Med, Scleroderma & Sarcoidosis Patient Care & Res Ctr, New Orleans, LA 70112 USA
[2] Univ Oslo, Inst Hlth & Soc, Sch Med, N-0316 Oslo, Norway
[3] Alex Pharmaceut, Translat Med Grp, Cambridge, MA USA
[4] Tulane Univ, Tulane Xavier Natl Ctr Excellence Womens Hlth, Mary Amelia Douglas Whited Community Womens Hlth, New Orleans, LA 70118 USA
基金
美国国家卫生研究院;
关键词
Systemic sclerosis; Screening; Raynaud's; Scleroderma; Fibrosis; Early diagnosis and referral; EULAR SCLERODERMA TRIALS; CONNECTIVE-TISSUE DISEASE; PULMONARY ARTERIAL-HYPERTENSION; SECONDARY RAYNAUDS-PHENOMENON; NAILFOLD CAPILLAROSCOPY; MYCOPHENOLATE-MOFETIL; CELL TRANSPLANTATION; IMMUNOLOGICAL TESTS; GROWTH-FACTOR; HIGH-RISK;
D O I
10.1097/MAJ.0b013e3182a55d24
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Systemic sclerosis (SSc) is a disease of unknown causative factor that manifests as a heterogenous group of multiorgan system manifestations and is characterized by vasculopathy and fibrosis of the skin and internal organs, with mortality related to pulmonary, cardiac, renal or gastrointestinal involvement. The prevalence of SSc may be underestimated in the general population. Cases are often undiagnosed or misdiagnosed, particularly cases with mild or no skin manifestations. Because of late referrals to rheumatologic care, many moderate-to-severe cases progress to irreversible end-organ damage that might have been prevented by early diagnosis. Early diagnosis of SSc with initiation of appropriate treatment is essential, with great impact on morbidity and mortality. This review examines presenting features, ensuing complications and treatment providing a focus on SSc as a treatable disease. Primary care providers play a pivotal role in recognizing initial symptoms associated with SSc and securing early diagnosis through early referral to specialists.
引用
收藏
页码:54 / 63
页数:10
相关论文
共 105 条
[1]   Overview of pathogenesis of systemic sclerosis [J].
Abraham, D. J. ;
Krieg, T. ;
Distler, J. ;
Distler, O. .
RHEUMATOLOGY, 2009, 48 :3-7
[2]   Prevalence and factors associated with left ventricular dysfunction in the EULAR Scleroderma Trial and Research group (EUSTAR) database of patients with systemic sclerosis [J].
Allanore, Y. ;
Meune, C. ;
Vonk, M. C. ;
Airo, P. ;
Hachulla, E. ;
Caramaschi, P. ;
Riemekasten, G. ;
Cozzi, F. ;
Beretta, L. ;
Derk, C. T. ;
Komocsi, A. ;
Farge, D. ;
Balbir, A. ;
Riccieri, V. ;
Distler, O. ;
Chiala, A. ;
Del Papa, N. ;
Simic, K. Pasalic ;
Ghio, M. ;
Stamenkovic, B. ;
Rednic, S. ;
Host, N. ;
Pellerito, R. ;
Zegers, E. ;
Kahan, A. ;
Walker, U. A. ;
Matucci-Cerinic, M. .
ANNALS OF THE RHEUMATIC DISEASES, 2010, 69 (01) :218-221
[3]  
Anderson ME, 2005, J RHEUMATOL, V32, P841
[4]   Systemic sclerosis in northwestern Spain -: A 19-year epidemiologic study [J].
Arias-Nunez, Maria C. ;
Llorca, Javier ;
Vazquez-Rodriguez, Tomas R. ;
Gomez-Acebo, Ines ;
Miranda-Filloy, Jose A. ;
Martin, Javier ;
Gonzalez-Juanatey, Carlos ;
Gonzalez-Gay, Miguel A. .
MEDICINE, 2008, 87 (05) :272-280
[5]   Clinical and laboratory features of Japanese patients with scleroderma and telangiectasia [J].
Ashida, R. ;
Ihn, H. ;
Mimura, Y. ;
Jinnin, M. ;
Asano, Y. ;
Kubo, M. ;
Tamaki, K. .
CLINICAL AND EXPERIMENTAL DERMATOLOGY, 2009, 34 (07) :781-783
[6]   Preliminary criteria for the very early diagnosis of systemic sclerosis: results of a Delphi Consensus Study from EULAR Scleroderma Trials and Research Group [J].
Avouac, J. ;
Fransen, J. ;
Walker, U. A. ;
Riccieri, V. ;
Smith, V. ;
Muller, C. ;
Miniati, I. ;
Tarner, I. H. ;
Bellando Randone, S. ;
Cutolo, M. ;
Allanore, Y. ;
Distler, O. ;
Valentini, G. ;
Czirjak, L. ;
Mueller-Ladner, U. ;
Furst, D. E. ;
Tyndall, A. ;
Matucci-Cerinic, M. .
ANNALS OF THE RHEUMATIC DISEASES, 2011, 70 (03) :476-481
[7]  
Avouac J, 2013, ANN RHEUM DIS
[8]   Insights Into the Pathogenesis of Systemic Sclerosis Based on the Gene Expression Profile of Progenitor-Derived Endothelial Cells [J].
Avouac, Jerome ;
Cagnard, Nicolas ;
Distler, Joerg H. ;
Schoindre, Yoland ;
Ruiz, Barbara ;
Couraud, Pierre Olivier ;
Uzan, Georges ;
Boileau, Catherine ;
Chiocchia, Gilles ;
Allanore, Yannick .
ARTHRITIS AND RHEUMATISM, 2011, 63 (11) :3552-3562
[9]   Characteristics of Joint Involvement and Relationships with Systemic Inflammation in Systemic Sclerosis: Results from the EULAR Scleroderma Trial and Research Group (EUSTAR) Database [J].
Avouac, Jerome ;
Walker, Ulrich ;
Tyndall, Alan ;
Kahan, Andre ;
Matucci-Cerinic, Marco ;
Allanore, Yannick .
JOURNAL OF RHEUMATOLOGY, 2010, 37 (07) :1488-1501
[10]  
Baroni SS, 2006, NEW ENGL J MED, V354, P2667