Recognizing Systemic Sclerosis: comparative analysis of various sets of classification criteria
Data nadesłania: 11-10-2016
Data ostatniej rewizji: 08-11-2016
Data akceptacji: 18-12-2016
Data publikacji online: 30-12-2016
Data publikacji: 30-12-2016
Reumatologia 2016;54(6):296-305
Systemic sclerosis is a complex disease characterized by autoimmunity, vasculopathy and tissue fibrosis. Although most patients present with some degree of skin sclerosis, which is a distinguishing hallmark, the clinical presentation vary greatly complicating the diagnosis. In this regard, new classification criteria were jointly published in 2013 by American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR). A recent major development in the classification criteria is improved sensitivity, particularly for detecting early disease. The new criteria allow more cases to be classified as having systemic sclerosis (SSc), which leads to earlier treatment. Moreover it is clinically beneficial in preventing the disease progression with its irreversible fibrosis and organ damage. The aim of this review is to give insight into new classification criteria and current trends in the diagnosis of systemic sclerosis.
Hoogen F, Khanna D, Johnson SR, et al. Classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum 2013; 65: 2737-2747.
Jordan S, Maurer B, Toniolo M, et al. Performance of the new ACR/EULAR classification criteria for systemic sclerosis in clinical practice. Rheumatology (Oxford) 2015; 54: 1454-1458.
Alhajeri H, Hudson M, Fritzler M, et al. 2013 American College of Rheumatology/European League against rheumatism classification criteria for systemic sclerosis outperform the 1980 criteria: data from the Canadian Scleroderma Research Group.Arthritis Care Res (Hoboken) 2015; 67: 582-587.
Aggarwal R, Ringold S, Khanna D, et al. Distinctions between diagnostic and classification criteria? Arthritis Care Res (Hoboken) 2015; 67: 891-897.
Lonzetti LS, Joyal F, Raynauld JP, et al. Updating the American College of Rheumatology preliminary classification criteria for systemic sclerosis: addition of severe nailfold capillaroscopy abnormalities markedly increases the sensitivity for limited scleroderma. Arthritis Rheum 2001; 44: 735-736.
Nadashkevich O, Davis P, Fritzler MJ. A proposal of criteria for the classification of systemic sclerosis. Med Sci Monit 2004; 10: CR615-621.
LeRoy EC, Medsger TA Jr. Criteria for the classification of early systemic sclerosis. J Rheumatol 2001; 28: 1573-1578.
Avouac J, Fransen J, Walker UA, et al. Preliminary criteria for the very early diagnosis of systemic sclerosis: results of a Delphi Consensus Study from EULAR Scleroderma Trials and Research Group. Ann Rheum Dis 2011; 70: 476-481.
Clements PJ1, Hurwitz EL, Wong WK, et al. Skin thickness score as a predictor and correlate of outcome in systemic sclerosis: high-dose versus low-dose penicillamine trial. Arthritis Rheum 2000; 43: 2445-2454.
Shand L, Lunt M. Relationship Between Change in Skin Score and Disease Outcome in Diffuse Cutaneous Systemic Sclerosis. Arthritis Rheumatism 2007; 56: 2422-2431.
Czirják L, Foeldvari I, Müller-Ladner U. Skin involvement in systemic sclerosis. Rheumatology (Oxford) 2008; 47 Suppl 5: v44-45.
Gerbracht DD, Steen VD, Ziegler GL, et al. Evolution of primary Raynaud’s phenomenon (Raynaud’s disease) to connective tissue disease. Arthritis Rheum 1985; 28: 87-92.
Koenig M, Joyal F, Fritzler MJ, et al. Autoantibodies and microvascular damage are independent predictive factors for the progression of Raynaud’s phenomenon to systemic sclerosis: a twenty-year prospective study of 586 patients, with validation of proposed criteria for early systemic sclerosis. Arthritis Rheum 2008; 58: 3902-3912.
Minier T, Guiducci S, Bellando-Randone S, et al. Preliminary analysis of the Very Early Diagnosis of Systemic Sclerosis (VEDOSS) EUSTAR multicentre study: evidence for puffy fingers as a pivotal sign for suspicion of systemic sclerosis. Ann Rheum Dis 2014; 73: 2087-2093.
Bruni C, Guiducci S, Bellando-Randone S, et al. Digital ulcers as a sentinel sign for early internal organ involvement in very early systemic sclerosis. Rheumatology (Oxford) 2015; 54: 72-76.
Zhang SZ, Xu D, Li MT, et al. Telangiectasia as a potential clinical marker of microvascular lesions in systemic sclerosis patients from EUSTAR data in China. Clin Exp Rheumatol 2015; 33 (4 Suppl 91): S106-110.
Pope JE. The Diagnosis and Treatment of Raynaud’s Phenomenon. A Practical Approach. Drugs 2007; 67: 517-525.
Moinzadeh P, Howell KJ, Ong V, et al. Progression of Isolated Raynaud’s Phenomenon to Systemic Sclerosis: Impact of ANA Subtype And Disease Subset [abstract]. Arthritis Rheum 2011; 63 Suppl 10: 677.
Ingegnoli F, Gualtierotti R, Orenti A, et al. Uniphasic Blanching of the Fingers, Abnormal Capillaroscopy in Nonsymptomatic Digits, and Autoantibodies: Expanding Options to Increase the Level of Suspicion of Connective Tissue Diseases beyond the Classification of Raynaud’s Phenomenon. J Immunol Res 2015; Article ID 371960.
Valentini G, Vettori S, Cuomo G, et al. Early systemic sclerosis: short-term disease evolution and factors predicting the development of new manifestations of organ involvement. Arthritis Res Ther 2012; 4: R188.
Spencer-Green G. Outcomes in primary Raynaud phenomenon: a metaanalysis of the frequency, rates, and predictors of transition to secondary diseases. Arch Intern Med 1998; 158: 595-600.
Herrick AL, Cutolo M. Clinical implications from capillaroscopic analysis in patients with Raynaud’s phenomenon and systemic sclerosis. Arthritis Rheum 2010; 62: 2595-2604.
Cutolo M, Pizzorni C, Tuccio M, et al. Nailfold videocapillaroscopic patterns and serum autoantibodies in systemic sclerosis. Rheumatology (Oxford) 2004; 43: 719-726.
Caramaschi P, Canestrini S, Martinelli N, et al. Scleroderma patients nailfold videocapillaroscopic patterns are associated with disease subset and disease severity. Rheumatology (Oxford) 2007; 46: 1566-1569.
Pucinelli ML, Atra E, Sato EI, et al. Nailfold capillaroscopy in systemic sclerosis: correlations with involvement of lung and esophagus. Rev Bras Rheumatol 1995; 35: 136-142.
Groen H, Wichers G, ter Borg EJ, et al. Pulmonary diffusing capacity disturbances are related to nailfold capillaroscopy changes in patients with Raynaud›s phenomenon with and without an underlying connective tissue disease. Am.
J Med 1990; 89: 34-41.
Bredemeier M, Xavier RM, Capobianco KG, et al. Nailfold capillary microscopy can suggest pulmonary disease activity in systemic sclerosis. J Rheumatol 2004; 31: 286-294.
Sebastiani M, Manfredi A, Colaci M, et al. Capillaroscopic skin ulcer risk index: a new prognostic tool for digital skin ulcer development in systemic sclerosis patients. Arthritis Rheum 2009; 61: 688-694.
Kayser C1, Sekiyama JY, Próspero LC, et al. Nailfold capillaroscopy abnormalities as predictors of mortality in patients with systemic sclerosis. Clin Exp Rheumatol 2013; 31 (2 Suppl 76): 103-108.
Salazar GA, Assassi S, Wigley F, et al. Antinuclear antibody-negative systemic sclerosis. Semin Arthritis Rheum 2015; 44: 680-686.
2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension European Heart Journal doi:10.1093/eurheartj/ehv317.
Steen V, Medsger TA. Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement. Arthritis Rheum 2003; 48: 516-522.
Schreiber BE, Valerio CJ, Keir GJ, et al. Improving the detection of pulmonary hypertension in systemic sclerosis using pulmonary function tests. Arthritis Rheum 2011; 63: 3531-3539.
Coghlan JG, Denton CP, Grunig E, et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis 2014; 73: 1340-1349.
Williams MH, Handler CE, Akram R, et al. Role of N-terminal brain natriuretic peptide (N-TproBNP) in scleroderma-associated pulmonary arterial hypertension. Eur Heart J 2006; 27: 1485-1494.
Cavagna L, Caporali R, Klersy C, et al. Comparison of brain natriuretic peptide (BNP) and NT-proBNP in screening for pulmonary arterial hypertension in patients with systemic sclerosis. J Rheumatol 2010; 37: 2064-2070.
Allanore Y, Borderie D, Avouac J, et al. High N-terminal probrain natriuretic peptide levels and low diffusing capacity for carbon monoxide as independent predictors of the occurrence of precapillary pulmonary arterial hypertension in patients with systemic sclerosis. Arthritis Rheum 2008; 58: 284-291.
Avouac J, Huscher D, Furst DE, et al. Expert consensus for performing right heart catheterisation for suspected pulmonary arterial hypertension in systemic sclerosis: a Delphi consensus study with cluster analysis. Ann Rheum Dis 2014; 73: 191-197.
http://detect-pah.com/ access: 08.11.2016.
Bouros D, Wells AU, Nicholson AG, et al. Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med 2002; 165: 1581-1586.
Bruni C, Guiducci S, Bellando-Randone S, et al. Digital ulcers as a sentinel sign for early internalorgan involvement in very early systemic sclerosis. Rheumatology 2015; 54: 72-76.
Valentini G, Marcoccia A, Cuomo G. Early systemic sclerosis: analysis of the disease course in patients with marker autoantibody and/or capillaroscopic positivity. Arthritis Care Res (Hoboken) 2014; 66: 1520-1527.
D’Angelo WA, Fries JF, Masi AT, Shulman LE. Pathologic observations in systemic sclerosis (scleroderma). A study of fifty-eight autopsy cases and fifty-eight matched controls. Am J Med 1969; 46: 428-440.
Legnani D, Rizzi M, Sarzi-Puttini P, et al. Diffusing Pulmonary Capacity Measured During Effort: A Possible Early Marker of Pulmonary Involvement In Systemic Sclerosis. Isr Med Assoc J 2015; 17: 739-743.
Allanore Y, Meune C, Vonk MC, et al. Prevalence and factors associated with left ventricular dysfunctionin the EULAR Scleroderma Trial and Research group (EUSTAR) database of patients with systemic sclerosis. Ann Rheum Dis 2010; 69: 218-221.
Lee JJ, Pope JE. Diagnosis and Management of Systemic Sclerosis: A Practical Approach. Drugs 2016; 76: 203-213.
Castro SV, Jimenez SA. Biomarkers in systemic sclerosis. Biomark Med 2010; 4: 133-147.
Copyright: © Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie. This is an Open Access journal, all articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0) License (https://creativecommons.org/licenses/by-nc-sa/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
Journals System - logo
Scroll to top