Highlights
- •A prompt detection of the different manifestations of systemic sclerosis affects prognosis but it is challenging in early stages when most of the typical signs are absent.
- •Patients with Raynaud phenomenon, abnormal findings in systemic sclerosis nailfold capillary pattern and the presence of specific autoantibody at baseline had very high probability of developing definite systemic sclerosis in the follow-up.
- •Internal organ involvement could be present since the early phase of the disease.
Keywords
Abbreviation:
SSc (Systemic Sclerosis)To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to European Journal of Internal MedicineAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Systemic sclerosis.Lancet. 2017; 390: 1685-1699
- Improved survival in systemic sclerosis is associated with better ascertainment of internal organ disease: a retrospective cohort study.QJM. 2010; 103: 109-115
- Early systemic sclerosis: assessment of clinical and pre-clinical organ involvement in patients with different disease features.Rheumatology. 2011; 50: 317-323
- Development of connective tissue disease in patients presenting with Raynaud’s phenomenon: a six year follow up with emphasis on the predictive value of antinuclear antibodies as detected by immunoblotting.Ann Rheum Dis. 1988; 47: 634-641
- 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
- Scleroderma (systemic sclerosis): classification, subsets and pathogenesis.J Rheumatol. 1988; 15: 202-205
- Very early and early systemic sclerosis in the Spanish scleroderma Registry (RESCLE) cohort.Autoimmun Rev. 2017; 16: 796-802
- Systemic sclerosis: current pathogenetic concepts and future prospects for targeted therapy.Lancet. 1996; 347: 1453-1458
- Criteria for the classification of early systemic sclerosis.J Rheumatol. 2001; 28: 1573-1576
- 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
- 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative.Arthritis Rheum. 2013; 65: 2737-2747
- Very early systemic sclerosis and pre-systemic sclerosis: definition, recognition, clinical relevance and future directions.Curr Rheumatol Rep. 2017; 19: 65
- Digital ulcers as a sentinel sign for early internal organ involvement in very early systemic sclerosis.Rheumatology. 2015; 54: 72-76
- From VEDOSS to established systemic sclerosis diagnosis according to ACR/EULAR 2013 classification criteria: a French-Italian capillaroscopic survey.Clin Exp Rheumatol. 2018; 36: 82-87
- Evidence for oesophageal and anorectal involvement in very early systemic sclerosis (VEDOSS): report from a single VEDOSS/EUSTAR centre.Ann Rheum Dis. 2015; 74: 124-128
- Interstitial lung disease in systemic sclerosis: data from the spanish scleroderma study group.Rheumatol Int. 2018; 38: 363-374
- Recommendations for screening and detection of connective tissue disease-associated pulmonary arterial hypertension.Arthritis Rheum. 2013; 65: 3194-3201
- Screening for pulmonary arterial hypertension in systemic sclerosis: a systematic literature review.Eur J Intern Med. 2020; 78: 17-25
- N-terminal pro brain natriuretic peptide: the new cornerstone of cardiovascular assessment in systemic sclerosis.Clin Exp Rheumatol. 2009; 27: 59-63
- Defining primary systemic sclerosis heart involvement: a scoping literature review.Semin Arthritis Rheum. 2019; 48: 874-887
- Cardiac troponin T and NT-proBNP as diagnostic and prognostic biomarkers of primary cardiac involvement and disease severity in systemic sclerosis: a prospective study.Eur J Intern Med. 2019; 60: 46-53
- Undifferentiated connective tissue disease at risk for systemic sclerosis: which patients might be labeled prescleroderma?.Autoimmun Rev. 2020; 19102659
- Early systemic sclerosis: short-term disease evolution and factors predicting the development of new manifestations of organ involvement.Arthritis Res Ther. 2012; 14: R188
- Registry of the Spanish network for systemic sclerosis: clinical pattern according to cutaneous subsets and immunological status.Semin Arthritis Rheum. 2012; 41: 789-800
Article info
Publication history
Published online: December 27, 2021
Accepted:
December 7,
2021
Received in revised form:
December 5,
2021
Received:
July 3,
2021
Identification
Copyright
© 2021 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.