European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes

Background:Systemic Sclerosis (SSc) overlap syndromes (SSc with polymyositis / dermatomyositis (PM/DM), rheumatoid arthritis (RA), etc.) still remain a group of very heterogenous and not very well studied clinical variants of SSc that are characterized by certain clinical and immunological features.Objectives:Identify clinical and immunological features of the SSc-overlap syndromesMethods:80 pts with SSc-PM/DM and 35 pts with SSc-RA undergoing standard clinical examination and laboratory immunological evaluation.Results:ANA Hep2 was positive in 98% of SSc-PM/DM pts; a-Scl-70 was in 34%, a - PM-Scl and RF were in 20%. ACA (6%), a-RNP (9%), and a - Jo-1 (5%) were significantly less common. Correlation analysis showed significant prevalence of conduction abnormalities in pts with a-Scl-70- (p<0.03); PM-Scl was rarely associated with cardiac arrhythmia (p<0.02) and pericarditis (p<0.03), but there was an association between ACA and presence of digital ischemia (p<0.04). Three pts with limited skin had Scl-70 and PM-Scl antibodies, two of them manifested clinical features of DM. A-Jo-1 was found in 3 pts with a longstanding disease (14,10 and 7 years), and one of these pts was also positive for a-Scl-70. All pts had limited skin and two had interstitial lung disease with FVC values of 79% and 74.8%.ANA Hep2 was positive in 96% of SSc-RA pts; a-Scl-70 – in 28%, and a-RNP - in 30%. RF-positivity was in 72% of pts, and Anti-CCP - in 27%. Simultaneous Anti-CCP and a-Scl-70 was found in one case, and Anti-CCP - anti-RNP – in another, both were associated with low RF titers. All pts had early joint involvement which became prevailing in subsequent years, and onset of the disease between 30 and 36 years. There was a correlation between laboratory signs of inflammatory activity and immunological disorders: ESR and a-Scl-70 (p<0.03). Anti-CCP and a-Scl-70 co-positivity was a significantly less frequent phenomenon (p<0.04). There was a remarkable 28% proportion of a-Scl-70 cases in SSc-RA with limited cutaneous which is usually characterized by ACA-positivity.Conclusion:SSc-PM/DM and SSc-RA appear to be an active disease from the immunological point of view, confirming therefore an important role of immune alterations in disease progression. Laboratory findings display specific pathogenetic features of SSc-overlap syndromes; laboratory abnormalities can be used to measure the activity and specify characteristics of the pathological process.Disclosure of Interests:None declared

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Skin thickness and collagen content in progressive systemic sclerosis and localized scleroderma

Arthritis & Rheumatism ◽

10.1002/art.1780220205 ◽

1979 ◽

Vol 22 (2) ◽

pp. 130-140 ◽

Cited By ~ 213

Author(s):

Gerald P. Rodnan ◽

Esther Lipinski ◽

Joan Luksick

Keyword(s):

Systemic Sclerosis ◽

Progressive Systemic Sclerosis ◽

Collagen Content ◽

Localized Scleroderma ◽

Skin Thickness

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Localized Scleroderma, Systemic Sclerosis and Cardiovascular Risk: A Danish Nationwide Cohort Study

Acta Dermato Venereologica ◽

10.2340/00015555-2842 ◽

2018 ◽

Vol 98 (3) ◽

pp. 361-365 ◽

Cited By ~ 7

Author(s):

J Hesselvig ◽

K Kofoed ◽

J Wu ◽

L Dreyer ◽

G Gislason ◽

...

Keyword(s):

Cohort Study ◽

Cardiovascular Risk ◽

Systemic Sclerosis ◽

Localized Scleroderma

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Systemic sclerosis complicated with localized scleroderma-like lesions induced by Köbner phenomenon

Journal of Dermatological Science ◽

10.1016/j.jdermsci.2017.12.005 ◽

2018 ◽

Vol 89 (3) ◽

pp. 282-289 ◽

Cited By ~ 9

Author(s):

Ryosuke Saigusa ◽

Yoshihide Asano ◽

Takashi Yamashita ◽

Takehiro Takahashi ◽

Kouki Nakamura ◽

...

Keyword(s):

Systemic Sclerosis ◽

Localized Scleroderma

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Scleroderma and Related Disorders

10.2310/fm.1211 ◽

2017 ◽

Author(s):

Kristine Phillips

Keyword(s):

Differential Diagnosis ◽

Antinuclear Antibodies ◽

Clinical Course ◽

Clinical Manifestations ◽

Diagnosis And Treatment ◽

Localized Scleroderma ◽

Eosinophilic Fasciitis ◽

Tomographic Images ◽

Computed Tomographic

Scleroderma spectrum diseases are a heterogeneous group of disorders that are distinguished by abnormalities of the connective tissue in the skin and, in some cases, other organs. Each disorder may be characterized by the extent of cutaneous and internal involvement, as well as histopathologic features of skin biopsy. Scleroderma spectrum diseases include systemic scleroderma, localized scleroderma, and eosinophilic fasciitis. This chapter reviews the classification, epidemiology, etiology, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, outcome measures, management, and clinical course of scleroderma as well as the definition and classification, etiology/genetics, differential diagnosis, and treatment of localized scleroderma. Also discussed are the definition and classification, epidemiology, etiology/genetics/pathogenesis, diagnosis, differential diagnosis, and treatment of eosinophilic fasciitis. Tables review the classification of—and antinuclear antibodies in—scleroderma as well as the key assessments and interventions in scleroderma management. Figures illustrate the disease's presentation and clinical manifestations, including several images of scleroderma of the hands; face, palmar, and buccal telangiectasias in a patient with scleroderma; a radiograph demonstrating calcinosis of the elbow; Raynaud’s phenomenon; high-resolution computed tomographic images of diffuse cutaneous scleroderma, scleroderma and severe pulmonary hypertension, and limited cutaneous scleroderma; plus an esophagram demonstrating hypomotility. This review contains 11 highly rendered figures, 3 tables, and 72 references.

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Systemic sclerosis

Oxford Textbook of Medicine ◽

10.1093/med/9780199204854.003.191103 ◽

2010 ◽

pp. 3664-3678

Author(s):

Christopher P. Denton ◽

Carol M. Black

Keyword(s):

Systemic Lupus Erythematosus ◽

Systemic Sclerosis ◽

Lupus Erythematosus ◽

Raynaud’S Phenomenon ◽

Raynaud's Phenomenon ◽

Systemic Lupus ◽

Overlap Syndromes ◽

Autoimmune Rheumatic Disease ◽

Skin Sclerosis ◽

Important Form

The scleroderma spectrum of disorders includes a number of diseases that have Raynaud’s phenomenon or skin sclerosis in common, comprising (1) limited cutaneous scleroderma; (2) systemic sclerosis (SSc)—the most important form of scleroderma—limited cutaneous SSc, diffuse cutaneous SSc, and overlap syndromes (with features of another autoimmune rheumatic disease, e.g. systemic lupus erythematosus); (3) Raynaud’s phenomenon—autoimmune (with antinuclear or other SSc-associated antibodies) or primary. These conditions affect women four times as often as men, most often beginning in the fifth decade....

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