Systemic sclerosis, scleroderma - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Systemic sclerosis (scleroderma) is a noncontagious, chronic, multisystem disease.
Initial symptoms are nonspecific and include fatigue, vague musculoskeletal complaints, diffuse swelling of hands, and Raynaud phenomenon.
Etiology and pathogenesis are unknown.
Disease course is variable, but the condition rarely subsides spontaneously.
There are two main subtypes of systemic sclerosis (SSc): limited cutaneous SSc and diffuse cutaneous SSc. The limited cutaneous form tends to have less severe internal organ involvement and a better prognosis, but these subjects still need to be followed closely for possible complications.

Fingers demonstrating sclerodactyly with finger curling, shiny skin at the fingers, and telangiectasias

From the collection of Maureen D. Mayes, University of Texas

Quick Reference

History & Exam
- Key Factors
- Other Factors
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Diagnostics Tests
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Treatment Options
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Definition

Epidemiology

Etiology

Pathophysiology

Images

Digital pits without ulcers
Hand demonstrating sclerodactyly with finger curling, shiny skin at the fingers, and telangiectasias
Telangiectasias on the hand
Telangiectasias on the fingers
Digital tip infarction
Digital tip infarction
Hands demonstrating Raynaud phenomenon
Fingers demonstrating sclerodactyly with finger curling, shiny skin at the fingers, and telangiectasias
Late-stage sclerodactyly with contractures due to severe skin tightening
Late-stage sclerodactyly with contractures due to severe skin tightening
Digital tip infarction

Citations

Key Articles

van den Hoogen F, Khanna D, Fransen J, et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Ann Rheum Dis. 2013 Nov;72(11):1747-55.[Abstract][Full Text]
Kowal-Bielecka O, Fransen J, Avouac J, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017 Aug;76(8):1327-1339.[Abstract][Full Text]
Baron M, Bernier P, Côté LF, et al. Screening and therapy for malnutrition and related gastro-intestinal disorders in systemic sclerosis: recommendations of a North American expert panel. Clin Exp Rheumatol. 2010 Mar-Apr;28(2 Suppl 58):S42-6.[Abstract][Full Text]
American College of Rheumatology. 2023 American College of Rheumatology (ACR) guideline for the treatment of interstitial lung disease in people with systemic autoimmune rheumatic disease. Aug 2023 [internet publication].[Full Text]
Iudici M, van der Goes MC, Valentini G, et al. Glucocorticoids in systemic sclerosis: weighing the benefits and risks - a systematic review. Clin Exp Rheumatol. 2013 Mar-Apr;31(2 Suppl 76):157-65.[Abstract]
Johnson SR, Brode SK, Mielniczuk LM, et al. Dual therapy in IPAH and SSc-PAH. A qualitative systematic review. Respir Med. 2012 May;106(5):730-9.[Abstract]

Other Online Resources

American College of Rheumatology
Scleroderma Foundation
National Institutes of Health: clinical trials

Referenced Articles

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2. Hoffmann-Vold AM, Gunnarsson R, Garen T, et al. Performance of the 2013 American College of Rheumatology/European League Against Rheumatism classification criteria for systemic sclerosis (SSc) in large, well-defined cohorts of SSc and mixed connective tissue disease. J Rheumatol. 2015 Jan;42(1):60-3.[Abstract]
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