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Systemic sclerosis

Updated March 14, 2025

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Updated 2024 EULAR guidelines for the management of systemic sclerosis .

Background

Overview

Definition

SSc is a systemic autoimmune disease characterized by diffuse fibrosis and vasculopathy of the skin and internal organs.

Pathophysiology

SSc is caused by a complex interplay of environmental factors that trigger an abnormal immune response in genetically predisposed individuals.

Epidemiology

The overall incidence and prevalence of SSc in the US are 5.6 cases per 100,000 person-years and 13.5-18.4 cases per 100,000 persons, respectively.

Disease course

The resultant vascular injury and endothelial dysfunction lead to vasoconstriction, excessive deposition of extracellular matrix and collagen, hypertrophy of intimal and medial layers of small blood vessels, and endothelial cell apoptosis, resulting in clinical manifestations of Raynaud's phenomenon, digital ulcers, pulmonary arterial hypertension, renal failure, obstructive vasculopathy, and fibrosis of the skin and internal organs.

Prognosis and risk of recurrence

The estimated 5-year and 10-year cumulative survival from the time of diagnosis is 74.9% and 62.5%, respectively.

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of systemic sclerosis are prepared by our editorial team based on guidelines from the American College of Rheumatology (ACR/ACCP 2024), the American Thoracic Society (ATS 2024), the British Society for Rheumatology (BSR 2024), the European League Against Rheumatism (EULAR 2024,2023), the American College of Rheumatology (ACR 2023), the European Society of ...

Screening and diagnosis

Diagnosis: as per BSR 2024 guidelines, guide clinical diagnosis of SSc by validated classification criteria.

ACR/EULAR classification criteria for systemic sclerosis

Calculator

When to use

Entry criteria

Skin thickening involves the fingers

Alternative scleroderma-like disorder diagnoses causing skin thickening such as nephrogenic sclerosing fibrosis, generalized morphea, eosinophilic fasciitis, scleredema diabeticorum, scleromyxedema, erythromelalgia, porphyria, lichen sclerosis, graft-versus-host disease, diabetic cheiroarthropathy) are not present

Skin thickening

Skin thickening of the fingers of both hands extending proximal to the metacarpophalangeal joints

Puffy fingers

Sclerodactyly of the fingers distal to the metacarpophalangeal joints but proximal to the proximal interphalangeal joints

None

Fingertip lesions

Digital tip ulcers

Fingertip pitting scars

None

Telangiectasia

Present

Absent

Abnormal nailfold capillaries

Present

Absent

Pulmonary arterial hypertension and/or interstitial lung disease

Present

Absent

Raynaud's phenomenon

Present

Absent

Systemic sclerosis-related autoantibodies (anti-centromere, anti-Scl-70, and/or anti-RNA polymerase III)

Present

Absent

Calculation

Please enter all the required inputs.

The calculator in question is the ACR/EULAR (American College of Rheumatology/European League Against Rheumatism) Classification Criteria for Systemic Sclerosis (SSc). This scoring system is used in the diagnosis and classification of systemic sclerosis, a chronic autoimmune disease characterized by fibrosis of the skin and internal organs. The ACR/EULAR criteria are applicable to patients who present with clinical features suggestive of systemic sclerosis, such as Raynaud's phenomenon, skin thickening, and internal organ involvement. The criteria are designed to identify patients with systemic sclerosis for inclusion in clinical studies and trials, and to aid in the diagnosis in clinical practice. The clinical utility of this calculator lies in its ability to stratify patients based on the likelihood of having systemic sclerosis. It assigns points for various clinical features and autoantibodies associated with the disease, with a higher score indicating a higher probability of systemic sclerosis. The ACR/EULAR criteria are not applicable to patients who have a scleroderma-like condition that better explains the manifestations (such as nephrogenic sclerosing fibrosis, generalized morphea, eosinophilic fasciitis, scleredema diabeticorum, scleromyxedema, erythromelalgia, porphyria, lichen sclerosis, graft-versus-host disease, diabetic cheiroarthropathy) or when skin thickening spares the fingers.

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Classification and risk stratification

Risk stratification: as per BSR 2024 guidelines, use the skin subset and SSc-associated autoantibody subset to stratify the risk of specific organ-based complications.

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Diagnostic investigations

Capillaroscopy: as per BSR 2024 guidelines, assess nailfold microcirculation with capillaroscopy as part of the initial evaluation and when a diagnosis of SSc is suspected.

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Autoantibody titers
Screening for ILD
Screening for pulmonary hypertension
Screening for chronic infections
Screening for cancer

Medical management

Management of skin disease, diffuse cutaneous SSc: as per BSR 2024 guidelines, define early diffuse cutaneous SSc by disease duration from the first non-Raynaud's phenomenon manifestation of < 3 years, although cases may show improvement in skin from 18 months, and some have clinical features of skin worsening and progression over > 5 years.

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Management of skin disease (non-fibrotic skin manifestations)
Management of skin disease (calcinosis)
Management of Raynaud's phenomenon (initial therapy)
Management of Raynaud's phenomenon (severe cases)
Management of digital ulcerations (PDE5 inhibitors and prostanoids)
Management of digital ulcerations (endothelin receptor antagonists)
Management of digital ulcerations (antiplatelet therapy)
Management of digital ulcerations (surgery)
Management of musculoskeletal disease
Management of ILD (general principles)
Management of ILD (immunosuppressants)
Management of ILD (corticosteroids)
Management of ILD (antifibrotic agents)
Management of ILD (IVIG/plasma exchange)
Management of ILD (stem cell and/or lung transplantation)
Management of ILD (supportive care)
Management of pulmonary hypertension (pharmacotherapy)
Management of pulmonary hypertension (supportive care)
Management of cardiac disease (evaluation)
Management of cardiac disease (immunosuppressants)
Management of cardiac disease (HFrEF)
Management of cardiac disease (HFpEF)
Management of gastrointestinal disease (GERD)
Management of gastrointestinal disease (motility disorders)
Management of gastrointestinal disease (diarrhea and constipation)
Management of gastrointestinal disease (fecal incontinence)
Management of gastrointestinal disease (SIBO)
Management of gastrointestinal disease (nutritional support)
Management of gastrointestinal disease (surgery)
Management of neurological disease
Management of scleroderma renal crisis

Nonpharmacologic interventions

Lifestyle modifications: as per EULAR 2024 guidelines, direct nonpharmacological management in patients with SSc toward improving health-related QoL.

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Physical activity
Lymphatic drainage massage

Therapeutic procedures

HSCT: as per BSR 2024 guidelines, consider offering autologous HSCT in selected patients with diffuse cutaneous SSc where the benefit is likely to be greater than the treatment-related risk. Obtain careful evaluation before autologous HSCT and do not perform it in case of severe internal organ disease.

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Specific circumstances

Pregnant patients

As per BSR 2024 guidelines:

Evaluate for significant renal, cardiac, or lung complications when planning pregnancy in SSc. Discontinue harmful medications and replace them with safer alternatives, such as azathioprine, if necessary.

Manage pregnancy within the context of robust medical support and integrated multidisciplinary care.

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Patient education

General counseling

As per BSR 2024 guidelines:

Take the impact of diagnosis and disease on health-related QoL into consideration in all patients with SSc.

Discuss sexual dysfunction sensitively with the engagement of specialist gynecology, urology, and sexual health clinical services.

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Preventative measures

Routine immunizations: as per BSR 2024 guidelines, consider offering vaccination against SARS-CoV-2, influenza, S. pneumoniae, and herpes zoster using a non-live vaccine.

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Prophylaxis for P. jirovecii pneumonia

Follow-up and surveillance

Respiratory monitoring: as per ACCP/ACR 2024 guidelines, consider obtaining pulmonary function testing, HRCT of the chest, or both, and ambulatory desaturation testing for monitoring patients with SSc-related ILD.

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