Screening and diagnosis

Classification and risk stratification

Perioperative care

Quality improvement

Patient education

Nonpharmacologic interventions

Therapeutic procedures

Respiratory support

Follow-up and surveillance

Specific circumstances

Preventative measures

Medical management

Surgical interventions

Inpatient care

Diagnostic procedures

Diagnostic investigations

Insufficient evidence to make a recommendation.

Strongly favor this test or intervention.

The ACR/EULAR classification criteria for systemic sclerosis calculator is a tool designed to evaluate various clinical and serological features related to systemic sclerosis, providing a numerical score that helps in determining if diagnostic criteria are met.

The calculator processes eight key inputs:

- **Entry criteria**: Options include skin thickening of the fingers or the absence of alternative diagnoses. Both options automatically contribute 25 points due to their foundational nature in the assessment.
- **Skin thickening**: The degree and location of skin thickening add points: 9 points for thickening on both hands extending beyond the metacarpophalangeal joints, 4 points for sclerodactyly limited to between joints, 2 points for puffy fingers, and 0 if none.
- **Fingertip lesions**: Presence of digital tip ulcers contributes 2 points, whereas fingertip pitting scars add 3 points. No lesions result in 0 points.
- **Telangiectasia**: If present, adds 2 points to the total score; if absent, it contributes 0 points.
- **Abnormal nailfold capillaries**: This condition, if present, adds another 2 points. If absent, it contributes no points.
- **Pulmonary arterial hypertension and/or interstitial lung disease**: This feature provides an additional 3 points if present, with 0 points if absent.
- **Raynaud's phenomenon**: The presence of this phenomenon also contributes 2 points, with no extra points if absent.
- **Systemic sclerosis-related autoantibodies**: Presence of specific autoantibodies yields 3 additional points; an absence results in 0 points.

Each input is assigned a score as per predefined scales, contributing to a comprehensive total score. The total score informs the interpretation:

| Total Score | Interpretation                        |
|-------------|---------------------------------------|
| ≥59         | Diagnostic criteria are met           |
| <59         | Diagnostic criteria are not met       |

In essence, this calculator sums these individual features' scores to provide a total that guides the diagnostic process, with 59 being the threshold above which the systemic sclerosis diagnostic criteria are deemed to be met.

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.

ACR/EULAR classification criteria for systemic sclerosis

Systemic sclerosis

Management of skin disease

Management of skin disease, diffuse cutaneous SSc

Management of interstitial lung disease

Management of ILD, immunosuppressants

Hematopoietic stem cell transplantation

HSCT

Management of digital ulcerations

Management of digital ulcerations, surgery

Lifestyle modifications

Management of pulmonary hypertension

Management of pulmonary hypertension, pharmacotherapy

Screening for pulmonary hypertension

Management of digital ulcerations, PDE5 inhibitors and prostanoids

Management of scleroderma renal crisis

Capillaroscopy

Management of ILD (general principles)

Routine immunizations

Management of gastrointestinal disease

Management of gastrointestinal disease, motility disorders

Risk stratification

Management of cardiac disease

Management of cardiac disease (HFrEF)

Management of skin disease (non-fibrotic skin manifestations)

Screening for cancer

Management of gastrointestinal disease (surgery)

Management of ILD (IVIG/plasma exchange)

General counseling

Management of ILD (supportive care)

Management of digital ulcerations, endothelin receptor antagonists

Respiratory monitoring

Management of ILD, antifibrotic agents

Management of cardiac disease (immunosuppressants)

Management of Raynaud's phenomenon

Management of Raynaud's phenomenon, severe cases

Management of ILD, stem cell and/or lung transplantation

Management of gastrointestinal disease, SIBO

Management of gastrointestinal disease, GERD

Management of gastrointestinal disease (diarrhea and constipation)

Management of cardiac disease (evaluation)

Prophylaxis for Pneumocystis jirovecii pneumonia

Prophylaxis for P. jirovecii pneumonia

Physical activity

Management of gastrointestinal disease (fecal incontinence)

Lymphatic drainage massage

Autoantibody titers

Management of Raynaud's phenomenon, initial therapy

Management of gastrointestinal disease (nutritional support)

Pregnant patients

Screening for chronic infections

Management of musculoskeletal disease

Management of skin disease (calcinosis)

Management of pulmonary hypertension (supportive care)

Management of neurological disease

Management of digital ulcerations (antiplatelet therapy)

Screening for interstitial lung disease

Screening for ILD

Management of cardiac disease (HFpEF)

Diagnosis

Management of ILD (corticosteroids)

Updated 2024 EULAR guidelines for nonpharmacological management of systemic sclerosis


Updated 2024 BSR guidelines for the diagnosis and management of systemic sclerosis


Updated 2024 EULAR guidelines for the management of systemic sclerosis


Systemic sclerosis

Background

Overview

Guidelines

Key sources