To whom should I refer a patient with scleroderma (systemic sclerosis)?

Referral Recommendations for Scleroderma (Systemic Sclerosis)

Refer all patients with suspected or confirmed systemic sclerosis to a rheumatologist, who should serve as the primary specialist coordinating multidisciplinary care. 1

Primary Referral: Rheumatology

• Rheumatology is the essential first referral for all patients with systemic sclerosis, as rheumatologists are the specialists trained to diagnose, classify, and coordinate the complex multisystem management required for this disease 1
• The EULAR guidelines emphasize that rheumatologists should lead the treatment approach, given SSc remains the rheumatic disease with the highest morbidity and mortality 1
• Less than half of patients are currently diagnosed by rheumatologists, representing a significant gap in optimal care delivery 2

Essential Subspecialty Referrals Based on Organ Involvement

Pulmonology (High Priority)

• Refer immediately if interstitial lung disease (ILD) is suspected or detected, as SSc-ILD is a leading cause of death 1, 3
• Patients positive for anti-topoisomerase I (Scl-70) antibodies require urgent pulmonology evaluation due to higher ILD frequency 3
• Pulmonology consultation is critical for managing pulmonary arterial hypertension, another major cause of mortality 1, 4

Cardiology

• Refer for baseline cardiac evaluation and ongoing monitoring, as cardiac involvement significantly impacts survival 1, 3, 4
• Essential for detecting and managing pulmonary arterial hypertension and scleroderma-related cardiac disease 1

Gastroenterology

• Refer for evaluation and management of gastrointestinal manifestations, which are extremely common in SSc 1
• Despite high prevalence of GI involvement, only 54% of patients see a gastroenterologist, indicating underutilization 2

Nephrology

• Refer urgently if blood pressure elevation or renal dysfunction develops, as scleroderma renal crisis is life-threatening 1, 3
• Regular blood pressure monitoring is essential to detect early renal crisis 3

Dermatology

• Consider referral for complex cutaneous manifestations, digital ulcers, or calcinosis management 1, 5
• Dermatologists can help differentiate SSc from scleroderma-like disorders (pseudosclerodermas) 5

Additional Specialists as Needed

Ophthalmology: Not routinely required for adult SSc (unlike juvenile localized scleroderma where screening for uveitis is recommended) 1

Physical and Occupational Therapy: Refer to improve functional impairment, address deconditioning, and maintain range of motion 3, 6

Orthodontics/Maxillofacial Surgery: Only relevant for juvenile localized scleroderma with facial/head involvement 1

Critical Distinction: Localized vs. Systemic Scleroderma

• For juvenile localized scleroderma (morphea): Refer to specialized pediatric rheumatology centers, as this condition primarily affects skin and subcutaneous tissues without internal organ involvement 1
• For systemic sclerosis (adult scleroderma): Refer to adult rheumatology with multidisciplinary subspecialty involvement based on organ manifestations 1

Common Pitfalls to Avoid

• Do not delay rheumatology referral while waiting for subspecialty evaluations—the mean time to diagnosis is already 2.4 years, which has not improved over three decades 2
• Ensure baseline screening occurs early: Less than 50% of patients receive appropriate baseline testing (echocardiogram, pulmonary function tests, CT chest) at diagnosis 2
• Avoid glucocorticoids in early diffuse cutaneous SSc due to increased scleroderma renal crisis risk—this requires rheumatology expertise to navigate 3
• Do not assume a single specialist can manage SSc alone—even with rheumatology as the lead, coordinated multidisciplinary care is mandatory for optimal outcomes 1, 6