Will a rheumatologist manage limited systemic sclerosis?

Rheumatologists Are the Primary Specialists Who Manage Limited Systemic Sclerosis

Yes, rheumatologists are the primary specialists who manage limited systemic sclerosis, as they have the expertise to coordinate the multisystem care required for this complex connective tissue disease. 1

Role of Rheumatologists in SSc Management

Rheumatologists play a central role in managing limited systemic sclerosis (SSc) for several key reasons:

• They are specifically identified by the European League Against Rheumatism (EULAR) as the specialists who should manage patients with SSc 1
• They coordinate the evidence-based pharmacological treatment of SSc-specific organ involvement according to established guidelines 1
• They lead multidisciplinary teams that may include other specialists depending on organ involvement 2

Management Approach for Limited Systemic Sclerosis

Assessment and Monitoring

• Regular monitoring for early detection of organ involvement through:
  • Pulmonary function tests
  • HRCT of the chest
  • Echocardiography
  • Capillaroscopy 2
• Complete serological testing including anti-centromere antibodies (commonly positive in limited SSc) 2

Organ-Specific Treatment Approaches

Raynaud's Phenomenon and Digital Ulcers

• First-line: Dihydropyridine-type calcium antagonists (usually oral nifedipine) 1
• Second-line: PDE-5 inhibitors for reducing frequency and severity of attacks 1
• Fluoxetine may be considered for SSc-related Raynaud's phenomenon 1, 2
• Bosentan for prevention of new digital ulcers 2

Skin Involvement

• Limited SSc typically has less extensive skin involvement and may not require aggressive systemic immunosuppression for skin manifestations alone 2
• Topical corticosteroids for limited lesions 2
• UVA1 phototherapy for circumscribed lesions 2

Gastrointestinal Involvement

• Proton pump inhibitors for gastroesophageal reflux disease 2
• Prokinetic agents for motility disorders 2
• Rotating antibiotics for bacterial overgrowth 2
• Nutritional support for malnutrition 2

Pulmonary Involvement

• Monitoring for development of interstitial lung disease and pulmonary hypertension 2
• For pulmonary hypertension: Combination therapy with PDE-5 inhibitors and endothelin receptor antagonists 2
• For interstitial lung disease: Mycophenolate mofetil as first-line therapy 2

Renal Involvement

• Monitoring for scleroderma renal crisis
• ACE inhibitors as first-line therapy if renal crisis occurs 2
• Avoidance of high-dose glucocorticoids which may precipitate renal crisis 2

Non-Pharmacological Management

• Patient education about the disease and its management 1, 2
• Support for physical exercise and smoking cessation 1
• Avoidance of cold exposure to reduce Raynaud's attacks 1
• Hand and mouth exercises for limited SSc 1
• Psychological support due to impact on quality of life 1, 2

Important Considerations and Pitfalls

1. Disease Progression Monitoring: Limited SSc patients often have a long history of Raynaud's phenomenon before other symptoms develop, but can still develop serious complications like pulmonary hypertension 3

2. Medication Cautions:

   • Glucocorticoids should be used cautiously as they may precipitate renal crisis 2
   • Immunosuppressive treatments require careful monitoring for adverse effects 2

3. Multisystem Approach: Despite being called "limited," this form of SSc can still cause life-threatening complications from pulmonary hypertension and small intestine hypomotility 3

4. Early Intervention: Early diagnosis and treatment appear to improve function and may impact mortality 4

By following these evidence-based guidelines under rheumatologist direction, patients with limited systemic sclerosis can receive comprehensive care addressing all affected organ systems, potentially improving quality of life and reducing morbidity and mortality.