What is the recommended management approach for a patient with systemic sclerosis?

Systemic Sclerosis Case Management

Systemic sclerosis management requires a holistic, multidisciplinary approach combining non-pharmacological interventions as foundational therapy alongside organ-specific pharmacological treatments, with patient education and self-management support as essential components from diagnosis onward. 1

Core Management Principles

Non-pharmacological management must complement—not substitute for—pharmaceutical treatment when pharmacotherapy is required. 1 The approach should be:

• Tailored and person-centered with active patient participation in care decisions 1
• Multidisciplinary involving rheumatologists, pulmonologists, gastroenterologists, cardiologists, and other specialists as organ involvement dictates 2
• Directed toward improving health-related quality of life as the primary outcome 1

Essential Non-Pharmacological Interventions

Patient Education and Self-Management Support

All patients with systemic sclerosis should receive structured patient education and self-management support from diagnosis. 1 This intervention improves:

• Hand function (Level of Evidence 2-4) 1
• Mouth-related outcomes (LoE 2) 1
• Health-related quality of life (LoE 2-4) 1
• Ability to perform daily activities (LoE 2-3) 1

Physical Exercise Programs

Physical exercise should be implemented for all patients unless contraindicated (LoE 2-4). 1 Specific exercise recommendations include:

• Orofacial exercises to improve microstomia (LoE 2-4) 1
• Hand exercises (self-administered stretching and mobility exercises) to improve hand function (LoE 2-4) 1
• Aerobic and resistance exercise to improve physical capacity (LoE 2-4) 1
• Home-based programs combining stationary bike aerobic exercise, upper limb muscular endurance training, and hand stretching following physiotherapist-supported education 1

Smoking Cessation

Smoking habits must be assessed at every visit, and cessation strategies implemented immediately (LoE 3-4). 1 Smoking directly worsens vasospasm and disease progression. 3

Cold Avoidance for Raynaud's Phenomenon

All patients should receive specific instructions for cold avoidance (LoE 4), which is particularly critical for those with severe Raynaud's phenomenon. 1 Practical measures include:

• Use of gloves and heating devices for hands 1, 3
• Avoidance of direct contact with cold surfaces 1, 3
• Thorough drying of skin after moisture exposure 1, 3
• Discontinuation of triggering medications (beta-blockers, ergot alkaloids, bleomycin, clonidine) 3

Organ-Specific Pharmacological Management

Raynaud's Phenomenon and Digital Ulcers

First-line pharmacotherapy is nifedipine (calcium channel blocker), which reduces both frequency and severity of attacks. 3 Escalation algorithm:

1. Nifedipine as initial therapy 3
2. Add phosphodiesterase-5 inhibitor (sildenafil or tadalafil) for inadequate response 3, 4
3. Intravenous iloprost for severe Raynaud's unresponsive to oral therapies 3, 4
4. Bosentan (endothelin receptor antagonist) for prevention of new digital ulcers, particularly in patients with multiple existing ulcers 3, 4

Scleroderma Renal Crisis

Angiotensin-converting enzyme inhibitors (ACEi) are first-line therapy for scleroderma renal crisis. 4 For inadequate response:

• Add calcium channel blocker or angiotensin receptor blocker 4
• Then add alpha-blocker 4

Critical pitfall: Avoid high-dose corticosteroids (≥15 mg/day prednisone) as this increases renal crisis risk 4-fold. 5 If corticosteroids are required, use ≤10 mg/day with close blood pressure and renal function monitoring. 5

Pulmonary Arterial Hypertension

For mild PAH, initiate endothelin receptor antagonist (ERA) as first-line therapy (72% expert consensus). 4 Escalation:

1. ERA as initial treatment 4
2. Add phosphodiesterase-5 inhibitor (77% agreement) 4
3. Add prostanoid (73% agreement) 4

For severe PAH, initial treatment options include:

• Prostanoid monotherapy (49% preference) 4
• Combination ERA + PDE5i (18% preference) 4
• Combination ERA + prostanoid (16% preference) 4

Interstitial Lung Disease

For induction therapy, use intravenous cyclophosphamide or mycophenolate mofetil. 4 For maintenance therapy, mycophenolate mofetil is preferred (chosen by 75% of experts). 4

High-resolution computed tomography (HRCT) is the gold standard for diagnosis as pulmonary function tests lack sensitivity and specificity in early disease. 2 Most patients demonstrate non-specific interstitial pneumonia (NSIP) pattern. 2

Gastrointestinal Involvement

Proton pump inhibitors are first-line for gastroesophageal reflux disease. 4 More than 50% of experts would exceed maximum recommended PPI dose if required. 4 Add promotility agents for dysmotility. 6

Skin Involvement

After methotrexate failure, mycophenolate mofetil is the preferred second-line agent (37% expert consensus). 4

Inflammatory Arthritis

Methotrexate is first-line therapy (60% consensus). 4 For inadequate response, add corticosteroids (37%) or hydroxychloroquine (31%). 4

Critical Monitoring Requirements

High-Risk Patient Identification

Patients with tendon friction rubs require intensive monitoring as they have >2-fold increased risk of scleroderma renal crisis, cardiac complications, and gastrointestinal disease. 5 Implement:

• Home blood pressure monitoring to detect early renal crisis 5
• Regular cardiac evaluation 5
• Gastrointestinal symptom assessment 5

Routine Screening

Screen all patients at diagnosis and regularly thereafter for:

• Interstitial lung disease (HRCT, pulmonary function tests including DLCO) 6, 2, 7
• Pulmonary hypertension (echocardiography, right heart catheterization when indicated) 6, 7
• Cardiac involvement (ECG, echocardiography, cardiac MRI when indicated) 6, 7
• Renal function (blood pressure, creatinine, urinalysis) 6, 7
• Digital ischemia and ulceration 6

Early organ involvement is often asymptomatic, making proactive screening essential for preventing irreversible damage. 6, 2

Common Pitfalls to Avoid

• Do not delay escalation of therapy in secondary Raynaud's phenomenon, as this leads to digital ulcers and poor outcomes 3
• Do not continue vasoconstrictive medications (beta-blockers, etc.) as this undermines all treatment efforts 3
• Do not underestimate non-pharmacological measures—they form the foundation of management and should be implemented immediately 3
• Do not use high-dose corticosteroids in patients with tendon friction rubs or diffuse cutaneous disease due to renal crisis risk 5
• Do not rely solely on symptoms for organ screening—many manifestations are subclinical until advanced 6, 2

Manual Lymph Drainage

For patients with puffy hands, manual lymph drainage could be considered to improve hand function (LoE 2). 1