From the Guidelines
In systemic sclerosis with very high PEF and low-normal PIF, management should focus on treating the underlying disease and addressing respiratory symptoms, with immunosuppressive therapy such as mycophenolate mofetil or cyclophosphamide, and regular pulmonary function testing every 3-6 months to monitor disease progression, as recommended by the most recent guidelines 1.
Key Considerations
- The pattern of very high PEF and low-normal PIF suggests upper airway dysfunction, specifically extrathoracic airway obstruction, which is consistent with the fibrotic changes that can affect the upper airways in systemic sclerosis 1.
- Immunosuppressive therapy, such as mycophenolate mofetil (1000-1500 mg twice daily) or cyclophosphamide (1-2 mg/kg/day), may help slow disease progression, and is recommended for patients with early diffuse cutaneous systemic sclerosis 1.
- For respiratory symptoms, inhaled corticosteroids like fluticasone (100-500 mcg twice daily) can reduce airway inflammation, and bronchodilators such as albuterol (2 puffs every 4-6 hours as needed) may provide symptomatic relief.
- Pulmonary rehabilitation is also beneficial to maintain respiratory muscle strength and function, and regular pulmonary function testing is essential to monitor disease progression 1.
Screening and Monitoring
- All patients with systemic sclerosis should undergo HRCT screening for interstitial lung disease, and pulmonary function tests, including spirometry and DLCO, should also be performed to provide baseline parameters 1.
- Patients should be monitored closely during the first 5 years of disease, and be referred for HRCT and/or PFTs according to individualized risk stratification 1.
- The functional assessment may also include the 6MWD test, which is widely recognized, simple, non-invasive, low-cost, and reproducible 1.
From the Research
Systemic Sclerosis and Pulmonary Function
- Systemic sclerosis (SSc) is a complex connective tissue disorder that can lead to interstitial lung disease (ILD) and pulmonary hypertension (PH) 2, 3, 4, 5, 6.
- High-resolution computed tomography (HRCT) is a crucial imaging modality for diagnosing and monitoring SSc-ILD, with key features including non-specific interstitial pneumonia (NSIP) pattern, peripheral ground-glass opacities, and extensive traction bronchiectasis 2.
- Pulmonary function tests, such as spirometry and diffusing capacity of the lungs for carbon monoxide, are essential for assessing lung involvement in SSc patients 3, 6.
Pulmonary Hypertension in Systemic Sclerosis
- Pulmonary hypertension (PH) is a common complication in SSc patients, with pulmonary arterial hypertension (PAH) being the most frequent form 4, 5.
- PAH in SSc is characterized by endothelial dysfunction, fibroblast activation, and inflammatory pathways, leading to vascular remodeling and right ventricular failure 4.
- Early diagnosis and treatment of PH in SSc patients are critical, with approved therapies including prostacyclins, endothelin antagonists, and phosphodiesterase type 5 inhibitors 4, 5.
Treatment and Outcomes
- Treatment decisions for SSc-ILD and PH are influenced by baseline and changes in pulmonary function tests, extent of ILD on HRCT, duration and degree of dyspnea, presence of pulmonary hypertension, and potential contribution of reflux 3.
- Mycophenolate mofetil is considered the initial treatment of choice for SSc-ILD, while nintedanib is a therapeutic option for patients with progressive fibrotic ILD despite immunosuppressive therapy or those contraindicated/unable to tolerate immunotherapy 3.
- Survival rates for SSc patients with ILD and PH are generally good, with a 3-year survival rate of 97% for all patients with SSc-associated ILD and 91% for those with coexisting PH 6.