From the Guidelines
Pulmonary manifestations of rheumatic diseases should be screened and monitored using a combination of pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) chest, as this approach provides complementary information about the presence and pattern of interstitial lung disease (ILD) and its physiologic impact. The epidemiology of ILD in the context of connective tissue disease (CTD) is difficult to determine due to undefined classification criteria, overlap syndromes, and undifferentiated CTD 1. However, it is known that nearly 20% of ILDs reported in Europe and the USA are associated with autoimmune rheumatic diseases and contribute to significant morbidity and early mortality in this setting 1.
Screening and Monitoring
The 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline conditionally recommends screening with PFTs and HRCT chest over PFTs alone for people with systemic autoimmune rheumatic diseases (SARDs) at increased risk of developing ILD 1. This is because PFTs alone may be insufficient to detect ILD, and HRCT chest has a high sensitivity for detecting ILD, even in patients with normal PFTs 1.
Diagnosis and Treatment
Treatment typically involves immunosuppression with corticosteroids, along with steroid-sparing agents like mycophenolate mofetil, cyclophosphamide, or rituximab 1. Pulmonary function tests and high-resolution CT scans are essential for diagnosis and monitoring, and early detection is crucial as lung involvement significantly impacts prognosis 1.
Monitoring Frequency
For people with SARD-ILD, the guideline suggests monitoring with PFTs every 3-6 months rather than either shorter or longer intervals for the first year, then less frequently once stable 1. For people with IIM-ILD and SSc-ILD, PFTs for monitoring every 3–6 months is recommended, while for people with RA-ILD, SjD-ILD, and MCTD-ILD, PFTs for monitoring every 3–12 months is suggested 1.
Key Points
- Early detection and monitoring of pulmonary manifestations of rheumatic diseases are crucial to improve outcomes.
- A combination of PFTs and HRCT chest is recommended for screening and monitoring.
- Treatment involves immunosuppression with corticosteroids and steroid-sparing agents.
- Monitoring frequency depends on the underlying disease and disease activity.
From the Research
Pulmonary Manifestations of Rheumatic Disease
- Rheumatic diseases can present with various pulmonary manifestations, including interstitial lung disease (ILD), pleural effusion, and pulmonary hypertension 2, 3, 4, 5, 6
- ILD is a severe pulmonary complication in inflammatory rheumatic diseases (IRD) and is associated with significantly increased morbidity and mortality 2
- The most common pulmonary abnormalities on high-resolution computed tomography (HRCT) in patients with IRD are ground-glass opacities, non-specific interstitial pneumonia, and usual interstitial pneumonia 2
Diagnosis and Screening
- A stepwise approach, including pulmonary function testing, chest radiography, and HRCT, can be used as a potential screening tool for detecting early ILD in patients with newly diagnosed IRD 2
- The combination of reduced diffusing capacity for carbon monoxide, chest X-ray, and pulmonary HRCT yields the highest sensitivity and specificity in detecting ILD at the onset of IRD 2
- Regular monitoring is important to enable the prompt identification of progression and early intervention to preserve lung function in patients with RA-ILD 6
Pathogenesis and Management
- The pathogenesis of PAH in rheumatic diseases is complex and involves multiple mechanisms, including obliterative vasculopathy, veno-occlusive disease, and immune-mediated vasculopathy 5
- The management of RA-ILD requires a multidisciplinary and individualized approach, taking account of the severity and progression of articular and lung disease, risk factors for the progression of RA-ILD, and the patient's preferences 6
- Treatment options for PAH in rheumatic diseases may include immunosuppressive therapy, vasodilator therapy, and supportive care 5