From the Guidelines
The new forms of Interstitial Lung Disease (ILD) include a wide range of conditions, such as idiopathic nonspecific interstitial pneumonia (iNSIP), idiopathic pleuroparenchymal fibroelastosis (iPPFE), and lymphangioleiomyomatosis (LAM), among others, as outlined in the 2022 American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Society clinical practice guideline 1.
Key Forms of ILD
- Idiopathic interstitial pneumonias (IIPs), including iNSIP, iPPFE, and idiopathic lymphoid interstitial pneumonia (iLIP)
- Autoimmune-related ILDs, such as rheumatoid arthritis (RA), systemic sclerosis (SSc), and systemic lupus erythematosus (SLE)
- Exposure-related ILDs, including hypersensitivity pneumonitis (HP), occupational ILDs, and medication-induced ILDs
- ILDs with cysts and/or airspace filling, such as lymphangioleiomyomatosis (LAM) and pulmonary alveolar proteinosis (PAP)
Diagnosis and Monitoring
The diagnosis of ILD is based on a combination of clinical, radiological, and pathological findings, as discussed in the guideline 1. High-resolution computed tomography (HRCT) is a crucial tool for diagnosing and monitoring ILD, with features such as fine reticulation, intralobular lines, and architectural distortion indicating early lung fibrosis.
Management
The management of ILD depends on the underlying cause and severity of the disease, with antifibrotic treatment indicated for patients with progressive pulmonary fibrosis (PPF) 1. The guideline recommends antifibrotic treatment for patients diagnosed with IPF and suggests its use in other types of ILD that manifest PPF.
Prognosis
The prognosis of ILD varies depending on the underlying condition, with some forms, such as IPF, having a poor prognosis, while others, such as iNSIP, may have a more favorable outcome 1. Regular follow-up with HRCT and pulmonary function tests is essential to monitor disease progression and adjust treatment accordingly.
From the Research
New Forms of Interstitial Lung Disease (ILD)
- Interstitial lung diseases (ILDs) other than idiopathic pulmonary fibrosis (IPF) have various treatment options, including immunosuppressive therapy and targeted therapies 2, 3.
- Targeted therapies, such as biological disease modifying antirheumatic drugs (DMARDS) and anti-fibrotic agents, are new treatment options for ILD secondary to systemic autoimmune rheumatic disease 3.
- Antifibrotic drug nintedanib has been hypothesized to play a role in treating progressing ILD other than IPF, but caution is warranted due to potential side effects and lack of clear definition of disease progression 4.
- Human immunoglobulin (IVIg) may be a useful complementary therapy in active progressive AS-ILD, but further investigation is required to determine its value as an initial treatment 5.
Emerging Therapies
- Novel therapies, such as biologic agents (antibodies and cell cycle inhibitors) or stem cell therapies, are being developed to target the fibrogenic process and/or secondary pulmonary hypertension (PH) in fibrotic lung disease 6.
- Lung transplantation remains an option for advanced lung disease that is progressive and unresponsive to non-surgical therapies 6.
Treatment Approaches
- Immunomodulatory agents, including corticosteroids, mycophenolate mofetil (MMF), azathioprine, methotrexate, cyclophosphamide, and rituximab, are used to treat ILDs other than IPF 2.
- Classification of ILDs is important for guiding treatment decisions, and therapy augmentation is dictated by the recognition of progression of disease 2.