The Impact of Progressive Pulmonary Fibrosis in Systemic Sclerosis-Associated Interstitial Lung Disease.
María Martín-LópezPatricia E CarreiraPublished in: Journal of clinical medicine (2023)
Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by immune dysregulation and progressive fibrosis, typically affecting the skin, with variable internal organ involvement. Interstitial lung disease (ILD), with a prevalence between 35 and 75%, is the leading cause of death in patients with SSc, indicating that all newly diagnosed patients should be screened for this complication. Some patients with SSc-ILD experience a progressive phenotype, which is characterized by worsening fibrosis on high-resolution computed tomography (HRCT), a decline in lung function, and premature mortality. To assess progression and guide therapeutic decisions, regular monitoring is essential and should include pulmonary function testing (PFT), symptom assessment, and repeat HRCT imaging when indicated. Multidisciplinary discussion allows a comprehensive evaluation of the available information and its consequences for management. There has been a shift in the approach to managing SSc-ILD, which includes the addition of targeted biologic and antifibrotic therapies to standard immunosuppressive therapy (particularly mycophenolate mofetil or cyclophosphamide), with autologous hematopoietic stem-cell transplantation and lung transplantation reserved for refractory cases.
Keyphrases
- interstitial lung disease
- systemic sclerosis
- newly diagnosed
- multiple sclerosis
- lung function
- high resolution
- pulmonary fibrosis
- rheumatoid arthritis
- computed tomography
- end stage renal disease
- cystic fibrosis
- idiopathic pulmonary fibrosis
- ejection fraction
- risk factors
- air pollution
- low dose
- acute myeloid leukemia
- chronic kidney disease
- chronic obstructive pulmonary disease
- type diabetes
- extracorporeal membrane oxygenation
- magnetic resonance imaging
- patient reported
- positron emission tomography
- bone marrow
- cancer therapy
- stem cells
- cardiovascular events
- healthcare
- coronary artery disease
- tandem mass spectrometry
- liver fibrosis
- cardiovascular disease
- quality improvement
- magnetic resonance
- wound healing
- mesenchymal stem cells
- drug delivery