Systemic sclerosis interstitial lung disease: unmet needs and potential solutions.
Vasiliki LiakouliAntonio CiancioFrancesco Del GaldoRoberto GiacomelliFrancesco CicciaPublished in: Nature reviews. Rheumatology (2023)
Systemic sclerosis (SSc), or scleroderma, is a rare, complex, systemic autoimmune disease of unknown aetiology, characterized by high morbidity and mortality often resulting from cardiopulmonary complications such as interstitial lung disease and pulmonary arterial hypertension. Despite substantial progress in unravelling the pathways involved in the pathogenesis of SSc and the increasing number of therapeutic targets tested in clinical trials, there is still no cure for this disease, although several proposed treatments might limit the involvement of specific organs, thereby slowing the natural history of the disease. A specific focus of recent research has been to address the plethora of unmet needs regarding the global management of SSc-related interstitial lung disease, including its pathogenesis, early diagnosis, risk stratification of patients, appropriate treatment regimens and monitoring of treatment response, as well as the definition of progression and predictors of progression and mortality. More refined stratification of patients on the basis of clinical features, molecular signatures, identification of subpopulations with distinct clinical trajectories and implementation of outcome measures for future clinical trials could also improve therapeutic management strategies, helping to avoid poor outcomes related to lung involvement.
Keyphrases
- interstitial lung disease
- systemic sclerosis
- clinical trial
- rheumatoid arthritis
- pulmonary arterial hypertension
- idiopathic pulmonary fibrosis
- end stage renal disease
- ejection fraction
- chronic kidney disease
- primary care
- prognostic factors
- risk factors
- coronary artery disease
- type diabetes
- gene expression
- patient reported outcomes
- climate change
- drug induced
- double blind
- dna methylation
- phase ii
- study protocol