A panel of experts in Europe defined a set of recommendations for the diagnosis and management of interstitial lung disease (ILD) in people with systemic sclerosis (SSc), based on a systematic literature review and expert consensus.
The guidelines were outlined in a report, “The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements,” published in the journal The Lancet Rheumatology.
ILD, the leading cause of mortality in SSc patients, refers to a group of lung disorders in which the tissue in and around the lung air sacs becomes inflamed and scarred, progressively compromising breathing and the lungs’ ability to transfer oxygen to the bloodstream.
SSc patients are at high risk of developing ILDs, with half of them showing the presence of one when first assessed by high resolution computed tomography (HRCT) scans.
While early diagnosis, assessment of disease severity, prediction of disease progression, and appropriate treatment of SSc-associated ILD (SSc-ILD) is crucial to achieve the best possible outcomes, there are no SSc-ILD-specific guidelines based on expert consensus.
The panel of 27 European experts — 16 rheumatologists, seven pulmonologists, and four internists — said they developed the first evidence-based expert consensus, and defined an algorithm for the identification and management of ILD in SSc patients.
They conducted a systematic review of literature from 1992 to 2018 to define an initial set of evidence-based draft statements for six aspects of SSc-ILD management: risk factors, screening, diagnosis and severity assessment, treatment initiation and options, disease progression, and treatment escalation.
To reach a consensus, the panel revised the statements using a modified Delphi method involving three rounds of online surveys, followed by face-to-face discussions. Consensus was considered achieved if at least 80% of experts agreed or disagreed on a statement.
The recommendations are briefly described below.
SSc-ILD risk factors
Respiratory symptoms, smoking history, ethnicity (Native American and African heritage), being male, and the presence of diffuse cutaneous SSc increase the risk of ILD in SSc patients, the panel said. Among laboratory biomarkers commonly used in clinical practice, the experts agreed that the presence of anti-topoisomerase I antibodies increases the risk of developing ILD, while anticentromere antibodies lower the risk.
The experts recommended that all SSc patients be screened at diagnosis for ILD to provide initial parameters. Initial screening should include HRCT, lung function tests — forced vital capacity (FVC) and diffusing capacity of carbon monoxide (DLCO) — and auscultation.
FVC is a measure of lung function defined as the amount of air a person can forcibly exhale after a deep breath, while DLCO measures lungs’ ability to transfer gas from inhaled air to the bloodstream.
Lung function tests should be repeated regularly — at a frequency defined by the clinician — while HRCT use should be guided by the combination of the patient’s symptoms (such as frequent cough or shortness of breath), lung function, and likelihood of developing ILD.
SSc-ILD diagnosis and severity assessment
The panel agreed that the main tool for diagnosing SSc-ILD is HCRT, with lung function tests (FEV and DLCO) and respiratory symptoms as supporting tools.
ILD severity should be assessed using more than one measure, including HRCT pattern and extent of fibrosis (scarring), lung function tests (FVC and DLCO), shortness of breath, exercise-induced blood oxygen saturation, and quality of life, they said.
SSc-ILD treatment initiation and options
Several factors can drive treatment initiation and assessment of appropriate therapy options for each SSc-ILD patient, including current clinical guidelines, previous clinical experience, patient quality of life, and a therapy’s effectiveness, safety and tolerability, the experts said.
All patients with severe ILD should be offered pharmacological treatment, and patients who are not receiving such treatment should be closely followed and monitored every three to six months. Treatment decisions (initiation, change, or discontinuation) should consider the patient’s current disease state and speed of progression.
Mycophenolate mofetil (brand names CellCept, Myfortic), cyclophosphamide (Cytoxan, Neosar), and Ofev (nintedanib), either as monotherapy or in combination with mycophenolate mofetil, were recommended as effective pharmacologic treatments for SSc-ILD patients, while glucocorticoid single therapy was considered an ineffective option.
SSc-ILD disease progression
Negative changes in lung function (FEV and/or DLCO), in the extent of fibrosis on HRCT, in exercise-induced oxygen saturation, or worsening of clinical symptoms suggest disease progression, the panel said.
While lung function testing was considered an appropriate and effective long-term follow-up measure for assessing SSc-ILD progression, the experts recommended that HRCT usage should be guided by a combination of the patient’s current disease severity and speed of progression.
SSc-ILD treatment escalation
A patient whose disease is progressing or showing an inadequate response to treatment should be considered for treatment escalation (modification of dose or of pharmacological treatment), the experts said.
If mycophenolate mofetil, cyclophosphamide, and nintedanib are not appropriate, the experts agreed that rituximab (brand names include Rituxan and MabThera) could be a therapeutic option. In some subsets of SSc-ILD patients, autologous hemopoietic stem cell transplantation or lung transplantation were considered effective treatments. The panel recommended that patients with advanced SSc-ILD be evaluated early for lung transplant suitability.
“These evidence-based expert consensus statements provide important clinical guidance for the early identification and medical management of systemic sclerosis-associated ILD, and offer a framework for future treatment decision-making,” the researchers wrote.
The modified Delphi study and medical writing support for the manuscript was funded by Boehringer Ingelheim International.
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