Prof. Tamera Corte
Most patients with connective tissue disease should be screened for interstitial lung disease, according to the first ERS/EULAR clinical practice guidelines for CTD-ILD.
The guidelines also stress that clinicians should consider patient-specific risks, extrapulmonary organ manifestations, potential side-effects and the risk of progressive or severe ILD when determining choice of treatment.
Treatment recommendations are “comprehensive and summarised per disease” and also include key factors for choice of treatment in the newly provided treatment algorithms, the authors noted, in a paper published in Annals of the Rheumatic Diseases [link here].
The expert group, which included pulmonologists and rheumatologists, recommended ILD screening for all patients with systemic sclerosis (SSc), mixed connective tissue disease (MCTD) and idiopathic inflammatory myopathies (IIM) with risk factors using high-resolution computer tomography (HRCT) at the time of diagnosis.
Most patients with IIM without risk factors, except for inclusion body myositis, as well as patients with rheumatoid arthritis (RA) and Sjögren Disease (SjD) who have risk factors for ILD should be screened for the condition using HRCT, the guidelines say.
Where there are potential alternative diagnoses or co-morbidities, the task force recommended bronchoalveolar lavage (BAL) along with other assessments to rule out differential diagnoses, although they noted that it is not generally used to diagnose ILD in CTDs.
At the time of an ILD diagnosis, all patients should have an assessment of disease severity and risk of progression, using pulmonary function tests, HRCT, the 6 minute walk test and patient-reported outcome measures (PROMs).
“PROMS can provide valuable insights into the patient’s perspective, capturing respiratory symptoms in a validated and standardised way, and assessing the impact of the disease on daily life and patient well-being, which can enhance the overall assessment of disease severity and treatment effectiveness,” the authors noted.
Clinicians should monitor CTD-ILD patients by conducting a comprehensive evaluation at each visit to identify those at high or low risk for poor prognosis, ILD progression and disease severity, the guidelines stress.
They also recommend following high-risk patients with lung function tests every three to six months early in the disease course and every six to 12 months after that point.
In addition, a control HRCT should be conducted routinely after 12 months and annually thereafter, if clinically necessary, according to the task force, which included Professor Tamera Corte, Director of Interstitial Lung Disease, Department of Respiratory Medicine, Royal Prince Alfred Hospital, Sydney.
In patients without physical limitations and PROMs, the guidelines suggest that 6 minute walk tests be conducted every six to 12 months.
Early and aggressive treatment
The guidelines recommend early and/or aggressive treatment for patients at risk of progressive or severe ILD, especially those with multiple risk factors or specific indicators such as anti-MDA-5 autoantibodies.
For patients with SSc-ILD, use of MMF, rituximab and cyclophosphamide was backed by the expert group.
The guidelines also advise that tocilizumab should be used for SSc-ILD patients with increased inflammatory markers or recent skin fibrosis progression.
The treatment algorithms also conditionally recommend nintedanib or combinations of nintedanib and MMF for SSc-ILD patients, and the use of immunosuppressive treatment plus pirfenidone for RA-ILD patients displaying a UIP pattern.
Immunosuppressive treatment is advised for patients with IIM-ILD, with combination therapy of immunosuppressants and glucocorticoids conditionally recommended.
It was also conditionally recommended for other CTD-ILDs, while nintedanib and combination therapy was conditionally backed for patients with pulmonary fibrosis.
“In addition to pharmacological therapy, we encourage adhering to non-pharmacological treatment and existing specific rheumatic disease recommendations in addition to our ILD-specific guideline to guarantee an optimised and holistic disease management,” the guideline authors noted.
The authors acknowledged that the guideline “predominantly builds on evidence of low and very low certainty,” and that this is a common challenge for rare diseases with small patient populations and a scarcity of randomised clinical trials.
They also stressed that the guidelines were developed specifically for adult patients, and that “some aspects may be different in childhood populations”.