Rare diseases

First framework for managing connective tissue disease-ILD


The world’s first comprehensive guidance on connective tissue disease-associated interstitial lung disease (CTD-ILD) has been developed by an Australian multidisciplinary expert group including two rheumatologists.

A 29-page position statement published in Respirology, includes diagnosis of CTD-ILD and the important role of the multidisciplinary meeting in management, as well as general principles to monitoring and treatment CTD-ILD and advice on several specific CTD-ILD.

These include systemic sclerosis, rheumatoid arthritis, idiopathic inflammatory myopathy (IIM), SLE and Sjogren’s disease.

Undifferentiated and mixed connective tissue disease as well as patients with ILD and some features of autoimmune disease but not meeting criteria for a defined CTD, further demonstrated the complexity of the CTD-ILD.

It also includes mention of vasculitis and ILD, and psoriasis and ILD.

The statement said treatment strategies were largely drawn from studies in SSc-ILD and extrapolated to the other CTD-ILD.

“Whilst reasonable in the absence of specific evidence, caution is required, especially in more progressive CTD-ILD, such as IIM-ILD,” it advised.

“In clinical practice, broad CTD-ILD treatment principles remain pertinent with therapeutic intervention based on ILD severity and rate of progression, other organ involvement and comorbidities.”

The statement said immunosuppression was the mainstay of treatment for severe and/or progressive CTD-ILD, however “when to start therapy, which agent to use and duration of therapy is a nuanced decision impacted by many clinical and patient specific factors”.

“An ever-expanding array of treatment options, including monoclonal antibodies targeting specific molecular pathways and the antifibrotic agents, are being evaluated with promising early results and are providing new insights into disease pathophysiology.”

CTD was not a relative or absolute contraindication to lung transplantation, but CTD-ILD only accounts for about 2% of procedures worldwide.

Non-pharmacological therapies including pulmonary rehabilitation and supplemental oxygen were often overlooked, the statement noted.

Multidisciplinary Expert Panel

The TSANZ statement was developed by a 26-member expert panel including respiratory physicians, rheumatologists, an immunologist, pathologist, radiologist, allied health, respiratory nurse and patient representative.

Panel chair Dr Gregory Keir, a Brisbane respiratory physician, told the limbic that the CTD-ILDs were a complicated group of conditions.

“There really isn’t a similar document available anywhere to the best of our knowledge so we just thought we would pull together a broad principles summary to give clinicians some guidance in making the diagnosis and an approach to treatment,” he said.

“Certain patients do follow general rules – and that’s what we have tried to lay out – but again we have also tried to highlight the importance of assessing every person with one of these conditions individually, and watching them closely because things can change. What you expect may not be what happens in reality.”

Dr Keir, who also convenes the TSANZ Orphan Lung Diseases, Lung Transplant, Interstitial Lung Disease and Pulmonary Vascular Disease SIG, said good quality evidence for treatment of CTD-ILDs was rare which in turn made access to medicines difficult.

“The two antifibrotics that are available on the PBS – pirfenidone and nintedanib – are only available for IPF. We think that they may work in some of the CTDs. There have been some studies that support that. But at the moment they are not approved for that indication. We have mentioned them, as a hope for the future, but we are not there yet.”

“Rituximab isn’t PBS-funded for these indications but often getting hospital-funded access or compassionate access is an option for treatment. Hopefully this document provides a bit of evidence to support the individual applications when doctors need to make them.”

He said a close working relationship between the specialties was critical to good patient care in CTD-ILD.

“What we choose for the lungs or what the rheumatologist chooses for the joints or muscles… there is always some overlap so we just need to make sure we are treating the entire person not just one organ system. The closer the collaboration, certainly in my experience, the easier it is to manage these complicated patients.”

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