International experts have reached consensus on classifying systemic sclerosis associated interstitial lung disease (SSc-ILD) patients in terms of disease severity, risk of progression and progression over time.
The consensus position (link here) was achieved using a proposed conceptual framework for SSC-ILD subsets, as outlined in a paper published in Rheumatology, which was then evaluated by experts (from disciplines including rheumatology, pulmonology and thoracic radiology) in patients.
The findings “will provide guidance to clinicians to improve future management and ultimately help elevate standards of care for patients with systemic sclerosis,” co-author Professor Christopher Denton, a Consultant Rheumatologist at Royal Free London NHS Foundation Trust, told the limbic.
While there had been “substantial” progress in understanding lung fibrosis in systemic sclerosis, it remained a challenging area as severity and rate of progression differed between patients and treatment options were limited and could have side-effects, Prof. Denton explained.
“Physicians need to decide when a patient is at risk of severe or progressive lung fibrosis, and when treatment is most needed..This paper will help to standardise approaches to management in one of the most challenging rheumatic diseases and will help UK rheumatologists and chest physicians by highlighting international expert consensus about the best way to evaluate lung disease,” he said.
The key message is the importance of a proactive approach, with accurate evaluation of disease severity from the outset using baseline lung function tests (LFT) and high resolution computed tomography (HRCT), and a plan for monitoring, co-author Professor Athol Wells, a Consultant Chest Physician at the Royal Brompton Hospital, told the limbic.
“Knowledge of severity, integrating symptoms, lung function tests and estimation of the amount of disease on HRCT, provides crucial information on the risk of progression and often dictates whether treatment should be introduced for the lungs, if not already in place to treat systemic disease,” he said.
And given that SSc-ILD is frequent in SSc, “baseline LFT and HRCT should be obtained at presentation or, at least, in the first year in order to allow the consensus to be applied”.
“Lung function tests are the cornerstone of identifying disease progression and a routine monitoring approach should be put in place, with earlier lung function tests than planned if symptoms suggest progression”.
Frequency of monitoring will be judged case by case, “in part depending on risk evaluation as above, but it is important to recognise that the risk of development and progression of lung disease is highest in the first four years from the onset of SSc”, Prof Wells added.
Professor Denton also emphasised the importance of collaboration between chest physicians and rheumatologists, “which is a cornerstone of good management for systemic sclerosis, and is recommended in all centres across the UK”.
Looking forward, the paper’s authors said their findings form the basis for a multi-dimensional assessment of SScILD (severity, risk of progression, and progression over time), and are “a step towards building classification criteria for these subsets”.
Read more about the consensus framework here