High-Res CT Has Key Role Detecting Lung Disease in Systemic Sclerosis

By Marilynn Larkin

February 04, 2020

NEW YORK (Reuters Health) - The first evidence-based consensus statements for systemic sclerosis-associated interstitial lung disease (SSc-ILD) recommend use of high-resolution computed tomography (CT) for screening and diagnosis, and offer guidance on when to initiate or escalate emerging treatment options.

"We believe that the evidence-based expert consensus statements provide important clinical guidance for the early identification and medical management of SSc-ILD, and offer a framework for future treatment decision making," Dr. Anna-Maria Hoffmann-Vold of Oslo University Hospital, Norway, told Reuters Health by email.

The most important take-home messages for clinicians taking care of SSc-ILD patients, according to Dr. Hoffmann-Vold, are the following:

- All patients with systemic sclerosis should be screened for SSc-ILD using high-resolution CT.

- High-resolution CT is the primary tool for diagnosing ILD in systemic sclerosis.

- Pulmonary function tests and clinical assessment of respiratory symptoms support screening and diagnosis.

- SSc-ILD severity should be measured with more than one indicator; dyspnea (with or without the 6-minute walk test) should be considered, as well as exercise-induced oxygen desaturation and quality of life.

- It is appropriate to treat all severe cases; several first-line treatment options have been suggested.

- For some patients, such as those who have mild disease and/or are stable, medication may not be needed. However, these patients should be followed closely and treatment initiated in case of progression.

- Follow-up assessments enable identification of disease progression, which was defined as changes in pulmonary function tests (forced vital capacity and diffusing capacity for carbon monoxide absolute values or FVC decline); changes in extent of fibrosis or pattern on high-resolution CT; changes in exercise-induced oxygen desaturation; and worsening of clinical symptoms.

- Progression pace, alongside disease severity, drives decisions to escalate treatment.

A panel of 27 Europe-based pulmonologists, rheumatologists, and internists with expertise in SSc-ILD participated in three rounds of online surveys, a face-to-face discussion, and a WebEx meeting, followed by two supplemental Delphi rounds, to establish consensus and define a management algorithm, according to the report published in The Lancet Rheumatology.

Overall, consensus was considered achieved if at least 80% of panelists indicated agreement or disagreement.

Between 2018 - 2019, consensus agreement was reached for 52 primary statements and six supplemental statements across six domains of management, and an algorithm was defined for clinical practice use.

"To date, there (had) been no single set of evidence-based guidelines on how to investigate (SSC-ILD), who to treat and when," Dr. Susanna Proudman of Royal Adelaide Hospital in Australia, author of a related editorial, commented in an email to Reuters Health.

The new statements, she noted, "serve as general principles for use in the clinic, but lack sufficient detail in some instances, mostly reflecting equipoise or lack of evidence at the time the systematic literature review that underpinned the selection of statements was performed."

"Examples include how to apply risk factors in the individual patient; cutoffs for changes in pulmonary function parameters such as forced vital capacity to define progression; how frequently to repeat screening tests and tests for progression; and the results of clinical trials for therapies such as rituximab," she noted.

"Other statements are likely to change clinical practice, such as the use of high-resolution CT scanning of the chest to screen for ILD at baseline, which has not been standard of care," she said. "Nonetheless, these statements form a framework for future investigations to address areas of uncertainty, including the promise of more sophisticated modeling of the individual patient's disease trajectory using novel biomarkers and detailed phenotypic data."

The study was funded by Boehringer Ingelheim International. Dr. Hoffmann-Vold and many coauthors have received fees from the company. Dr. Proudman was a principal investigator on a clinical trial funded by the company.

SOURCE: http://bit.ly/36K1kxw and http://bit.ly/2RKKgTx The Lancet Rheumatology, online January 14, 2020.

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