Collaboration with pulmonology key to managing ILD in autoimmune disease
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SAN DIEGO — A clear intervention plan and collaboration with pulmonology are key to managing patients with interstitial lung disease in autoimmune conditions, according to a speaker at the Congress of Clinical Rheumatology West.
“There are new therapies we have to offer our patients,” Elizabeth R. Volkmann, MD, MS, scleroderma program director, and co-director of the connective tissue disease-interstitial lung disease program, at the University of California, Los Angeles, told attendees. “There is also more awareness of ILD that I don’t think we had 10 years ago.”
According to Volkmann, pulmonary complications are the leading cause of death in connective tissue diseases. As such, understanding treatment and management strategies is critical to minimizing negative outcomes in ILD, she added.
Specifically, recognizing whether a patient is demonstrating non-specific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP) or lymphoid interstitial pneumonia (LIP) is essential to mapping out a treatment plan. Volkmann encouraged fellow rheumatologists to review the criteria for these conditions and the connective tissue diseases in which they are likely to manifest.
Once this information has been obtained, the first step in treatment is to determine the specific therapeutic needs.
“This can be answered in a few questions,” Volkmann said. “Is the ILD progressing? Does the patient have risk factors for ILD progression? Is the ILD affecting how the patient feels and functions?”
Clinicians should also understand the patient’s risk factors for ILD progression, including disease-specific risk factors associated with their connective tissue condition and the underlying risk factors, like lower baseline forced vital capacity (FVC) and diffusing capacity of the lungs for carbon monoxide (DLCO).
“Identify risk factors so you know if the patient is likely to get worse quickly,” Volkmann said. “Then you know you need to treat them. You really have to consider the individual patient, and their baseline functional status.”
She recommended pulmonary function testing, FVC and DLCO assessment every 3 months, along with CT scan of the lungs once a year.
“The CT can give you insight as to how the disease is evolving,” Volkmann said.
The next step is to identify the initial treatment plan. Understanding the radiographic pattern of ILD is essential in this regard, as is pathology report, according to Volkmann.
“The radiographic pattern can dictate whether anti-inflammatory or anti-fibrotic approach will work,” she said.
Meanwhile, patterns such as NSIP and organizing pneumonia may respond better to an anti-inflammatory approach, Volkmann added, noting that patterns such as LIP have demonstrated more “variable” response to an anti-inflammatory approach.
Collaboration with pulmonology colleagues is encouraged, as is obtaining a clear assessment of extra-pulmonary manifestations that require intervention, according to Volkmann. This collaboration can lead to discussion of glucocorticoid therapy.
“The first question is, 'Do you need to start steroids?’” she said.
If the patient does require steroids, the next task is to plan for tapering them.
“Decide who is going to taper them and delegate who will prescribe the steroid-sparing agents,” Volkmann said.
For managing both ILD and the extra-pulmonary manifestations of the patient’s condition, she recommended aiming for a “unifying treatment approach.”
“You want to aim for one treatment rather than two or three to limit toxicities,” Volkmann said.
As for specific agents, Volkmann noted that mycophenolate can benefit FVC, radiographic fibrosis and skin fibrosis. Meanwhile, rituximab (Rituxan, Genentech) can also benefit FVC and skin fibrosis, and tocilizumab (Actemra, Genentech) has use for FVC and early diffuse systemic sclerosis (SSc). Nintedanib (Ofev, Boehringer Ingelheim) has treatment effects for FVC, SSc-ILD and connective tissue disease-associated progressive pulmonary fibrosis.
“Now we have options,” Volkmann said.
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Volkmann EA. Treatment of autoimmune interstitial lung disease in 2022. Presented at the Congress of Clinical Rheumatology West; Oct. 20-23, 2022 (hybrid meeting).
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