SAN DIEGO — A recent FDA approval for systemic sclerosis-associated interstitial lung diseases may portend a new hope for clinicians managing these complicated disorders, according to a presentation at the 2019 Congress of Clinical Rheumatology West.
Dinesh Khanna, MBBS, MSc, professor of rheumatology and internal medicine at the University of Michigan, described the approval of nintedanib (Ofev, Boehringer Ingelheim) as “very exciting news” for physicians. During his session, Khanna provided an overview of diagnosis and treatment for various forms of ILD within the context of this newly available medication.
“The spectrum of ILD varies depending on the underlying connective tissue disease,” Khanna said. He added that the histologic pattern seen in the lung biopsy can determine the radiologic features of the disease, influence clinical presentation and predict response to therapy and prognosis.
Khanna cautioned that 15% of idiopathic pulmonary fibrosis (IPF) is ultimately diagnosed as CTD-ILD. “The good news is that CTD-ILD confers better survival than IPF,” he said.
Correctly interpreting a pulmonary function test is critical to making a correct diagnosis, according to Khanna. Rheumatologists should understand how to interpret the ratio of forced expiratory volume in one second (FEV1) to forced vital capacity (FVC), total lung capacity, and diffusion capacity.
Khanna also explored some common combinations of ILD that should be on the radar of every clinician. Systemic sclerosis-associated ILD may affect about 80% of patients. “This disease is largely irreversible,” he said. “The goal is stabilization. Early intervention is critical.”
Systemic sclerosis-associated ILD requires long-term immunosuppressive therapy. “Disease may flare once you take away immunosuppressive therapy,” Khanna said.
Rheumatoid arthritis-associated ILD occurs in 20%-30% of patients, many of them older men with a smoking history, high RF, active joint disease, high disease activity, or other extra-articular manifestations. “In up to 20% of these patients, lung disease may precede joint disease,” he said.
Inflammatory myopathy and ILD occurs more often in women, according to Khanna. He suggested that 7% have myositis before they develop ILD, while 60% have concomitant myositis. “However, a few of them are amyopathic,” he said. “It becomes hard for a rheumatologist to make a diagnosis.”
Regarding treatment for these patients, beyond nintedanib, Khanna was realistic. “I do not have any recommendations or guidelines,” he said. “We throw everything at them.”
Rituximab (Rituxan, Genentech) is a good place to start, according to Khanna. Other treatments may include cyclophosphamide, abatacept (Orencia, Bristol-Myers Squibb), pirfenidone (Esbriet, Genentech), mycophenolate mofetil, or steroids.
“For severe progressive ILD or non-responsive disease, autologous HSCT or lung transplant are options,” he said. “But I believe rituximab will become one of the early treatments for early scleroderma ILD.” —by Rob Volansky
Khanna D. Interstitial Lung Disease in Connective Tissue Disorders. Presented at: Congress of Clinical Rheumatology West. September 26-29, 2019; San Diego.
Disclosure: Khanna reports his role as coordinating primary investigator for ongoing and recently completed industry-funded trials of abatacept, brentuximab, pirfenidone, riociguat, tofacitinib and tocilizumab in scleroderma.