Physicians Hall Front

Study Reveals Potential New Therapy for Interstitial Lung Disease in Patients with Immunodeficiency

Milwaukee, August 3, 2020 – Research published recently in the Journal of Allergy and Clinical Immunology highlights the successful treatment of an interstitial lung disease in children with common variable immunodeficiency (CVID) pioneered by researchers at the Medical College of Wisconsin (MCW) led by John Routes, MD, professor of pediatrics, medicine, microbiology and immunology at MCW and chief of allergy and clinical immunology at Children’s Wisconsin. As Children’s is a national referral center for CVID patients, and in particular those with interstitial lung disease, the work of these local researchers has the potential to change the standard practice on how this disease is treated.

CVID is an antibody deficiency that leaves the immune system unable to defend itself against bacteria and viruses, resulting in severe ear, sinus and respiratory infections. CVID can occur at any time in life, but in the U.S., the most common age of onset is in the second decade of life. Patients who have been diagnosed with CVID are also at risk of contracting a life threatening complication, granulomatous and lymphocytic interstitial lung disease (GLILD). GLILD occurs in approximately 20-30 percent of CVID patients and is one of the most common causes of death, yet there has not been an established standard of care for the treatment of this disorder. In the past steroids were used, but they have been proven ineffective.

Dr. Routes and his team of researchers set out to determine if an immunosuppressive regimen of the drugs rituximab, azathioprine or mycophenolate mofetil could improve high-resolution CT scans and pulmonary function in patients with CVID and GLILD. The results of this study of 39 patients show that immunosuppressive therapy is an effective treatment for GLILD patients. The regimen improved the radiographic abnormalities and pulmonary function of the patients, with a majority of them experiencing sustained remissions.

Dr. Routes is hopeful that this study will change the standard practice on how the disease is treated. “There is a widespread lack of understanding of CVID and GLILD,” he said. “Our results suggest that combination immunosuppression should be considered as first-line therapy in the treatment of GLILD.”

Because it is now known that a CVID-like immunodeficiency can result from mutations in genes involved in immunity, the researchers also conducted whole exome sequencing on all the patients. As a result, they found mutations in more than 20 percent of the patients, but the immunosuppressive treatment was equally effective in patients with or without a damaging mutation.

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