A team of researchers at the Medical College of Wisconsin and Children's Hospital of Wisconsin Research Institute defined a new treatment for a potentially fatal lung disease in patients with a primary immunodeficiency known as common variable immunodeficiency (CVID).The findings are published in the Journal of Clinical Immunology.
Common variable immunodeficiency (CVID) is the most common primary immunodeficiency that requires regular treatment with medication, specifically immunoglobulin (antibodies) replacement therapy. With immunoglobulin therapy, deaths from infection in patients with CVID have decreased and deaths due to non-infectious complications have increased. One of the most frequent causes of death now in CVID is a lung disease known as granulomatous-lymphocytic interstitial lung disease (GLILD). In GLILD, white blood cells abnormally accumulate in the lung, which leads to a progressive decline in lung function and, eventually, respiratory failure. The researchers evaluated patients with GLILD to see if rituximab and azathioprine (combination chemotherapy) would improve pulmonary function and/or radiographic abnormalities by killing these white blood cells in the lung.
"The most common medication used for GLILD are corticosteroids but in our hands this type of medication did not clear the disease. Therefore, we designed therapy aimed at killing the specific type of lymphocytes, B cells and T cells, we found in abnormal numbers in the lung biopsies we examined. We reasoned if we killed the lymphocytes in the lung, lung function and radiographic abnormalities would both improve, which is what we found." said John M. Routes, M.D., professor and chief of asthma, allergy and immunology at the Medical College of Wisconsin (MCW), medical director of allergy/clinical immunology at Children's Hospital and researcher at the Research Institute.
Other authors of the paper include Nicole M. Chase, M.D.; James W. Verbsky, M.D., Ph.D.; Mary K. Hintermeyer, A.P.N.P.; Jill K. Waukau, B.S.; Aoy Tomita-Mitchell, Ph.D.; James T. Casper, M.D.; Sumit Singh, M.D.; Kaushik S. Shahir, M.D.; William B. Tisol, M.D.; Melodee L. Nugent, M.A.; R. Nagarjun Rao, M.D.; A. Craig Mackinnon, M.D., Ph.D.; Lawrence R. Goodman, M.D.; and Pippa M. Simpson, M.D.; MCW, Children's Hospital and the Research Institute.
About the Medical College of Wisconsin
The Medical College of Wisconsin is the state's only private medical school and health sciences graduate school. Founded in 1893, it is dedicated to leadership and excellence in education, patient care, research and service. More than 1,200 students are enrolled in the Medical College's medical school and graduate school programs. A major national research center, it is the largest research institution in the Milwaukee metro area and second largest in Wisconsin. In FY 2010 – 11, faculty received more than $175 million in external support for research, teaching, training and related purposes, of which more than $161 million is for research. This total includes highly competitive research and training awards from the National Institutes of Health (NIH). Annually, College faculty direct or collaborate on more than 2,200 research studies, including clinical trials. Additionally, more than 1,350 physicians provide care in virtually every specialty of medicine for more than 400,000 patients annually.
About Children's Hospital of Wisconsin
Children's Hospital of Wisconsin is the region's only independent health care system dedicated solely to the health and well-being of children. The hospital, with locations in Milwaukee and Neenah, Wis. is recognized as one of the leading pediatric health care centers in the United States. Children's Hospital provides primary care, specialty care, urgent care, emergency care, community health services, foster and adoption services, child and family counseling, child advocacy services and family resource centers. In 2010, Children's Hospital invested more than $105 million in the community to improve the health status of children through medical care, advocacy, education and pediatric medical research. Children's Hospital achieves its mission in part through donations from individuals, corporations and foundations and is proud to be a Children's Miracle Network Hospital. For more information, visit the website at chw.org.
Journal
Journal of Clinical Immunology