Immunodeficient patients with secondary lung disease benefit from combined chemotherapy

August 30, 2012

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 (GLILD). In GLILD, white blood cells abnormally accumulate in the lung, which leads to a progressive decline in lung function and, eventually, . 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 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, 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.

Explore further: For puzzling childhood immune disorder, gene research opens door to first diagnostic test

Related Stories

For puzzling childhood immune disorder, gene research opens door to first diagnostic test

May 11, 2011
A new genomics study, led by the Center for Applied Genomics at The Children's Hospital of Philadelphia, sets the stage for the first predictive diagnostic test in a serious immunodeficiency disease in children. If the disorder, ...

Study finds treatment for rare lung disease

April 11, 2011
An Oregon Health & Science University researcher has co-authored an international study that revealed a drug approved to prevent rejection in organ transplant patients helped treat a rare lung disease in women.

Recommended for you

Drug increases speed, safety of treatment for multiple food allergies

December 11, 2017
In a randomized, controlled phase-2 clinical trial, an asthma medication increased the speed and safety of a protocol used to treat children for several food allergies at once, according to a study by researchers at the Stanford ...

Immunotherapy drug nearly eliminates severe acute graft-versus-host disease

December 9, 2017
Results from a phase 2 clinical trial, presented by Seattle Children's Research Institute at the 59th American Society of Hematology (ASH) Annual Meeting, show that the drug Abatacept (Orencia) nearly eliminated life-threatening ...

Location, location, location: Immunization delivery site matters

December 1, 2017
In vaccination, a certain subpopulation of dendritic cells is vital to triggering the body's adaptive immune system, report researchers at The Jackson Laboratory (JAX), Yale University and Astra-Zeneca.

An anti-aging protein could be targeted to rejuvenate immune cells

November 30, 2017
Anti-aging proteins have long been shown to protect against age-related diseases, such as cancer, neurodegeneration, and cardiovascular disease. A study by researchers at the Gladstone Institutes now reveals that one such ...

Scientists find key to miscarriages in blood clotting disorder

November 28, 2017
Monash University researchers have potentially shed light on why women with the rare autoimmune disorder Antiphospholipid syndrome (APS) are prone to successive pregnancy losses.

How do cells release IL-1? After three decades, now we know

November 28, 2017
Researchers at Boston Children's Hospital have identified, for the first time, the molecule that enables immune cells to release interleukin-1 (IL-1), a key part of our innate immune response to infections. Findings were ...

0 comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.