Inflammatory complications of immunodeficiency disease may benefit from imaging technique

November 30, 2016, British Society For Immunology
FDG PET-CT scan shows pre and three months' post treatment with immunosuppressant therapy in a patient with GLILD. Pre-treatment image shows widespread abnormal uptake of the tracer in the lung parenchyma and lymph nodes both above and below the diaphragm. After treatment, this is no longer the case. Credit: (C) S Jolles, University Hospital of Wales

A new proof of concept study has shown that an imaging technique more commonly used to assess cancer patients may also be of help in assessing disease and treatment effects in patients with inflammatory diseases. The study is published in Clinical & Experimental Immunology.

Common variable immunodeficiency (CVID) is the commonest serious primary immunodeficiency of adults. Patients are not only unable to manufacture antibodies to protect them from infections but also suffer from inflammatory and autoimmune complications. CVID affects approximately 1 in 25,000 adults. While treatment with antibody replacement and antibiotics has significantly improved infections, the non-infectious inflammatory, autoimmune and malignant complications have emerged as major therapeutic challenges. One complication of CVID is called granulomatous lymphocytic (GLILD). This is a complex condition to diagnose and monitor as onset may be insidious leading to lung involvement, enlarged lymph-nodes and spleen. Currently, a combination of clinical features, laboratory markers, lung function measures and radiological investigations are used to diagnose and make treatment decisions about GLILD though there remains a lack of information to help clinicians determine the optimal frequency of testing and timing of treatment.

In this proof of concept study, Dr Stephen Jolles and colleagues from the University Hospital of Wales used an imaging technique called FDG PET-CT to simultaneously assess and metabolic activity in a patient with GLILD. FDG PET-CT imaging allows the detailed anatomical structure obtained by a CT scan to be overlaid and combined with PET images of rates of labelled glucose uptake into cells within the tissues; the clinical utility of which has already been proven in oncological imaging. However, FDG PET-CT's use to assess inflammatory is an emerging field and this paper is the first time that this technique has been employed to assess both anatomical and metabolic extent of the disease and to assess treatment response in the setting of GLILD.

The images (supplied) show that FDG PET-CT provides new insights into GLILD, showing widespread, high levels of metabolic activity not restricted to the lungs but affecting lymph nodes (even when these are normal in size) throughout the body before treatment. After treatment, clear improvements in both anatomical and functional activity can be observed. The combined FDG PET-CT demonstrates that GLILD is only the pulmonary facet of a highly metabolically active multisystem disease. The very high levels of metabolic activity may have implications for the development of autoimmunity and malignancy, both of which occur at higher frequency in patients with GLILD.

PET-CT images of the upper chest pre and three months' post treatment with immunosuppressant therapy in a patient with GLILD are shown. The level of FDG uptake is shown by the intensity of color. Prior to treatment, an enlarged lymph node exhibits intense abnormal FDG uptake. Following treatment, the lymph node at this site normalizes in size and shows no abnormal tracer uptake. Credit: (c) S.Jolles, University Hospital of Wales

Larger studies are now needed to assess the potential role of this technology in the ongoing care of patients with GLILD and it is likely that it will be increasingly used in a wider range of inflammatory disorders.

Lead researcher Dr Stephen Jolles from the University Hospital of Wales said:

"Patients who develop granulomatous lymphocytic interstitial lung disease (GLILD) as a complication of common variable immunodeficiency (CVID) often have poorer outcomes. Given the multi-systemic nature of this disease, it is currently difficult for clinicians to accurately monitor and assess the optimal timing of treatment as well as the effect of treatment and its duration.

This study is the first time that the , FDG PET-CT, has been used to assess the combined lung structure and in a patient with GLILD before and after . The images are striking and enlightening in that they reveal the highly metabolically active multi-systemic nature of the disease. It remains to me amazing the ability of this technique to overlay structure with metabolic functional activity in this way."

Explore further: Immunodeficient patients with secondary lung disease benefit from combined chemotherapy

More information: S. Jolles et al, FDG PET-CT imaging of therapeutic response in granulomatous lymphocytic interstitial lung disease (GLILD) in common variable immunodeficiency (CVID), Clinical & Experimental Immunology (2016). DOI: 10.1111/cei.12856

Related Stories

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 ...

Novel gene variants found in a difficult childhood immune disorder

February 26, 2015
Genomics researchers analyzing a rare, serious immunodeficiency disease in children have discovered links to a gene crucial to the body's defense against infections. The finding may represent an inviting target for drugs ...

IV immunoglobulin use up in interstitial lung disease

August 11, 2016
(HealthDay)—Intravenous immunoglobulin (IVIg) is increasingly being used for interstitial lung disease (ILD), despite a lack of evidence for its use, according to research published online Aug. 2 in the Annals of the American ...

Scientists identify epigenetic basis of common variable immunodeficiency through study of monozygotic twins

June 17, 2015
Researchers of the Chromatin and Disease Group from the Bellvitge Biomedical Research Institute (IDIBELL) and La Paz Hospital (IDIPAZ) have identified epigenetic alterations in Common Variable Immunodeficiency (CVID), the ...

Missing genetic link found in a challenging immune disease

April 22, 2015
In the largest genetic study to date of a challenging immunodeficiency disorder, scientists have identified a gene that may be a "missing link" between overactive and underactive immune activity. The gene candidate also plays ...

Development of autoimmunity in patients with common variable immune deficiency

September 24, 2013
Common variable immune deficiency (CVID) is a genetic disease associated with enhanced susceptibility to infection, autoimmunity, and decreased antibody production. Mutations in the tumor necrosis factor receptor superfamily ...

Recommended for you

Chronic inflammation causes loss of muscle mass during aging

January 12, 2018
People start losing muscle mass at the age of 40—about some 10 percent of the total muscle mass for each 10-year period, which may lead to fall-related injuries, slowing metabolism and reduced quality of life. Today, very ...

Breathing exercises help asthma patients with quality of life

December 13, 2017
A study led by the University of Southampton has found that people who continue to get problems from their asthma, despite receiving standard treatment, experience an improved quality of life when they are taught breathing ...

Study highlights the need for research into prevention of inflammatory bowel disease

December 7, 2017
Countries in Africa, Asia, the Middle East and South America have seen a rise in incidence of inflammatory bowel disease as they have become increasingly industrialised and westernised, a new study has found.

Air pollution can increase asthma risk in adults, even at low levels

November 24, 2017
Living close to a busy road can be bad for your respiratory health if you are middle aged, new Australian research has found.

Evidence found of oral bacteria contributing to bowel disorders

October 20, 2017
(Medical Xpress)—An international team of researchers has found evidence that suggests certain types of oral bacteria may cause or exacerbate bowel disorders. In their paper published in the journal Science, the group describes ...

New compound discovered in fight against inflammatory disease

September 22, 2017
A 10-year study by University of Manchester scientists for a new chemical compound that is able to block a key component in inflammatory illness has ended in success.

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.