Interferon-γ May Be the Cause of CF With Co-existing Endometriosis in Female Patients

New research is shedding light on a possible link between endometriosis in females who have cystic fibrosis.

Cystic fibrosis (CF) is a genetic disorder caused by mutations to the cystic fibrosis transmembrane conductance regulator (CFTR) gene that mostly affects the lungs, but also the pancreas, liver, kidneys and intestine. The disease’s morbidity and mortality is mainly caused by repetitive infections in the lungs, often related to the virulent pathogen Bulkholderia cenocepacia. CF with co-existing endometriosis is a rare but possible association.

It is estimated that endometriosis affects 10 percent of reproductive-age women and happens when endometrium (inner lining of the uterus) cells that normally grow inside the uterus begin to grow outside the uterus, leading mainly to infertility and pelvic pain, usually during menstruation. The pain is caused by the inflammation associated with the cyclic detachment of cells from the wrong site (pelvic cavity, for example) during menstruation.

Nevertheless, pathophysiology of both diseases is poorly understood. In both diseases, interferon-γ (INF-γ), an important activator of the immune response produced by leukocytes, seems to be upregulated. Not only that, but the p38 mitogen activated protein kinase (p38 MAPK) pathway was also previously shown to participate in the pathogenesis of both diseases and to be induced by INF-γ.

A team of researchers from Italy led by Dr. Raffaella Mormile, are hypothesizing that women with endometriosis may be more seriously affected by cystic fibrosis lung disease. The greater production of INF-γ and consequent activation of p38 MAPK pathway may lead to a higher frequency of pulmonary infections and a more rapid rate of lung damage. The worse outcome of patients afflicted with both CF and endometriosis is compelling researchers to address them with novel therapeutic strategies.

Investigators aim to find out if the usage of inhibitors of the p38 MAPK pathway or antibodies against INF-γ might be an effective alternative treatment for women affected with both conditions. As such, they reiterate that clinical trials are needed in order to study this therapeutic proposal.

Glossary of Terms:

[wikibox lang=”en”]Interferon gamma[/wikibox]

[wikibox lang=”en”]P38 mitogen-activated protein kinases[/wikibox]