Lots of really good and/or interesting things feature the letter T and a hyphen—including, if you have pulmonary fibrosis (PF), rare T-cells.
Pulmonary fibrosis literally means “lung scarring.” It is an umbrella term for a family of diseases called “interstitial lung diseases.” Normally, our lungs are pliant, appropriately lubricated organs that function to transfer oxygen into the blood that flows through teeny air sacs called alveoli. In PF, the walls between those air sacs (the interstitium) become so stiff with thickened scarring that oxygen can’t pass through them into your bloodstream.
According to the American Lung Association:
- About 140,000 Americans have been diagnosed with PF
- PF most commonly affects people ages 50 – 75
- There’s no cure
- Symptoms include:
- shortness of breath
- dry cough
- unexplained weight loss
- widening and rounding of fingertips and toes (clubbing)
- muscle and joint aches
- Causes may include:
- long-term environmental exposure to (in alpha order) asbestos, bird and animal droppings, cigarette smoke, coal dust, grain or metal dusts, and/or silica dust;
- radiation treatment from lung or breast cancer;
- some medications;
- certain medical conditions (dermatomyositis, mixed connective tissue disease, pneumonia, polymyositis, rheumatoid arthritis, sarcoidosis, scleroderma, systemic lupus erythematosus)
PF with no known cause is referred to as idiopathic pulmonary fibrosis (IPF).
What’s so T-T-Terrific?
Because pulmonary fibrosis is progressive and has such serious consequences, researchers are working hard to find a cure. Currently, there are a few treatments that help patients manage the symptoms of their disease, but no treatments that modify its course.
But recently some researchers working with mice made a serendipitous discovery: a rare immune T-cell that behaved unexpectedly.
Now, in case you don’t know, T-cells and helper T-cells are white blood cells produced in the thymus—that’s what the “T” indicates. They play an important role in the human immune system. Another term you need to know is cytokine. Cytokines are proteins involved in communicating information between your body’s cells. Some cytokines encourage inflammation and others are anti-inflammatory.
Scientists already knew that the pro-inflammatory cytokine called IL-17A was a key factor in developing pulmonary fibrosis. They also knew that IL-17A was produced by T helper 17 cells (Th17). So, they thought that mice who didn’t have Th17 wouldn’t develop pulmonary fibrosis after being exposed to a substance known to cause the disease.
Surprise! Surprise! Surprise!
Instead, the researchers discovered a surprisingly vigorous production of rare T-cells called gamma delta T-cells working overtime to create fibrosis in those poor little mice.
It was a real “A-ha!” moment that may have wonderful implications for people who have PF. Researchers now have another target to aim at when developing immunotherapy treatments for the disease!
Science is so cool.
If you want to keep following the researchers’ progress on this front, you’ll find an article here that gives you more information.