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Deadly ‘bagpipe lung’ case brings new attention to old disease

Hypersensitivity pneumonitis is a disease of the lungs in which your lungs become inflamed as an allergic reaction to inhaled dust, fungus, molds or chemicals.

If you’re a musician who plays a wind instrument, such as a saxophone, a trombone or the bagpipes — be sure to clean your instrument regularly and thoroughly.

Otherwise, you risk developing an inflammatory lung disease known as hypersensitivity pneumonitis (HP).

That’s the message of a case study published this week in the journal Thorax.  It describes how a 61-year-old bagpiper died in 2014 of a particularly persistent case of HP, which was caused by fungi growing in his bagpipes.

The man might have survived if the source of his fungal infection — the instrument he played daily — had been identified earlier.

Many names

As the American Lung Association notes, HP “is a disease of the lungs in which your lungs become inflamed as an allergic reaction to inhaled dust, fungus, molds or chemicals.”

Initial symptoms are flu-like: a dry cough, shortness of breath, tightness in the chest, fever, chills and fatigue. The disease is treatable, although it requires complete avoidance of the source of allergen. Prescription steroids may also be needed. In tenacious cases, immunosuppressive drugs are sometimes prescribed.

The illness has many names, depending on the source of the inhaled allergen. “Farmer’s lung,” for example, is caused by mold on hay and grain; “bird fancier’s lung,” by particles on feathers or by bird droppings; “humidifier lung,” by a fungus that grows in humidifiers or air conditioners; and “hot tub lung,” by bacteria in the tub’s water vapor.

Now the illness has another name: “bagpipe lung.”

A long illness

The patient at the center of the new Thorax case study was referred to an interstitial lung disease clinic at the University Hospital of South Manchester in the United Kingdom in April 2014. He had a seven-year history of dry cough and progressive breathlessness. Five years earlier, he had been diagnosed with HP, but treatment with immunosuppressive drugs had failed to help.

Doctors were puzzled about the source of the man’s HP.

“He did not have any exposure to birds or pigeons. His house showed no evidence of mould or water damage, and he had no symptoms of connective tissue disease,” write Dr. Jenny King and her colleagues at the University Hospital of South Manchester. “The precipitating trigger for his HP was unknown.”

The only symptom relief the man experienced was during a three-month visit to Australia in 2011.

In September 2014, the man’s condition had worsened considerably, and he was admitted to the hospital. He was breathing rapidly and found to be hypoxic (a condition marked by insufficient amounts of oxygen in the blood).

It seems that during this hospital admission someone thought to take samples from the man’s bagpipes, an instrument he had been playing daily, even throughout the years he had been diagnosed with HP.

Significantly, he had not taken his bagpipes to Australia.

The samples revealed the presence of a microscopic hothouse of fungi, “including Paecilomyces variotti, Fusarium oxysporum, Penicillium species, Rhodotorula mucilaginosa, Trichosporon mucoides, pink yeast and Exophiala dermatitidis,” write the researchers.

But it was too late for the bagpipe player. His condition continued to deteriorate, and he died on Oct. 10, 2014.

Advice to musicians

“This is the first case report identifying fungal exposure, from a bagpipe player, as a potential trigger for the development of HP,” King and her colleagues write. “The clinical history of daily bagpipe-playing coupled with marked symptomatic improvement when this exposure was removed and the identification of multiple potential precipitating antigens isolated from the bagpipes make this the likely cause.”

Other cases of musicians developing HP from their instruments have been reported in the medical literature, including two relatively recent cases involving a trombone player and a saxophone player.

The British bagpiper’s case represents, however, the first known one in which a musician died from instrument-contracted HP.

After his diagnosis, the trombone player “began to regularly clean his trombone with 91% isopropyl alcohol, and his symptoms of cough and breathless resolved,” King and her colleagues note. The saxophone player recovered with the help of steroid treatment and by regularly drying his instrument after use and cleaning it with a disinfectant.

“Wind instrument players need to be aware of the importance of regularly cleaning their instruments and of potential risks,” write the researchers.

“Physicians should be aware of this potential risk factor and promote wind instrument hygiene,” they add.

FMI: You can read the case study in full on the Thorax website.

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