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Breakthrough in diagnosis of idiopathic pulmonary fibrosis

A harmful lung disease could soon be diagnosed without the need for dangerous surgical biopsy procedures, according to new US research.

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive illness that causes huge breathing problems, with massive reduction in oxygen harming vital organs.

There are a calculated 5,000 fresh IPF cases in the UK every year and the condition causes more deaths than either leukaemia, ovarian cancer, or kidney cancer.

Scientists now believe that suspected IPF patients could be spared the significant risks of lung biopsy and handed a diagnosis based solely on clinical and radiological findings alone.

They found that a honeycomb pattern can be detected on the lungs, using a special scanning procedure called HRCT.

This honeycomb pattern is caused by progressive scanning of lung tissue and harm to the lung’s air sacs, known as usual interstitial pneumonia (UIP). This ultimately results in the organ being unable to supply the body with enough oxygen.

The US study findings are published in the journal The Lancet Respiratory Medicine.

There is no cure for IPF and the majority of sufferers only live three to five years after it is detected. Treatment is complicated by the fact that a definitive diagnosis often necessitates a surgical lung biopsy.

In a sufferer with progressive breathlessness who has no substantial environmental exposures attributable to pulmonary fibrosis, or evidence of collagen vascular diseases, the UIP pattern on HRCT is characteristic of IPF.

When patients suspected of having IPF do not have the definitive UIP pattern on HRCT images, worldwide guidelines advise a surgical lung biopsy to make a confident diagnosis of IPF in such patients.

Ganesh Raghu

Ganesh Raghu

Professor Ganesh Raghu, of the University of Washington Medical Centre in Seattle, who headed the study, said: “Surgical lung biopsy is associated with substantial risks and many patients are too elderly, sick, and/or have co-morbid conditions to tolerate the invasive procedure.”

Dr Noel Snell, director of research at the British Lung Foundation, said:“Although IPF affects people of all ages it is more likely to be diagnosed in older people, in whom invasive biopsies can be particularly uncomfortable and may not even be possible.

“This in turn means a timely diagnosis cannot always be made, which is crucial in a disease as progressive as IPF.”

He added: “This study offers hope for less invasive and earlier detection of the condition in the future.

“However, despite the improvements in diagnosis that this research might yield, prognosis for IPF remains poor. Rates of IPF continue to rise, and there is still no known cause or cure for the disease.”

 

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Readers' comments (3)

  • In the UK less than 20% of patients have a surgical lung biopsy to diagnose IPF. A biopsy is only done where there is diagnostic uncertainty from the HRCT. This is different to the US where a biopsy is more commonly done. The BTS guidelines for ILD and IPF diagnosis give a clear outline of the diagnostic criteria that needs to be met to diagnose IPF by HRCT. Every patient where an ILD is suspected should have a HRCT, if this isn't diagnostic then a lung biopsy may be indicated - there is nothing new here.

    Unsuitable or offensive?

  • My father passed away March 2012 with
    I P F and I am raising monies through the BLF I wont to start up a support group in the south east for sufferers and their carers. Would you be able to help me with this.

    Julia Walters

    Unsuitable or offensive?

  • Hi Julia
    It is an objective for the Action for Pulmonary Fibrosis charity to help individuals set up support groups. You can contact them at www.actionforpulmonaryfibrosis.org
    We need more support groups throughout the country.

    Unsuitable or offensive?

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