Vigilance for Velcro crackles needed to avoid diagnostic delay in ILD

Respiratory physicians have called for doctors to do better in diagnosing ILD at an earlier stage – by bringing out the stethoscope to listen for Velcro-type crackles.

Writing in an editorial in Respirology, Dr Margaret Wilsher and Professor Lutz Beckert of Auckland University said delays in diagnosis of ILD meant patients had lost physiological reserve before they started on disease-modifying therapy.

“That is never to be recovered and nor is their functional performance, and possibly their economic prospects,” they said.

“For some years, the disease has been lurking in the posterior and basal recesses of the lungs, indolently fibrosing the delicate alveolar architecture yet without silence or radiographic obscurity – if only we had listened and looked carefully.”

The physicians were responding to a review of medical records from more than 2,000 patients with pulmonary fibrosis in the UK’s Optimum Patient Care Research Database.

The study, based in primary care, showed there was increasing healthcare resource utilisation over the 10 years prior to diagnosis of pulmonary fibrosis – effectively many missed opportunities for an earlier diagnosis.

Five years before diagnosis about 18% of patients had multiple healthcare visits for respiratory complaints which increased dramatically to 79% in the year before diagnosis. They found 38% of patients had five or more healthcare visits for lower respiratory tract symptoms.

The editorial said earlier identification and especially earlier referral to a specialist ILD centre would provide an opportunity to potentially alter the disease trajectory and improve patients’ quality of life.

“Despite a reported increase in the incidence of IPF, it is not always included in the initial differential diagnosis of new dyspnoea and cough, and delays or missed opportunities occur at every step of the diagnostic pathway: access to specialist investigations, failure to report ILD on incidental or diagnostic computed tomography (CT), referral to respiratory physician and access to funded anti-fibrotic medication.”

“We can do better than this. First, we can bring out the stethoscope! Velcro-like crackles are evident in early ILD, are heard before chest X-ray abnormalities become apparent and correlate with usual interstitial pneumonia pattern and extent of fibrosis on CT.”

“They are not specific but relatively uncommon in healthy older people and subtly different to the crackles heard in heart failure and pneumonia which are more easily diagnosed in the appropriate clinical setting.”

“Funding entities should consider the greater utility of CT scanning in detecting early fILD and diagnostic pathways should ensure thoracic radiologist interpretation,” they added.

“As lung cancer CT screening becomes more pervasive, and with that the detection of patients with fILD or interstitial-like abnormalities (ILA), then there is an opportunity to potentially identify patients at risk.”

The authors also called for increased awareness of ILD in primary care, amongst community and hospital specialists and relevant professional bodies, so that patients are referred in a timely way.

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