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Diagnosing and treating ILD

By Mindo - 10th May 2016 | 9 views

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ung disease causes one-in-five deaths in Ireland, according to figures compiled by the Irish Thoracic Society. It is also the third-most common cause of emergency hospital admissions, according to the <em>European Lung White Book</em>, produced by the European Respiratory Society (ERS). The highest death rates for interstitial lung diseases (ILD), of more than 2.5 per 100,000, are seen in Ireland, the UK, Scandinavia, the Netherlands and Spain, according to the ERS.

To address the Irish context, a recent Interstitial Lung Disease Study Day, supported by Roche, featured a wide-ranging programme with a breadth of speakers imparting expertise in their respective fields.

The packed meeting — attended by respiratory nurse specialists, specialist physiotherapists, respiratory scientists and secondary care experts — first heard from Dr Kate O’Reilly, Consultant in Respiratory Medicine, Mater Hospital, Dublin. Dr O’Reilly delivered a talk titled ‘ILD Overview: Classification and Diagnosis’. Dr O’Reilly gave a “sense of the scope of ILDs”, explaining that many of the talks at the study day would address a range of ILDs.

“GP education is vital in ILD and idiopathic pulmonary fibrosis (IPF),” she told the attendees. “IPF and sarcoidosis are the most common ILDs. We do not know what causes IPF, but we suspect that IPF is under-recognised and under-diagnosed. The median survival in IPF has historically been  two-to-five years after diagnosis and this is similar to lung cancer.”

As the fibrosis advances, the lung becomes smaller and heavier and a lung with advanced fibrosis can weigh four times more than a normal lung, Dr O’Reilly explained.

While the presenting symptoms of IPF may be non-specific, dyspnoea and cough are very commonly seen, Dr O’Reilly explained. “It’s important to do a good systems review and enquire about the use of antibiotics and/or illicit drugs, e-cigarettes and whether they work in an environment with asbestos.” She pointed out that exposure to environmental antigens such as pigeon droppings can cause another form of ILD called hypersensivity pneumonitis.

<h3 class=”subheadMIstyles”>‘Honeycombing’</h3>

She also presented examples of CT scans which showed a ‘honeycombing’ of the lungs associated with ILD and provided an overview of how the disease should be addressed in a multidisciplinary team setting.

“If the radiologist reports a specific [UIP] pattern in the right clinical setting, we can diagnose IPF without a lung biopsy,” she told the meeting.

Dr O’Reilly concluded with case studies to illustrate the complexities of ILD. She drew attention to the CT scan of a 43-year-old patient who had manifestations of rheumatological disease, including ‘mechanic’s hands’ (a term used to signify cracking and splintering of the skin on the fingers). The CT scan of the patient’s lungs showed widespread abnormalities.

“I will finish ‘on a high’ because this patient had a lung transplant in late 2014,” Dr O’Reilly told the meeting, explaining that the patient had antisynthitase syndrome — a rare syndrome associated with ILD, which sometimes mimics pneumonia — and double lung transplantation was deemed the best therapeutic option. “He still needs immunosuppression because he still has the antisynthetase and the myositis, but he’s doing very well from a respiratory point of view.”

Dr Emmet McGrath, Consultant in Respiratory Medicine, St Vincent’s Hospital, Dublin, presented on the topic, ‘Current ILD Treatment Options and the Rationale for Early Treatment’. “For a long time, there were no drug advancements but now there have been some developments,” he explained. “Half of IPF patients die within three-to-five years of diagnosis so we are very passionate about trying to improve patient outcomes.”

It is vital to narrow-down the type of ILD a patient has because both the treatment and outcome can be massively different, he told the attendees. The work of the multidisciplinary team is vital and physicians discuss each case with each other and at Dr McGrath’s clinic, the care team holds dedicated ILD meetings, which is the European standard.

He presented slides illustrating the lung ‘honeycombing’ effect typically seen in scans and outlined the different approaches to slow- or rapid-progressing disease. However, it is extremely important to identify and treat those who experience acute exacerbations, he explained.

“It can sometimes be difficult to determine the pathogenesis but sometimes there can be a series of ‘silent insults’, which heal abnormally and result in scarring,” he told the meeting. “There are multiple cytokines and chemokines involved and that’s why the new drug developments are so exciting, because they target these pathways. ”

He presented data from a range of publications looking at a variety of study populations and British Thoracic Society, American Thoracic Society and ERS treatment guidelines. Studies found that in the past, patients who were on triple therapy were in fact being adversely affected by their medications.

He then presented data from trials on pirfenidone, including its development and efficacy. The guidelines and updates have changed over the years but in 2015, the ATS issued a conditional recommendation for the use of pirfenidone, the meeting heard.

Pirfenidone has been shown to be a good inhibitor of lung function decline, said Dr McGrath. “Pooled data also showed that it improved all-cause mortality and IPF-related mortality,” he added. “The inclusion criteria for the pirfenidone trials were also very strict, which is to be welcomed.”

It also behooves the physician to manage patient expectations when a therapy is prescribed: “The lung still declines but this therapy slows that down significantly and that’s fantastic,” he said, “but the first consultation is often the hardest — it’s important for patients to know that there is no ‘cure’, as such.”

<h3 class=”subheadMIstyles”>ILD clinic</h3>

The study day — which saw presentations from speakers interspersed with lively and challenging Q&A sessions — next heard from Ms Bernadette Bowen, Respiratory Nurse Specialist, Cork University Hospital, who delivered a talk on ‘Nursing Challenges in Ireland — Running an IPF Clinic: Practical Insights’. She outlined the criteria for pirfenidone use and added that the therapy regulates the TGF beta and TNF alpha pathways and the recommended dose is three capsules three times a day, she added. “So we know it works in the practical setting and we know it has an acceptable side-effect profile,” she told the meeting.

She explained that in her specialist ILD clinic, the patient experience with pirfenidone was prospectively evaluated in consecutive patients. The doses in the patients varied, however she noted that one tablet per day was recognised as not being effective and that tolerability was assessed on the basis of the ASCEND study.

Patients taking pirfenidone at her clinic show “very encouraging” signs, she said, including improved scores in the Six-Minute Walk Test. “The long waiting list for pulmonary rehabilitation is a real challenge… we need to catch these patients and get them into the clinic earlier in their disease. It can be sad and difficult, because when we see them late in the disease, both we and the patients work so hard but often, in just a few months they die.

“But we do find that the social and personal interaction is really valuable for them, so we have to keep that in mind and balance it against the effort it takes for them to get to clinic.”

Next to speak was Ms Lynn Fox, Respiratory Nurse Specialist, Mater Hospital, Dublin, who helped to set up the nurse-led IPF clinic in the Mater Hospital, Dublin, which is run in conjunction with Dr O’Reilly’s clinic. She presented four case studies from the clinic, which illustrated a range of considerations when treating IPF, including a case of interstitial pneumonia in a man who lost several of his siblings to lung disease, highlighting the influence of genetic factors in some cases.

In another heavily-smoking patient, Ms Fox pointed out that smoking cessation was heavily emphasised, as tobacco use inhibits the efficacy of pirfenidone.

“In terms of symptom management, there is no algorithm for treatment,” she explained. “Quality of life is vital and we also have to be sure to be sensitive to patients in their transition from active to palliative care.”

“Psychological support and anxiety management are also necessary,” she said. “Fatigue is also a major factor — one of the patients in our case studies said he felt like he had been ‘hit by a bus’ and chronic fatigue has all kinds of consequences, including poor decision-making. This, combined with cough, can lead to depression, embarrassment and ultimately social withdrawal.”

Ms Irene Byrne, Senior Physiotherapist, Mater Hospital, Dublin, took to the podium to describe pulmonary rehabilitation for IPF patients and the associated practical considerations. She explained that rehabilitation is an area that can cause “a great deal of angst” among patients. She expressed her desire to present the attendees with a number of resources, with which they could build a ‘box of tools’ to help patients rehabilitate.

She drew comparisons between IPF and COPD, in the way it can “rampage and progress”, although COPD progresses more slowly. “One size does not fit all for IPF,” she said. “We need to talk to the patient to establish what they expect, what they know, what they need.”

She presented data that showed clearly that improving lower limb strength improves outcomes and called for patients to be given permission to change their level of oxygen supply in certain circumstances, rather than rigidly adhering to guidelines in every case.

Ms Byrne left the attendees with a number of resources, including the Irish Lung Fibrosis Association ( and advised giving patients a STALL card to help them develop an effective breathing technique during their rehabilitation.

The STALL card is a wallet/purse-sized card that contains simple guidance for patients who experience breathlessness and can also be given to a patient’s carer to assist them when a patient is breathless, and can be downloaded from the ILFA website.

Ms Patricia Davis, Respiratory Nurse Specialist, St James’s Hospital, Dublin, provided an overview of the <em>Irish Guidelines on Long Term Oxygen Therapy </em>(available from the Irish Thoracic Society,, explaining: “In developing these guidelines, we actually received clarity on prescribing habits,” she said.

“For the future, we would ideally like to have dedicated oxygen clinics.”  When patients plan to fly, there needs to be discussion with the oxygen clinic and the airline carrier needs to be informed of the arrangements, she said.

“The guidelines are valuable, but sometimes clinical judgment needs to differ from the guidelines in the practical setting,” she explained.  “We have to put the patient at the centre of care and often, this involves finding a balance between an acceptable quality of life and the target saturations.”

Ms Nicola Cassidy, Committee Member, Irish Lung Fibrosis Association, next delivered her talk, ‘ERS/ELF Statement on IPF: Patient perspective on Patient-Centered Care’.

She explained that the Irish Lung Fibrosis Association is a voluntary organisation set up in 2002 to support patients and families affected by IPF.

In 2013, the ERS Task Force on diagnosis and management of IPF and the ELF invited patient organisations across Europe to contribute patient perspectives for the Task Forces’ major publication, due in 2016. 

ILFA organised five focus group meetings across Ireland. Some of the major concerns for patients included the communication of their diagnosis, and access to psychological support, pulmonary rehabilitation programmes, and palliative care as their condition progresses.

She said the impending updated statement will include the patient voice and noted that patients said they valued clear communication and empathy. For some, the poor manner in which their diagnosis was delivered was more upsetting than the diagnosis itself.

In the penultimate presentation, Dr Faith Cranfield, Consultant in Palliative Medicine, St Francis Hospice and Connolly Hospital, Dublin, provided an overview of generalist and specialist palliative care in her talk ‘Palliative Care for IPF Patients: Who Initiates this Discussion?’

She stressed the importance of a real dialogue with patients and commented: “Physicians can some- times be wary of addressing fear in patients… it’s about looking at the whole person, rather than just the illness itself. Doctors like having answers and sometimes with patients with advanced illnesses, we just don’t have them.”

She said that in providing palliative care, physicians rely heavily on community-based services, including occupational therapists and physiotherapists, although she stressed how under-resourced these areas of patient care are. However, she said these personnel are vital to help patients manage expectations, know their limitations  and “pace themselves”.

Dr Cranfield also urged the delegates to consult the Palliative Care Needs Assessment Guide (available at and to use it as a resource when evaluating what palliative care needs their patients have, and whether they require referral to specialist palliative care services.

She also referenced the online resource Oncotalk (, which provides guidance for difficult conversations specially designed for those who deal with palliative care patients.

<h3 class=”subheadMIstyles”>Lung transplant</h3>

In the final presentation of the day, Dr Peter Riddell, SpR in Respiratory Medicine, St Vincent’s Hospi-tal, Dublin, provided a broad over-view on ‘Lung Transplant for IPF Pa-tients’.

He explained that one trans-plant procedure is “up to 24 hours of work”, involving doctors, nurses, pi-lots and a range of other sup-port staff, and the operation itself can typically involve six-to-eight hours on the operating table for the patient.

 He said that a patient should be con-sidered for transplant when they have advanced (DLCO <40 per cent), or pro-gressive (FVC decline of 10 per cent) disease. He outlined the transplant ‘jour-ney’, from referral, to the procedure itself, to follow-up care and postop-erative measures, such as early intu-bation and intensive rehabilitation.

 He described the key messages as: “The need to stratify disease [limited vs advanced IPF]; early referral for transplant assessment; the need to optimise rehab po-tential; and the significant survival benefit of transplantation.”

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