Table 5.

Final AE-IPF consensus statement

AE-IPF definition

Development or worsening of breathlessness within the preceding 30 days that is otherwise unexplained New diffuse chest infiltrates on chest X-ray and/r HRCT that are otherwise unexplained Exclusion of infection, in as far as possible, according to routine clinical practice and standard local practice, through microbiological studies and viral studies Exclusion of alternative causes as per routine clinical practice including left heart failure, PE, and identifiable causes of acute lung injury

AE-IPF treatment path

Supportive measures should be provided to increase patient comfort

AE-IPF treatment path Acute treatment Supportive measure: Oxygen

Oxygen should be supplied to correct hypoxia and improve dyspnea It is advised to titrate SpO2 to >88% High-flow oxygen delivery mechanism, e.g., Optiflow™ (Fisher & Paykel Healthcare), may be required A test dose of a benzodiazepine (generally lorazepam) may be prescribed followed by monitoring of SaO2 to avoid respiratory depression If a benzodiazepine is beneficial, dose should be titrated accordingly to manage symptoms Opiates may be prescribed in combination with a benzodiazepine, or alone if a benzodiazepine test dose is not tolerated

Supportive measure: psychological and spiritual support

Psychological and/or spiritual support should be offered to all patients as appropriate

Anti-infectives a

A broad spectrum respiratory antibiotic should be prescribed as determined by clinical judgment in conjunction with local guidelines If a patient has recently been admitted as an inpatient they should additionally be covered for hospital acquired infection Anti-virals should not be prescribed routinely but only in cases of strong clinical suspicion and in accordance with local guidelines

Immunosuppressants

Corticosteroids should be considered in all patients unless specifically contra-indicated

Long-term corticosteroid dosing should be determined based on the individual patient

Cyclophosphamide/azathioprine/MMF should not be prescribed

The use of biologics, e.g., rituximab, is not recommended

Anti-coagulants

LMW heparin and/or anticoagulants should be initiated prophylactically to prevent DVT according to standard hospital policy, unless patients are already receiving anticoagulation therapy

Anti-fibrotic therapy

If patients are already receiving anti-fibrotic therapy then this should be continued Patients should not be initiated on anti-fibrotic therapy in the setting of an acute exacerbation

Escalation

Intubation and mechanical ventilation are not part of standard care Where possible the decision not to intubate or initiate mechanical ventilation should be discussed with patients at an early stage after IPF diagnosis CPAP can provide a helpful supportive measure End of life care should be discussed and agreed with patients and their families

Transplant

If patients are already on the transplant waiting list then the transplant unit should be informed If patients recover from AE-IPF review suitability for transplant referral, ideally at an early follow-up appointment in the clinic

Long-term management

Appropriate long-term management is essential following AE-IPF by a clinician with specialist expertise in IPF management As part of long-term management a review should assess the need for transplant referral As part of long-term management a review should assess the need for anti-fibrotic treatment As part of long-term management a review should assess the suitability for inclusion in clinical trials As part of long-term management a review by a respiratory physiotherapist should assess the need for pulmonary rehabilitation Appropriate supportive and palliative care mechanisms should be put in place

Discharge planning

Discharge planning should ensure adequate home oxygen is in place prior to discharge if required Discharge planning should ensure an early post-discharge review at an ILD clinic is in place (within 4–6 weeks) An appropriate discharge letter should be provided

Preventative therapies

There is no clear evidence to provide recommendations for preventative therapies at present

Additional information

Note that this is an opinion-led standard of care and that there is an absence of an evidence base

AE-IPF acute exacerbations of idiopathic pulmonary fibrosis, CPAP continuous positive airway pressure, DVT deep venous thrombosis, HRCT high-resolution computed tomography, ILD interstitial lung disease, LMW low molecular weight, MMF mycophenolate mofetil, PE pulmonary embolism, SpO ₂ peripheral oxygen saturation

^aNegative diagnosis is never completely certain