Acute exacerbation of interstitial lung disease (AE-ILD) (2024)

October 4, 2023 by Josh Farkas

CONTENTS

• Definition and background
• Clinical presentation
• Differential diagnosis
• Investigation
  • History
  • Physical examination
  • Laboratory studies
  • CT scan
  • Bronchoscopy
• Treatment
  • Medical therapy
    • D/c potential pneumotoxins
    • Antibiotics
    • Immunosuppressive therapy
    • Antifibrotic therapy
  • Respiratory support
    • Noninvasive respiratory support
    • Invasive mechanical ventilation
  • Lung transplantation
• Prognosis

abbreviations used in the pulmonary section:

• AE-ILD: Acute exacerbation of ILD 📖
• AIP: Acute interstitial pneumonia (Hamman-Rich syndrome) 📖
• ANA: Antinuclear antibody 📖
• ANCA: Antineutrophil cytoplasmic antibodies 📖
• ARDS: Acute respiratory distress syndrome 📖
• ASS: Antisynthetase Syndrome 📖
• BAL: Bronchoalveolar lavage 📖
• BiPAP: Bilevel positive airway pressure 📖
• COP: Cryptogenic organizing pneumonia 📖
• CPAP: Continuous positive airway pressure 📖
• CPFE: Combined pulmonary fibrosis and emphysema 📖
• CTD-ILD: Connective tissue disease associated interstitial lung disease 📖
• CTEPH: Chronic thromboembolic pulmonary hypertension 📖
• DAD: Diffuse alveolar damage 📖
• DAH: Diffuse alveolar hemorrhage 📖
• DIP: Desquamative interstitial pneumonia 📖
• DLCO: Diffusing capacity for carbon monoxide 📖
• FEV1: Forced expiratory volume in 1 second 📖
• FVC: Forced vital capacity 📖
• GGO: Ground glass opacity 📖
• GLILD: Granulomatous and lymphocytic interstitial lung disease 📖
• HFNC: High flow nasal cannula 📖
• HP: Hypersensitivity pneumonitis 📖
• IPAF: Interstitial pneumonia with autoimmune features 📖
• IPF: Idiopathic pulmonary fibrosis 📖
• IVIG: Intravenous immunoglobulin 📖
• LAM: Lymphangioleiomyomatosis 📖
• LIP: Lymphocytic interstitial pneumonia 📖
• MCTD: Mixed connective tissue disease 📖
• NIV: Noninvasive ventilation (including CPAP or BiPAP) 📖
• NSIP: Nonspecific interstitial pneumonia 📖
• NTM: Non-tuberculous mycobacteria 📖
• OP: Organizing pneumonia 📖
• PAP: Pulmonary alveolar proteinosis 📖
• PE: Pulmonary embolism 📖
• PFT: Pulmonary function test 📖
• PLCH: Pulmonary Langerhans Cell Histiocytosis 📖
• PPFE: Pleuroparenchymal fibroelastosis 📖
• PPF: Progressive pulmonary fibrosis 📖
• PVOD/PCH Pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis 📖
• RB-ILD: Respiratory bronchiolitis-associated interstitial lung disease 📖
• RP-ILD: Rapidly progressive interstitial lung disease 📖
• TNF: tumor necrosis factor
• UIP: Usual Interstitial Pneumonia 📖

definition & background

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definition of acute exacerbation of interstitial lung disease (AE-ILD)

• Interstitial lung diseases may be marked by abrupt flares of disease activity.
• Based on the 2016 definition of acute exacerbation of idiopathic pulmonary fibrosis, the following may be a reasonable definition of an acute exacerbation of interstitial lung disease (AE-ILD): (35433309)
  • (1) Patient has a known diagnosis of underlying interstitial lung disease.
  • (2) Acute clinical deterioration within <1 month duration.
  • (3) CT scan shows new bilateral GGO (ground glass opacities) and/or consolidation superimposed on a background pattern consistent with the interstitial lung disease.
  • (4) Deterioration is not fully explained by cardiac failure or fluid overload.
• However, an acute exacerbation can occasionally be the initial presentation of interstitial lung disease. (Fishman 2023)

which diseases are we talking about here?

• ILD (interstitial lung disease) is a broad term use to refer to a wide range of poorly understood lung diseases. This chapter is predominantly written regarding the following interstitial lung diseases:
  • Idiopathic pulmonary fibrosis (IPF).
  • Nonspecific interstitial pneumonitis (NSIP).
  • Connective tissue-associated interstitial lung disease (CT-ILD).
• IPF (idiopathic pulmonary fibrosis) is one of the most common interstitial lung diseases. Acute disease exacerbations are common among these patients. Given that exacerbations of IPF are common, this is one of the better studied examples of exacerbations of interstitial lung disease. As such, exacerbations of IPF are often used as a template to understand and treat exacerbations of less common interstitial lung diseases.

common triggers of ILD exacerbation

• No identifiable trigger (~50%).
• Infection (~20%).
• Subacute progression or end-stage disease (~15%).
• Heart failure or severe pulmonary hypertension (~5%).
• Venous thromboembolic disease (~5%).
• Diffuse alveolar hemorrhage.
• Peri-procedural exacerbation: (35433309)
  • Bronchoscopy.
  • Surgical lung biopsy.
  • Lung cancer resection.
  • Intubation and mechanical ventilation.
• 💡 There is a grey area around whether certain conditions (especially infection) should be regarded as triggers for exacerbation, as alternative diagnoses, or perhaps as both. In clinical practice, it may be impossible to determine how much pulmonary dysfunction is due to infection versus exacerbation of interstitial lung disease.

clinical presentation

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• Dyspnea is the most common symptom.
• Cough is frequent.
  • Nonproductive cough is often seen.
  • Copious sputum production may suggest superimposed infection.
• New or worsening hypoxemia is expected. Hypoxemia may be quite severe, especially among patients with chronic hypoxemia (e.g., patients frequently require high-flow nasal cannula support).(33957435)
  • One characteristic of interstitial lung disease is that the severity of hypoxemia is often disproportionateto the severity of dyspnea.

differential diagnosis

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See Also

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• Infection:
  • Community-acquired pneumonia.
  • Pneumocystis.
  • Invasive aspergillosis.
  • Other opportunistic pneumonias (e.g., cryptococcus neoformans, endemic mycoses).
• Heart failure:
  • Cardiogenic pulmonary edema.
  • Decompensated pulmonary hypertension.
• Pulmonary embolism.
• Drug-induced lung disease.
• Diffuse alveolar hemorrhage (among patients with underlying connective tissue disease or vasculitis).
• Aspiration pneumonitis.
• COPD exacerbation.
• Pneumothorax.
• Interstitial lung disease:
  • Acute exacerbation of interstitial lung disease (decline of <1 month).
  • Subacute/chronic deterioration of interstitial lung disease (decline over >1 month).
• (The broader differential diagnosis includes any cause of ARDS. For a more complete list of disorders causing ARDS: 📖).

investigation

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Interstitial lung disease exacerbation may occasionally be the initial presentation of an underlying rheumatological disorder. If this is a possibility, patients should be evaluated for an underlying rheumatologic diagnosis.

history

medication review

• Patients with underlying interstitial lung disease are at increased risk of drug-induced lung disease.
• Review any recent or current medications to evaluate for potential drug-induced lung disease. Medications that should receive particular attention include:
  • Cancer therapies (e.g., checkpoint inhibitors, chemotherapeutics).
  • Biologic therapies.
  • DMARDs (disease-modifying antirheumatic drugs).
• Pneumotox.com is the reference standard to evaluate for the risk of drug-induced lung disease. Unfortunately, determining the absolute risk of harm from any specific drug is often unknown.

infection history

• Travel?
• Animal exposure?
• Residence in TB-endemic country?
• Review prior culture results? (drug-resistant organisms?)
• Recent healthcare contact? (admission, IV antibiotics?)

rheumatological review of systems

• Small joint arthritis?
• Skin changes (including rash/thickening/tightening/fissuring)?
• Raynaud phenomenon?
• Dry eyes / dry mouth?
• Muscle weakness?

patient questionnaire for patients with unknown underlying ILD:

• For patients whose underlying interstitial lung disease diagnosis is unclear, an exhaustive review of systems may be useful. A questionnaire by CHEST may be helpful with this: 📄

physical examination

examination findings related to ILD

• Digital clubbing?
• Fissuring of the fissures? (Mechanic's hands).
• Thickening or tightening of the skin?
• Other rashes or skin lesions?

POCUS

• Pulmonary:
  • Evaluate for pneumothorax.
  • Evaluate for pleural effusion.
  • (Many patients with interstitial lung disease will have B-lines diffusely, so this doesn't necessarily indicate anything.)
• Cardiac:
  • Evaluate volume status.
  • Evaluate for features of pulmonary hypertension (e.g., right ventricular dilation).
  • Evaluate for features of left-sided heart failure (e.g., left atrial dilation, impaired left ventricular systolic function).
  • Formal echocardiogram should be obtained if needed.

laboratory evaluation: studies to consider

infectious evaluation

• Viral pathogens:
  • Nasopharyngeal PCR for influenza, COVID.
  • Extended PCR for other viral pathogens.
• Bacterial pathogens:
  • Blood cultures.
  • Sputum culture and gram stain.
  • Procalcitonin.
  • Urine legionella & pneumococcus antigens.
• Pneumocystis evaluation in patients on significant, chronic immunosuppression:
  • Beta-D-glucan level.
  • Induced sputum for pneumocystis PCR.
• Fungal evaluation in patients with substantial consolidation (especially if nodular):
  • Fungal culture & smear of any sputum produced.
  • Beta-D-glucan level.
  • Galactomannan level.
  • Serum cryptococcal antigen.
  • Urine histoplasma & Blastomyces antigens.

other laboratory studies

• CRP (C-reactive protein).
• D-dimer.

rheumatologic evaluation

• (This is useful only if an underlying rheumatologic disorder is suspected. Tests most likely to affect management are in bold.)
• ANA (antinuclear antibody) and anti-double-stranded DNA antibody.
• RF (rheumatoid factor) and anti-CCP (cyclic citrullinated peptide) autoantibodies.
• Extractable Nuclear Antigens (ENA panel) – RNP, Sm, SSa, SSb.
• MyoMarker 3 plus panel – evaluation for idiopathic inflammatory myositis.(34488971) This is a send-out test to the Mayo Clinic (test ID FMYO3).
  • 💡 The myomarker panel may be the test most likely to affect management in the context of an ILD exacerbation. Identification of anti-synthetase syndrome (ASS) or anti-MDA5 antibodies may have major implications for therapy.
• Creatine kinase (CK).
• Anti-topoisomerase [anti-Scl-70] antibody.
• ANCA antibodies – if the history and imaging raise the possibility of diffuse alveolar hemorrhage (DAH).

chest CT scan

chest CT angiogram is an essential study in acute exacerbation of ILD

• Chest CT scan is pivotal for the diagnosis and evaluation of ILD exacerbation. For example, the entire definition of an ILD exacerbation is based on the CT scan.
  • CT may rapidly exclude alternative diagnoses, such as:
    • Pulmonary embolism.
    • Pneumothorax.
    • Pleural effusion.
    • Atelectasis.
  • In a patient with an ILD exacerbation, there must be new bilateral ground-glass opacities and/or consolidations. If these are absent, then the diagnosis of ILD exacerbation is excluded.
• How to protocol the CT scan:
  • CT angiography (“PE protocol”) is generally preferred, as this will allow for simultaneous evaluation of pulmonary embolism and the lung parenchyma. Patients with interstitial lung disease may be at increased risk of pulmonary embolism. (21737559) Please note that contrast dye is not nephrotoxic, so this study may be obtained regardless of renal function. 📖
  • If D-dimer excludes PE, then a noncontrast CT scan could be protocoled to focus on the lung parenchyma. This may provide somewhat better images of the lung tissue.
• If lung nodules, cavitation, or unusual patterns are seen on CT scan, this may raise the index of concern for alternative diagnoses (e.g., fungal pneumonia).

CT scan findings in acute exacerbation of IPF (AE-IPF)

• Chronic findings due to IPF are seen, along with superimposed acute ground-glass opacities (GGO) and/or consolidation. Comparison to prior CT scans may help provide a baseline, to determine which findings are chronic versus which are acute.
• Acute abnormalities may often be classified as diffuse, multifocal, or peripheral:
  • Peripheral pattern: Patients with peripheral opacities have a more favorable prognosis than patients with multifocal or diffuse opacities. (Walker 2019) Unlike multifocal patterns, peripheral pattern doesn't tend to progress over time.(31994940) Histologically, patients with peripheral opacities may tend to have either OP (organizing pneumonia) or extensive fibroblastic foci, rather than DAD (diffuse alveolar damage). (18451320) Clinically, a peripheral pattern may correlate with improvement following steroid therapy.(8976924)
  • Multifocal pattern (e.g., patchy involvement of both central and peripheral lung regions) – this may progress to diffuse opacification.
  • Diffuse pattern: this may carry a higher risk of mortality. (31994940)

bronchoscopy

bronchoscopy is a high-risk procedure in these patients

• Bronchoscopy itself is a known trigger of exacerbation of idiopathic pulmonary fibrosis, so it can definitely cause harm.(31177864) Among patients admitted to the general medicine ward with ILD exacerbation, a quarter of patients who undergo bronchoscopy subsequently deteriorate and require ICU admission.(35433309)
• One series found that only 13% of bronchoscopies in acute exacerbation of ILD yielded abnormal results, leading to a change in management among 3% of patients.(31177864, 27712021) Among most patients with ILD exacerbation, the likelihood that a bronchoscopy will reveal a novel diagnosis which improves their outcomes is extremely low.

factors that may favor bronchoscopy:

• Patient is likely to tolerate the procedure without requiring intubation (e.g., only requiring a few liters of oxygen).
• Patient is heavily immunosuppressed, raising concern for a broad range of possible opportunistic infections.
• CT scan features suggest fungal or mycobacterial infection (e.g., nodules, cavitation).

factors which argue against bronchoscopy

• Non-intubated patient on high-level oxygen support, who is at risk of deterioration and requiring intubation post bronchoscopy.
• Absence of substantial immunosuppression.
• CT scan lacks nodules, cavitation, or other features that suggest an unusual infection.

When in doubt, it is generally wise to proceed with empiric therapy (e.g., steroids plus antibiotics) while awaiting the results from a noninvasive infectious workup.

medical therapy

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discontinue any potentially offensive medications

• Consider discontinuation of any medications which may be associated with interstitial lung disease.
• As discussed above, the patient's medication list should be cross-checked against pneumotox.com.

antibiotics

• It is often impossible to exclude infection initially. Consequently, most patients will be covered initially with antibiotics.
• For patients who have not received substantial immunosuppression, coverage of typical bacterial pathogens may be sufficient (e.g., ceftriaxone plus azithromycin).
  • Azithromycin has some immunomodulatory properties, so it may be especially desirable.(35433309)Several retrospective studies suggest a benefit of azithromycin among patients with an exacerbation of idiopathic pulmonary fibrosis. (31994940) A negative procalcitonin cannot exclude atypical pneumonia, so a full course of azithromycin should generally be completed (e.g., 500 mg azithromycin IV daily for 3-5 days).
• For patients at high risk of pneumocystis 📖, empiric coverage may be considered (trimethoprim-sulfamethoxazole 💉). A combination of steroid, azithromycin, plus trimethoprim-sulfamethoxazole will provide broad coverage of pneumocystis, typical bacterial pneumonia, atypical bacterial pneumonia, or ILD exacerbation.
• Antibiotic therapy may be narrowed over time, based on the results of laboratory studies.

immunosuppressive therapy

(1) AE-IPF (acute exacerbation of IPF)

• Steroid:
  • IPF is not generally a steroid-responsive disorder. There is no high-quality evidence that steroid is beneficial for acute exacerbation of IPF. However, steroid is weakly recommended in guidelines and generally utilized.(27299520)
  • The ideal steroid dose is unknown. Historically, very high steroid doses were often utilized (e.g., 1,000 mg/day pulse doses of methylprednisolone). However, recent trends are towards utilization of lower doses.
  • A common regimen may be as follows: (31177864; 33312849)
    • Three days of high-dose IV methylprednisolone (e.g., 125 mg daily, or 60 mg IV q6hrs).
    • Transition to oral prednisone ~1 mg/kg/day ideal body weight, with a taper to discontinuation over 2-4 weeks. However, if there is no response to steroid, more rapid tapering may be considered (discussed further below).
  • Possible bacterial infection is not a contraindication to steroid administration (such patients may often benefit from a combination of steroid plus antibiotics). However, if CT imaging suggests that a fungal or mycobacterial infection is likely, then steroid is contraindicated.
• Other immunosuppressive therapies:
  • A myriad of advanced immunosuppressive regimens have been used in small case series (e.g., cyclophosphamide, cyclosporine, tacrolimus, rituximab, plasma exchange, intravenous immunoglobulin). To date, there is no high-quality evidence that any of these therapies are effective. Isolated reports have suggested benefits from various therapies, but these reports are subject to numerous sources of bias:(27299520)
    • Sicker patients may be deemed “too sick” to receive more advanced therapies.
    • Treatment with more advanced therapies may be restricted to patients who survive long enough to be considered for them (survivorship bias).
    • Historical control groups may have received suboptimal critical care support (based on ongoing improvement in supportive critical care over time).
    • Publication bias causes positive studies to be published, whereas negative studies are unpublishable.
  • Recently, a randomized controlled trial demonstrated that cyclophosphamide increases mortality among patients with idiopathic pulmonary fibrosis. (EXAFIP trial; 34506761)
  • Given known risks of these treatments, their use is difficult to justify.

(2) acute exacerbation of CTD-ILD (connective tissue disease-related interstitial lung disease)

• Steroid:
  • There is a stronger rationale for use of steroid in patients with CT-ILD than in IPF.
  • Similar doses as compared to IPF may be reasonable (discussed above).
  • ⚠️ High-dose steroid should be used with caution in patients with scleroderma, since steroid may induce a scleroderma renal crisis. (30578331)
• Other immunosuppressive regimens:
  • Patients with myositis-associated interstitial lung disease may require very aggressive and complex regimens of immunosuppressive therapy. This is discussed further here: 📖
  • Patients with other types of CTD-ILD may may be treated under the paradigm of rapidly progressive CTD-ILD as discussed further here: 📖.
  • Consultation with rheumatologists and/or pulmonologists with expertise in interstitial lung disease may help determine the optimal immunosuppressive regimens for these patients.

(3) acute exacerbation of other forms of interstitial lung disease:

• Other interstitial lung diseases may be more steroid-responsive than IPF (e.g., nonspecific interstitial pneumonia).
• Steroid therapy is often utilized similarly as in patients with IPF (See #1 above).

evaluation of the response to steroid

• Response to steroid may be evaluated over several days.
  • The primary factor to follow is oxygen requirement and radiological opacities (e.g., serial chest radiograph).
  • Some patients have low baseline CRP (C-reactive protein) levels and steroid doesn't affect the CRP level. There is no evidence surrounding this, but lack of effect on biomarkers such as CRP might theoretically suggest that steroid isn't having a biological effect.
• If there is a response to steroid, this suggests a significant component of organizing pneumonia (OP). Steroid may be gradually tapered with careful monitoring of clinical effectiveness.
• If there is no response to steroid (and especially if the patient continues to deteriorate), this suggests a predominantly fibrotic process or diffuse alveolar damage (DAD). It could be sensible to stop or to more abruptly taper steroid in this context. (37397591) Prolonged steroid therapy for steroid-refractory patients may merely expose them to risks of delirium, steroid-induced myopathy, or critical illness neuropathy.

diuresis & volume management

• As in other patients with ARDS, the therapeutic goal is euvolemia.
• Among patients receiving significant fluid inputs, diuretics should be used to maintain an even fluid balance. One study of intubated patients with ILD exacerbation found that net positive fluid balance correlated with increased risk of mortality.(32355133)
• Among patients with cardiac dysfunction and possible/suspected heart failure, diuresis is often trialed. Any suggestion of volume overload on the chest CT scan (e.g., bilateral effusions, septal thickening) supports a trial of diuresis.

antifibrotic therapy

• There is no evidence evaluating the use of antifibrotic therapy for acute exacerbation of interstitial lung disease.
• For patients who were taking antifibrotic therapy prior to admission (e.g., nintedanib), it makes sense to continue this therapy. (35433309)
• Initiation of antifibrotic therapy during an ILD exacerbation is reasonable, if this is logistically feasible. (37397591)

respiratory support

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noninvasive respiratory support

• Early noninvasive respiratory support may be useful to prevent respiratory muscle fatigue.
• High-flow nasal cannula (HFNC) is generally preferred for patients with interstitial lung disease. This has the advantage of comfort and of avoiding augmentation of tidal volumes.
• CPAP or BiPAP may be considered in patients who are failing to respond to HFNC, or in patients with coexisting respiratory disorders (e.g., obstructive sleep apnea, obesity hypoventilation, or COPD). Given that intubation is associated with a very high mortality in patients with ILD exacerbation, a trial of CPAP or BiPAP makes sense. Available case series suggest that this may succeed in roughly half of patients. (37397591)

invasive mechanical ventilation

background on invasive mechanical ventilation

• Invasive mechanical ventilation has poor success rates among patients with exacerbations of interstitial lung disease (especially idiopathic pulmonary fibrosis).
• The reason for poor outcomes following intubation may include the following:
  • (1) Patients with interstitial lung disease often have high levels of lung inflammation (e.g., diffuse alveolar damage). Superimposed ventilator-induced lung injury may exacerbate this further, leading to a vicious spiral of increasing airway pressures and escalating lung injury.
  • (2) Patients are often treated with steroid and noninvasive respiratory support initially. The subset of patients who deteriorate despite these interventions and require intubation have steroid-refractory disease, which is extremely difficult to treat.
  • (3) The median survival for patients with idiopathic pulmonary fibrosis who survive an exacerbation is 2-4 months. So, this is a severe disease overall – even among patients who are able to survive the exacerbation. (27299520, 33957435)
• Guidelines and review articles warn against intubation for most patients with progressive idiopathic pulmonary fibrosis. For example:
  • The ATS/ERS/JRS/ALAT guidelines on idiopathic pulmonary fibrosis state that “mechanical ventilation is not recommended for the majority of patients with respiratory failure.” (35486072)
  • Syed et al. state that “The outcomes for ILD patients that require invasive mechanical ventilation are so poor that outside of transplant candidates, some experts have advised against endotracheal intubation for these patients apart from those patients that have clearly reversible causes for respiratory decompensation. (37397591)

factors to consider in determining whether intubation would be beneficial

• (1) Presence of any superimposed causes of respiratory failure that may be more readily treatable (e.g., pulmonary embolism, pleural effusion, infection, volume overload).
• (2) Duration of medical therapy prior to intubation:
  • Patients who require intubation very shortly after admission (prior to initiation of medical therapy) may be more likely to recover.
  • Patients who have deteriorated despite best medical therapy for several days have refractory disease, which is less likely to improve.
• (3) Baseline functional status, frailty, and patient wishes.
• (4) If significant pulmonary hypertension is present, this may impair the patient's ability to respond well to mechanical ventilation. In the extreme situation of a patient with severe pulmonary hypertension and severe interstitial lung disease, intubation may occasionally precipitate cardiac arrest.
• (5) Type of underlying lung disease:
  • Outcomes are worse in patients with IPF (idiopathic pulmonary fibrosis).
  • Outcomes are better for patients with connective tissue-associated interstitial lung disease or NSIP (nonspecific interstitial pneumonia). (35433309)
• (6) Candidacy for lung transplantation: in rare cases, intubation may be utilized as a bridge to transplantation.(31994940)

management of intubated patients

• The strategy for mechanical ventilator management is generally similar to the management of other patients with ARDS.
• Patients with combined interstitial lung disease and pulmonary hypertension may benefit from inhaled pulmonary vasodilators, but this is only a transient maneuver to stabilize patients (rather than definitive therapy).
• The management of intubated patients with ARDS is explored further here: 📖

lung transplantation

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• Lung transplantation may be an option for selected patients with established interstitial lung disease:
  • Ideally transplant evaluation should begin prior to disease exacerbation, with listing of patients on transplantation lists prior to hospital admission. This may facilitate prompt transplantation when patients deteriorate.
  • For patients who have not undergone transplantation evaluation, transplantation is logistically challenging. These patients may nonetheless be discussed with a lung transplantation service or regional referral center.
• ECMO may be considered for highly selected patients, as a bridge to transplantation. (33312849)

prognosis

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prognosis of acute exacerbation of IPF causing critical illness

• Short-term mortality is high, with a median survival of ~3 months. (27299520, 33957435) In-hospital mortality is on the order of 50%. (31994940, 33312849)
• Long-term survival is poor (e.g., one-year mortality is ~80%). (Shah 2019)
• Survivors generally don't regain their prior baseline function.

palliative care

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• Based on poor short-term and long-term prognosis, palliative care consultation is generally beneficial.
• Low-dose opioids may be helpful to simultaneously treat symptoms of breathlessness and cough.
• Many patients get “stuck” on high-flow nasal cannula. This leads to a condition where the patient is mentating, eating, and communicating normally – yet unable to improve and leave the hospital. This situation is extremely stressful for patients and families. If this condition persists for many days or weeks, patients usually elect to transition to comfort-directed care (including discontinuation of high-flow nasal cannula).

questions & discussion

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