Interstitial lung diseases (ILDs) comprise a large and heterogenous group of disorders. Patients with ILD typically experience breathlessness, persistent cough, fatigue, anxiety, and depression.¹ The management of ILD requires an individualized and multidisciplinary approach that takes into account the severity of ILD, presence of fibrosis, evidence of progression, comorbidities and patient’s preferences.²

A holistic approach is essential to the management of ILDs.³ It means to provide support that looks at the whole person and adjust the treatment to the individual needs. Holistic care does therefore not only focus on the disease itself but also on the wellbeing of the patient. It may include a variety of pharmacological and non-pharmacological interventions to manage the disease, reduce symptoms, and/or improve quality of life.

Management of the disease

The pharmacological treatment is guided by the underlying diagnosis and disease course. In most fibrotic ILDs with a suspicion of inflammation-driven disease, first-line therapy consists of immunomodulation with the use of glucocorticoids and/or immunosuppressive therapy. Preventing exposures and events that may drive further disease progression is also essential. For instance, avoiding the offending antigen is a priority in patients with chronic hypersensitivity pneumonitis.⁴

Antifibrotic treatment

In IPF, antifibrotic treatment should be given immediately upon diagnosis.¹ Antifibrotic treatment is considered in cases with progressive fibrosis despite appropriate first-line therapy. Nintedanib has been approved by the Food and Drug Administration (FDA) and European Medicines Agency (EMA) for patients with SSc-ILD, and progressive fibrosing ILDs. It has shown to significantly slow down forced vital capacity (FVC) decline in patients with progressive lung fibrosis.^5-8

Lung transplantation

Lung transplantation may be considered in patients with advanced pulmonary fibrosis who are relatively fit without relevant comorbidities.⁹ Extrapulmonary disease or severe coexisting conditions may disqualify some patients for lung transplantation, especially those with connective tissue disease (CTD).⁴

SUPPORTIVE CARE
Non-pharmacological management

Non-pharmacological management should be considered throughout the disease course.⁴Non-pharmacological care in chronic respiratory diseases mainly focuses on managing breathlessness and emotional well-being. The intensity of breathlessness is greatly influenced by reactions to the perception of breathlessness.¹⁰

As breathlessness does not solely depend on the severity of lung pathology, optimizing treatment of the underlying disease does not guarantee good symptom control.

Algori thm for the t reatment of fibrosing ILDs4

MMF=Mycophenolate Mofetil; CPM=Cyclophosphamide; TCL=Tocilizumab; AZA=Azathioprine; RTX=Rituximab; ABA=Aba-tacept; MTX=Methotrexate; IFX=Infliximab; ADA=Adalimumab

Lifestyle

Diet

Malnutrition or weight loss are common in patients with pulmonary fibrosis and may negatively affect the quality of life. Moreover, losing muscle mass can accelerate the deterioration in lung function. Also, overweight patients may find it harder to breathe and exercise, which is associated with a decreased quality of life and worse health outcomes.¹¹ A healthy diet is important for patients to prevent nutritional deficiencies and obtain or maintain a healthy weight.

Pulmonary rehabilitation

Pulmonary rehabilitation is an evidence-based, multidisciplinary and comprehensive medical intervention dedicated to patients with chronic diseases and reduced daily activity. The combination of pulmonary rehabilitation with an individualized treatment plan reduces the symptoms, improves physical fitness, enhancing the functional and psychological status, and quality of life of patients with ILD.¹²

Smoking cessation

Smoking is a risk factor for various lung diseases, including ILD. Cessation of smoking can improve outcomes in ILD and should be encouraged.¹³

Supplemental oxygen

In patients with fibrosing ILD hypoxia is common, even at rest in advanced disease. Despite little evidence, international guidelines recommend supplemental oxygen use in patients with severe resting hypoxia.¹⁴ As based on expert opinion, supplemental oxygen is indicated in patients with resting hypoxemia (PaO2 of <55 mm Hg, oxygen saturation as measured by pulse oximetry of <89%, or PaO2 of <60 mm Hg and cor pulmonale or polycythemia).⁴The main reasons for supplemental oxygen use would be to improve physical functioning and ultimately survival.¹⁴

Pulmonary rehabilitation and use of ambulatory oxygen in patients with isolated exertional hypoxemia has shown to improve the quality of life, reduce breathlessness, and increase walking ability.⁴

Vaccination

Vaccinations avoid infections that may worsen pulmonary fibrosis.⁹

Psychological and social care

The symptom burden and quality of life is not merely a consequence of the underlying physiological disorder but also depends on the patient’s adaptation to the illness.¹⁵ Psychological and lifestyle interventions can be used to prevent or reduce anxiety and depression. Improving patients’ skills and knowledge to manage their disease through self-management programs helps patients to carry out medical regimens specific to a long-term disease and guide behavioural change that improves well-being.¹⁶

For instance, cognitive behavioural techniques are increasingly incorporated into pulmonary rehabilitation programs to induce behavioural change.^15,17

Dignity-conserving therapy can help reduce loss of self-esteem, the feeling of being a burden to others and can provide an opportunity to reflect on things that matter.¹⁷

Examples of questions used in dignity therapy:

What would you want your family to know and remember about you?
What are your most important accomplishments/at what times did you feel most alive?
What have you learned that you would want to pass on to others?

Also, attendance to peer support groups, online forums or pulmonary rehabilitation programs may be helpful. In some countries, there are nurse-led support and education programs, with initial reports suggesting high patient satisfaction.¹⁴

Support for religious and spiritual aspects

When faced with a chronic disabling condition, patients frequently express existential, spiritual and religious aspects. It is essential that healthcare providers recognise these needs and facilitate support if needed. Many hospitals have specialistic carers available to support patients of many cultural and religious backgrounds.

The diamond model provides sample questions regarding five themes in spiritual/existential matters.¹⁸

Managing fear and anxiety

The physical burden of chronic respiratory diseases pose significant challenges in daily activities, social relationships, self-perception and emotional functioning. Some patients adapt well to their disability, while others respond with fear, anxiety or depression.¹⁹ Depression and anxiety often go unrecognised and untreated. It is therefore important to explore patients’ fears and worries by asking questions.²⁰

Managing breathlessness with thought

The Breathing Thinking Functioning model is a proposal, based on current evidence, that grasps the complexity of factors that perpetuate breathlessness. It therefore provides a rationale and focus to manage breathlessness.¹⁰

The Breathing Thinking Functioning model

The Breathing Thinking Functioning model can be used to explain mechanisms of worsening breathlessness and provide strategies on how to break the vicious cycle by behavioural change.¹⁰

Challenge misconceptions and provide non-pharmacological interventions for breathlessness¹⁰

PHARMACOLOGICAL MANAGEMENT OF BREATHLESSNESS

International statements recommend the use of oral and parenteral opioids to treat refractory breathlessness despite optimal non-pharmacological treatment in advanced lung disease.^21,22 However, patients with mild or moderate symptoms should first be managed with non-pharmacological treatments because long-term safety data of opioids is lacking.^21,23,24Studies support the use of sustained-release low-dose (10-30 mg) morphine in patients with severe breathlessness despite optimal non-pharmacological treatment.^24,25

BEST METHOD ACCORDING TO CURRENT EVIDENCE:

Benzodiazepines

Benzodiazepines are widely used for the relief of breathlessness in advanced malignant and non-malignant diseases. There is however little evidence for the efficacy of benzodiazepines to relieve breathlessness.^26,27 They may be considered as a second-or third-line treatment, when opioids and non-pharmacological interventions have failed to control breathlessness.²⁷

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