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Table 1 Treatable traits in interstitial lung diseases according to pulmonary, aetiological, comorbidities and lifestyle domains

From: Walking the path of treatable traits in interstitial lung diseases

Treatable trait

Assessment tool

(Potential) treatment option

Expected benefits of treatment*

Aetiological

CTDs/Vasculitis

Clinical features

Serum antibodies

Refer to rheumatologist

Screening for extra-respiratory involvement

Immunosuppressive drugs

Prevent or reduce lung damage

Reduce mortality

Drugs

Drug history

Assess risk–benefit of stopping potentially harmful drug

Reduce lung damage

Improve lung function

Exposure-related (organic and inorganic)

Environmental/ work/ domestic history of exposure

Serum precipitins

Prevent or stop exposure

Prevent or reduce lung damage

Improve outcomes

Reduce mortality

Genetic

Family history

Age of onset

DNA genetic testing

Refer to geneticist

Family screening

Targeted therapy

Improve outcomes

Lifestyle

Smoking

Patient reported

Urine cotinine levels

Tobacco cessation support

Nicotine replacement

Antidepressant drug

Improve quality of life

Improve lung function

Prevent or reduce lung damage

Adherence to treatment

Patient and relatives feedback

Education

Written instructions

Self-management

Family and social support

Improve outcomes

Exposure to air pollution

PM10 and NO2 concentrations

Reduce exposure

Reduce disease progression

Reduce exacerbation

Lack of exercise/ Deconditioning of skeletal muscle

Cardiopulmonary exercise testing

6MWT

Prescribed exercise programs

Pulmonary rehabilitation

Improve quality of life

Improve lung function

Improve exercise capacity

Diet

Patient reported

Diet instructions

Improve quality of life

Pulmonary

Progressive fibrosis

Patient reported symptoms

Pulmonary function tests

HRCT

Optimization of therapy

Consider antifibrotics

Referral to lung transplant center

Slow lung function decline

Reduce mortality

Prevent exacerbation

Eosinophilic inflammation

HRCT

BAL

CBC

Steroid therapy

Adjust immunosuppression

Prevent or reduce lung damage

Improve quality of life

Neutrophilic inflammation

HRCT

BAL

CBC

Azithromycin

Reduce lung damage

Improve quality of life

Acute exacerbation

HRCT

BAL

Antifibrotic therapy

Systemic glucocorticoids

Improve survival

Acute infection

Patient reported symptoms

Sputum cultures

BAL

Airway clearance

Antibiotic therapy

Prophylaxis with influenza and pneumococcal vaccination

Adjust immunosuppression

Prevent exacerbation

Reduce mortality

Reduce hospitalization

Chronic infection or recurrent infection

Patient reported symptoms

Sputum cultures

BAL

Airway clearance

Adjust immunosuppression

Consider prophylactic antibiotics

Prevent exacerbation

Improve quality of life

Slow lung function decline

Chronic respiratory failure

Patient reported symptoms

ABG

6MWT

Polysomnography

Long term oxygen therapy

Non-invasive ventilation

Referral to lung transplant center

Pulmonary rehabilitation

Palliative care

Improve quality of life

Improve survival

Intractable chronic cough

Patient reported symptoms

Scores (LCQ, VAS, CQLQ)

Antitussive

Thalidomide

Gabapentin

Improve quality of life

Emphysema / Obstructive ventilatory defects

Pulmonary function tests

HRCT

Bronchodilator therapy

Improve quality of life

Slow lung function decline

Comorbidities

GERD

Symptoms

Oesophageal pH monitoring

Manometry

Upper Endoscopy

Diet instructions

PPIs, H2-receptor antagonists, pro-kinetics

Fundoplication

Improve outcomes

Reduce lung damage

Pulmonary hypertension

Echocardiography

Consider RHC

Referral to lung transplant center

Trial with PH targeted therapies in selected patients (e.g.: Treprostinil)

Oxygen/Non-invasive ventilation

Improve quality of life

Improve outcomes

Reduce mortality

Congestive heart failure

NT-proBNP

Echocardiography

Targeted pharmacological treatment

ICD implantation

Improve quality of life

Reduce mortality

OSA

Sleep study

Diet instructions

CPAP treatment

Improve quality of life

Reduce mortality

Lung cancer

HRCT

PET- CT

Biopsy

Surgical resection

Radiotherapy

Chemotherapy

Reduce mortality

Diabetes

Fasting glucose persistently above 125 mg/dl

Random glucose levels above 200 mg/dl occurring in the context of high-dose glucocorticoid therapy

Diet instructions

Lifestyle instructions

Insulin

Oral hypoglycemics

Improve quality of life

Reduce systemic complication of diabetes

Reduce mortality

Osteoporosis/Osteopenia

Bone densitometry

Diet instructions

Lifestyle instructions

Pharmacological therapy

Improve quality of life

Reduce risk of fractures

Reduce mortality

Pulmonary embolism

CT pulmonary angiogram

Anticoagulants

Reduce mortality

Obesity

BMI

Diet instructions

Pulmonary rehabilitation

Bariatric surgery

Improve quality of life

Improve exercise tolerance

Cachexia/Malnutrition

BMI

Nutritional support

Pulmonary rehabilitation

Improve quality of life

Frailty

FFP binary score

Frailty Index

Nutritional supportive

Pulmonary rehabilitation

Physical activity programs

Improve quality of life

Reduce mortality

Anxiety/Depression

Patient reported

scores (K-BILD; SGRQ-IPF)

Counseling/cognitive behavioural therapy

Antidepressant/Anxiolytics

Pulmonary rehabilitation

Improve quality of life

Improve adherence to treatment

  1. CTD connective tissue disease, PM10 particulate matter 10, NO2 nitrogen dioxide, 6MWT six-minute walk test, HR-CT high-resolution computed tomography, BAL bronchoalveolar lavage, CBC complete blood count, ABG arterial blood gas, LCQ leicester cough questionnaire, VAS visual analogue scale, CQLQ cough-specific quality of life questionnaire, GERD gastro-esophageal reflux disease, PPI proton pump inhibitor, RHC right heart catheterization, NT-proBNP N-terminal pro-B-type natriuretic peptide, ICD implantable cardioverter defibrillator, OSA obstructive sleep apnea, CPAP continuous positive airway pressure, PET-CT Positron emission tomography computed tomography, BMI body mass index, FFP Fried’s frailty phenotype, K-BILD King's brief interstitial lung disease, SGRQ-IPF St George's respiratory questionnaire idiopathic pulmonary fibrosis
  2. *Most of benefits are speculative. Most of them are based on case report/case series or benefits coming from evidences in other diseases