TABLE 1.
Assessment and management of the most frequent problems in the best supportive care of patients with idiopathic pulmonary fibrosis (IPF)
Symptom/factors limiting QoL | Tool for assessment | Interventions |
General well-being | EQ-5D SF-36/RAND SGRQ-IPF# K-BILD# ATAQ-IPF# |
Management of symptoms influencing QoL Mindfulness/meditation Physical rehabilitation¶ Nutritional support |
Dyspnoea | mMRC SGRQ-IPF# K-BILD# ATAQ-IPF-cA# UCSD SOB# |
Physical rehabilitation¶ Supplemental oxygen Treatment of PH with sildenafil¶ Pharmacological interventions (morphine/benzodiazepines)¶ |
Cough | LCQ# VAS# CQLQ# |
Poor effect of usual anti-tussive drugs Systemic steroids Thalidomide¶ Gabapentin Opiates PSALTI |
Anxiety/depression | EQ-5D SF-36/RAND K-BILD# SGRQ-IPF# ATAQ-IPF-cA# |
Counselling/cognitive behavioural therapy Antidepressants Physical rehabilitation Nutritional support (loss of appetite) |
Weight loss | NA | Nutritional support |
Comorbidities | NA | Treatment of PH with sildenafil¶ Anti-reflux measures in patients with GORD |
QoL: quality of life; EQ-5D: EuroQoL 5 dimension questionnaire [28]; SF-36/RAND: 36-item Short-Form Health Survey, developed by RAND (Santa Monica, CA, USA) [29]; SGRQ-IPF: St George's Respiratory Questionnaire, IPF-specific version [34]; K-BILD: King's Brief Interstitial Lung Disease questionnaire [38]; ATAQ-IPF: A Tool to Assess Quality of Life in IPF [35]; mMRC: modified Medical Research Council Dyspnoea Questionnaire [42]; ATAQ-IPF-cA: cross-Atlantic ATAQ-IPF [36]; UCSD SOB: University California San Diego Shortness of Breath Questionnaire [46]; LCQ: Leicester Cough Questionnaire [41]; VAS: visual analogue scale cough [47]; CQLQ: Cough Quality-of-Life Questionnaire; NA: not applicable; PH: pulmonary hypertension; PSALTI: physiotherapy, and speech and language intervention; GORD: gastro-oesophageal reflux disease. #: validated for use in IPF patients; ¶: efficacy somehow proved by randomised controlled trials involving patients with IPF.