Development and validation of a novel questionnaire to describe and assess sensations and triggers associated with refractory and unexplained chronic cough.

INTRODUCTION: Refractory or unexplained chronic cough (RUCC) is a common clinical problem with no effective diagnostic tools. The Sensations and Triggers Provoking Cough questionnaire (TOPIC) was developed to characterise cough in RUCC versus cough in other conditions. METHODS: Content analysis of participant interviews discussing the sensations and triggers of chronic cough informed TOPIC development. Participants with chronic cough completed the draft-TOPIC (a subset repeating 5-7 days later), St George's Respiratory Questionnaire (SGRQ), Cough Severity Diary (CSD) and Global Rating of Change Scale. The draft-TOPIC item list was reduced in hierarchical and Rasch analysis to refine the questionnaire to the TOPIC. RESULTS: 49 items describing the triggers and sensations of cough were generated from participant interviews (RUCC n=14, chronic obstructive pulmonary disease (COPD) n=11, interstitial lung disease (ILD) n=10, asthma n=11, bronchiectasis n=3, cystic fibrosis n=7). 140 participants (median age 60.0 (19.0-88.0), female 56.4%; RUCC n=39, ILD n=38, asthma n=45, COPD n=6, bronchiectasis n=12) completed draft-TOPIC, where items with poor 'fit' for RUCC were removed to create TOPIC (8 trigger items, 7 sensation items). Median TOPIC score was significantly higher in RUCC (37.0) vs ILD (24.5, p=0.009) and asthma (7.0, p<0.001), but not bronchiectasis (20.0, p=0.318) or COPD (18.5, p=0.238), likely due to small sample sizes. The Rasch model demonstrated excellent fit in RUCC (χ2=22.04, p=0.85; PSI=0.88); as expected. When all participant groups were included, fit was no longer demonstrated (χ2=66.43, p=0.0001, PSI=0.89) due to the increased heterogeneity (CI=0.077). TOPIC correlated positively with SGRQ (r=0.47, p<0.001) and CSD (r=0.63, p<0.001). The test-retest reliability of TOPIC (intraclass correlation coefficient) was excellent (r=0.90, p<0.001). CONCLUSIONS: High TOPIC scores in the RUCC patients suggest their cough is characterised by specific sensations and triggers. Validation of TOPIC in cough clinics may demonstrate value as an aid to identify features of RUCC versus cough in other conditions.

Bronchoscopy for refractory/unexplained cough with mucus.

INTRODUCTION: Refractory/unexplained cough (RUCC) is typically associated with throat symptoms and a dry cough. Some patients attending specialist cough clinics however, report sputum production (>1 tablespoon daily) and atypical sensations (urge-to-cough in chest). Bronchoscopy findings in this specific cohort have rarely been described. AIMS: We aimed to evaluate bronchoscopy, bronchoalveolar lavage (BAL) cell differential and microbiology findings in RUCC with mucus production. METHODS: We retrospectively reviewed case notes, procedure results and treatment of patients undergoing bronchoscopy for RUCC with more than a tablespoon of sputum daily. RESULTS: Data were included from 54 patients with RUCC, normal or trivial findings on CT (Computerised Tomography) imaging and no response to guideline-directed treatment of their cough. Most (84%) patients had BAL neutrophilia and excessive dynamic airway collapse (EDAC) was seen in 31%. Treatment strategies in these patients differed to those adopted in typical RUCC associated with a dry cough. Management was influenced or changed in 48/54 (89%) of the patients undergoing bronchoscopy. CONCLUSIONS: Bronchoscopy provides high diagnostic value in RUCC with mucus production (>1 tbsp daily), identifying specific treatable traits including neutrophilic airway inflammation and EDAC.

Primary Ewing sarcoma of the lung: a challenging case.

Ewing sarcoma usually develops in the second decade of life as a primary osseous malignancy. An extraskeletal primary source of this condition from the lung parenchyma is extremely uncommon. We report the case of a 33-year old man with primary Ewing sarcoma of the lung diagnosed upon postsurgical histological examination. Initially presenting with unilateral pleural effusion, our patient underwent various investigations before final diagnosis. This case report reviews the available literature of similarly reported cases and discusses the current developments on managing this rare and aggressive disease. As a supplementary learning point, this case reminds us always to consider unusual possibilities and seek further sub-specialist opinion when presented with unresolved clinical and radiological abnormalities that require further exploring beyond the primary line of investigations and treatment.