Figures
- Figure 1
Proportion of the different rare lung disease among patients of general practitioners. IPF: idiopathic pulmonary fibrosis.
Tables
- Table 1
Adult questionnaire: GP distribution by country
Country Respondents n Italy 127 Portugal 10 Slovenia 7 UK 4 Spain 2 Netherlands 2 Turkey 1 Switzerland 1 Sweden 1 Poland 1 Finland 1 Pakistan 1 Mexico 1 India 2 Germany 1 Egypt 1 Cyprus 1 Unknown 6 - Table 2
Paediatric questionnaire: GP distribution by country
Country Respondents n Italy 28 Portugal 8 UK 3 Netherlands 2 Sweden 1 Slovenia 1 Switzerland 1 Egypt 1 Pakistan 1 - Table 3
Number of patients (adult and paediatric) per GP
Patients in GP’s care GPs n % of all GPs# % of GPs who responded to the question Data missing 8 4.1 >1500 73 37.2 38.83 1500–1001 66 33.7 35.11 1000–501 38 19.4 20.21 500–250 11 5.6 5.85 Total 196 100.0 100.0 - Table 4
Proportion of paediatric patients (aged 0–17 years) in the care of GPs with both adult and paediatric patients
Paediatric patients % GPs n % of all GPs# % of GPs who responded to the question Data missing 87 44.4 0 13 6.6 11.93 5–10 47 24.0 43.12 10–15 12 6.1 11.01 15–20 11 5.6 10.09 50–100 26 13.3 23.85 Total 196 100.0 100.0 - Table 5
Rare lung diseases present among patients of GPs surveyed
Rare lung disease Patients n Sarcoidosis 15 CF 14 Idiopathic pulmonary fibrosis 13 AATD 10 LAM 7 Other rare lung diseases# Pulmonary alveolar proteinosis 6 Bronchiectasis 6 PAH 5 Interstitial lung disease 3 Langerhans cell histiocytosis 2 Others 28 Total 109 #: n=50.
- Table 6
Results of the questionnaire relating to AATD
Wrong responses What are the main symptoms/signs/manifestations that could be suggestive AATD? Wrong option: osteomyelitis 53% All other options: grading=0 10 (0–53)% What type of pattern of lung/respiratory function test would be suggestive of AATD? Wrong option: restrictive pattern 68% All other options: grading=0 11 (2.5–68)% What type of blood lab examination is suggestive of AATD? Wrong option: increased peripheral blood α1-antitrypsin 21% All other options: grading=0 10 (5–23)% Data are presented as median (min–max), unless otherwise stated. Definition of wrong option/answer: responses were considered wrong when a grade >1 was attributed. The percentage of wrong responses was calculated on valid responses (i.e. after missing and “I don’t know” responses were excluded) and is the sum of two contributions. Wrong option: responses were considered wrong when a grade >0 was attributed to the wrong option in the questionnaire. All other options: responses were considered wrong when a grade = 0 was attributed to the other options in the questionnaire. The summary figure is reported in terms of median (minimum–maximum) of the percentages of wrong responses to each option.
- Table 7
Results of the paediatric questionnaire relating to PAH and LAM
Wrong responses What are the main symptoms/manifestations that could be suggestive of PAH? Wrong option: vision abnormalities 62% All other options: grading=0 10 (1–62)% What are the main findings that could be suggestive of PAH? Wrong option: loose shoulders 49% All other options: grading=0 4 (0–49)% What type of pattern of lung/respiratory function abnormalities would be suggestive of PAH? Wrong option: sleep apnoea/hypopnea 84% All other options: grading=0 26 (1–84)% What are the main symptoms/signs/manifestations that could be suggestive of LAM? Wrong option: erythema nodosum 64% All other options: grading=0 9 (1–64)% What type of pattern of lung function test would be suggestive of LAM? Wrong option: respiratory acidosis 81% All other options: grading=0 11 (3–81)% What type of finding at imaging studies would be suggestive of LAM? Wrong option: cerebrovascular abnormalities on brain CT 60% All other options: grading=0 12 (3–61)% Data are presented as median (min–max), unless otherwise stated. The percentage of wrong responses was calculated on valid responses (i.e. after missing and “I don’t know” responses were excluded) and is the sum of two contributions. Wrong option: responses were considered wrong when a grade >0 was attributed to the wrong option in the questionnaire. All other options: responses were considered wrong when a grade = 0 was attributed to the other options in the questionnaire. The summary figure is reported in terms of median (minimum–maximum) of the percentages of wrong responses to each option. CT: computed tomography.
- Table 8
Results of the questionnaire relating to rare lung diseases
Wrong responses Which findings of imaging suggest CF? Wrong option: bilateral pleural effusion 79% All other options: grading=0 5 (0–13)% Which findings of imaging suggest PCD? Wrong option: biliary stones 78% All other options: grading=0 0 (0–18)% Which investigations should be undertaken to diagnose PCD? Wrong option: arterial blood gas analysis 83% All other options: grading=0 5 (0–9)% What are the main symptoms/signs/manifestations that could be suggestive of lung disease in a child with immunodeficiency? Wrong option: hyperhidrosis 88% All other options: grading=0 0 (0–8)% What are the main physical signs and findings that could be suggestive of lung disease due to immunodeficiency? Wrong option: normal physical examination 70% All other options: grading=0 5 (0–22)% Data are presented as median (min–max), unless otherwise stated. The percentage of wrong responses was calculated on valid responses (i.e. after missing and “I don’t know” responses were excluded) and is the sum of two contributions. Wrong option: responses were considered wrong when a grade >0 was attributed to the wrong option in the questionnaire. All other options: responses were considered wrong when a grade = 0 was attributed to the other options in the questionnaire. The summary figure is reported in terms of median (minimum–maximum) of the percentages of wrong responses to each option.
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