Idiopathic pulmonary fibrosis in Sweden: report from the first year of activity of the Swedish IPF-Registry

Figures & data

Table 1 The Swedish Idiopathic Pulmonary Fibrosis Registry variables

Demographics	Gender
	Date of birth
	Age
	Reference hospital
Diagnosis (IPF)	Time of diagnosis
	ICD-10
	Base for diagnosis:  - Clinical–radiological  - Biopsy-proven  - Based on MDC
Clinical	Height, Weight, BMI
information	Smoking status:  - Ex-smoker  - Current smoker  - Never smoker
	Pack-year
	Profession, professional/environmental exposure
	Chronic comorbidities
	Ongoing treatments
	Main symptoms and time of onset
	Clinical findings:  - Presence of gastric reflux  - Drumstick fingers  - Crackles at auscultation
Lung function	Lung plethysmography: FEV1, FEV1%, FVC, FVC%, FEV1/FVC, TLC, TLC%
	Diffusion capacity: DlCO%
	Pulse oximetry at rest; arterial blood gas analysis
	6MWT: saturation at rest and lowest value during/after the test; walk distance
Radiology	Chest X-ray
	HR CT:  - UIP pattern  - Possible UIP pattern  - Inconsistent with UIP pattern
Pathology	BAL: total cell count, cell differentials
	Lung biopsy (open or VATS):  - UIP pattern  - Probable UIP-pattern  - Possible UIP-pattern  - Not UIP-pattern
Quality of life	K-BILD questionnaire
IPF therapy	Date of start, date of stop
	IPF specific therapies:  - Pirfenidone  - Nintedanib  - NAC  - Triple therapy
	Best supportive therapy:  - Long-term oxygen therapy  - Physiotherapy  - Psychological support  - Palliation (morphine, benzodiazepine)
	Adverse events:  - Date of onset  - Type of AE  - Severity (according to CTCAE)  - Intervention  - Suspension/modification of the therapy
Outcome	Death:  - Date of death  - Cause of death
	Transplantation:  - Date of transplantation
	Follow-up:  - Lung function testing  - Radiology  - Quality of life (K-BILD)  - Ongoing treatments/AEs/modification of IPF-therapies

IPF: idiopathic pulmonary fibrosis; ICD-10: International Statistical Classification of Diseases and Related Health Problems – Tenth Revision; MDC: multi-disciplinary conference; BMI: body mass index; FEV1: forced expiratory volume in 1 s; FEV1%: forced expiratory volume in 1 s, % of the expected value; FVC: forced volume capacity; FVC%: forced volume capacity, % of the expected value; TLC: total lung capacity; TLC%: total lung capacity, % of the expected value; DlCO%: Diffusion capacity of carbon monoxide, % of the expected value; 6MWT: 6-min walking test; HR CT: high-resolution computed tomography; UIP: usual interstitial pneumonia; BAL: bronchoalveolar lavage; VATS: video-assisted thoracoscopy; K-BILD: King's Brief Interstitial Lung Disease Questionnaire; NAC: N-acetyl-cysteine; AE: adverse event; CTCAE: Common Terminology Criteria for Adverse Events.

Fig. 1 The distribution of the IPF-patients recorded in the Swedish IPF-Registry between October 2014 and October 2015 according to month (x-axis) and participating centre (y-axis).

Table 2 Demographics and main clinical features of the patients included in the Swedish Idiopathic Pulmonary Fibrosis Registry

	Men	Women	Total
Patients (N, %)	50 (70.4%)	21 (29.6%)	71 (100)
Age (Median, range)	70 (47–86)	68.5 (51–84)	70 (47–86)
Smoking (N, %)	Never smokers: 12 (24%) Ex-smokers: 30 (60%) Current smokers: 0 (0%) Missing info: 8 (16%)	Never smokers: 8 (38.1%) Ex-smokers: 10 (47.6%) Current smokers: 3 (14.3%)	Never smokers: 20 (28.2%) Ex-smokers: 40 (56.4%) Current smokers: 3 (4%) Missing info: 8 (11.4%)
Pack-years among ex-smokers (M±SD)	24.59±14	11911±6	20.7914±6
BMI(M±SD)	26.8±3.3	26.8±3.3	27±4.5
Reflux (N,%)	10 (20%)	4 (19%)	14 (19.8%)
FEV1% of predicted (M±SD)	77.59±16.9	77.5±19	77.5±17.3
FVC% of predicted (M±SD)	70.2915	77.4920.4	72.3916.9
FEV1/FVC(M±SD)	82.2±5.8	80±6.2	81.5±6
TLC, % of predicted (M±SD)	64.5±14	62.2±12.2	70.6±17.3
DlCO, % of predicted (M±SD)	50.9±16.9	54.5±19.4	52.1±17.6

M, mean; SD, standard deviation; BMI, body mass index; FEV1%, forced expiratory volume in 1 s; FVC, forced vital capacity; TLC: total lung capacity; DlCO%: diffusing capacity of the lung for carbon monoxide.