Exploring Clinical and Epidemiological Characteristics of Interstitial Lung Diseases: Rationale, Aims, and Design of a Nationwide Prospective Registry—The EXCITING-ILD Registry

Table 1

Comparison of the distribution of interstitial lung diseases (ILDs) in respiratory physicians’ prospective registries [18].

	Flanders (Belgium) (1992–1996)		Germany (1995)	Italy (1997–1999)	Spain/RENIA (1998–2000)	Spain/SEPAR (2000-2001)	Greece (2004)
	Prevalent cases	Incident cases	Incident cases	Prevalent cases	Incident cases	Incident cases	Prevalent cases	Incident cases
Subjects ()	362	264	234	1138	744	511	967	254
Idiopathic
Sarcoidosis	112 (31)	69 (26)	83 (35)	344 (30)	87 (12)	76 (15)	330 (34)	60 (23)
IPF/IIP	62 (17)	50 (19)	76 (32)	417 (37)	287 (39)	215 (42)	234 (24)	66 (25)
COP/BOOP	10 (2.3)	9 (3.4)	16 (6.8)	57 (5.0)	38 (5.1)	53 (10)	51 (5.3)	18 (7.0)
(C)EP	9 (2.2)	7 (2.7)		27 (2.3)	—	—	21 (2.2)	7 (2.7)
CTD	27 (7.5)	19 (7.2)	5 (2.1)	—	69 (9.3)	51 (19)	120 (12)	30 (12)
Vasculitis	5 (1.4)	4 (1.5)	2 (0.8)	25 (2.2)	—	—	14 (1.5)	6 (2.3)
EG/HX	13 (3.6)	7 (2.7)	—	73 (7.2)	6 (0.8)	15 (3)	37 (3.8)	7 (2.7)
Nonidiopathic
EAA	47 (13)	32 (12)	25 (11)	50 (4.3)	38 (5.1)	34 (7)	25 (2.6)	7 (2.7)
Drug	12 (3.3)	12 (5)	6 (2.6)	21 (1.8)		21 (4)	17 (1.8)	4 (1.5)
Pneumoconiosis^‡	19 (5.0)	18 (6.8)	6 (2.6)	—	55 (7.4)	—	20 (2.0)	8 (3.1)
Variable aetiology
Nonspecific fibrosis	33 (9.1)	27 (10)	12 (5.1)	—	69 (9.3)	—	82 (8.5)	40 (15)
Others	13 (3.8)	10 (3.8)	—	124 (11)	76 (10)	9 (2)	15 (1.5)	6 (2.3)

Data are presented as (%), unless otherwise stated. RENIA: Registry of Interstitial Pneumopathies of Andalusia; SEPAR: Sociedad Española de Neumología y Cirugía Torácica; IPF: idiopathic pulmonary fibrosis; IIP: idiopathic interstitial pneumonia; COP: cryptogenic organising pneumonia; BOOP: bronchiolitis obliterans organising pneumonia (not necessarily cryptogenic); (C)EP: (chronic) eosinophilic pneumonia; CTD: connective tissue disease; EG/HX: eosinophilic granuloma/histiocytosis X; EAA: extrinsic allergic alveolitis (hypersensitivity pneumonitis).
Goodpasture’s, granulomatosis with polyangiitis (Wegener’s), Churg-Strauss, and so forth.
Radiation was also included in the Italian and SEPAR registries.
Coal worker’s pneumoconiosis was excluded in the Flemish, Italian, and SEPAR registries.
Adapted from the European Lung White Book Chapter 22 [18].