
Figure 1
Classification of Idiopathic Interstitial Pneumonias. IIP: Idiopathic Interstitial Pneumonia.
Adapted from Am J Respir Crit Care Med. 2013, Vol 188, Iss. 6, pp. 733–748 [2].
Table 1
Prevalence and Incidence of Interstitial Lung Diseases in Developed and Developing Countries.
| Developed countries | Developing countries | |||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Europe | America | Asia | Asia | Europe/Asia | ||||||||||||
| Flanders (Belgium) 1992–1996 | Germany 1995 | Italy 1997–1999 | Spain/RENIA 1998–2000 | Spain/SEPAR 2000–2001 | Greece 2004 | Denmark 2003–2009 | EXCITING-ILD (Germany) 2014–2016 | New Mexico (United States of America) 1988–1990 | Saudi Arabia 2008–2011 | India 1997 | India Registry 2012–2015 | Turkey 2007–2009 | ||||
| Prevalent cases | Incident cases | Incident cases | Prevalent cases | Incident cases | Incident cases | Prevalent cases | Incident cases | Incident cases | Incident cases | Prevalent cases | Incident cases | Incident cases | Incident cases | Incident cases | Incident cases | |
| Subjects | 362 | 264 | 234 | 1138 | 744 | 511 | 967 | 254 | 431 | 201 | 258 | 202 | 330 | 260 | 1084 | 2245 |
| Unknown etiology | ||||||||||||||||
| Sarcoidosis | 112 (31) | 69 (26) | 83 (35) | 344 (30) | 87 (12) | 76 (15) | 330 (34) | 60 (23) | – | 46 (23) | 30 (11.6) | 16 (7.8) | 67 (20) | 140 (53.8) | 85 (7.8) | 771 (37.6) |
| IPF/IIP* | 62 (17) | 50 (19) | 76 (32) | 417 (37) | 287 (39) | 215 (42) | 234 (24) | 66 (25) | 121 (28)/186 (43) | 64 (32)/82 (41) | 58 (22.5) | 63 (31.2) | 77 (23.3)/108 (32.3) | 79 (30.4) | 148 (13.7) | 408 (19.9)/532 (26) |
| COP-BOOP | 10 (2.3) | 9 (3.4) | 16 (6.8) | 57 (5) | 38 (5.1) | 53 (10) | 51 (5.3) | 18 (7) | 10 (3) | 4 (2) | – | 1 (0.5) | 7 (2.1) | – | – | 58 (2.8) |
| (C)EP | 9 (2.2) | 7 (2.7) | – | 27 (2.3) | – | – | 21 (2.2) | 7 (2.7) | 4 (1) | – | 3 (1.2) | 1 (0.5) | 1 (0.3) | – | – | 19 (1) |
| CTD | 27 (7.5) | 19 (7.2) | 5 (2.1) | – | 69 (9.3) | 51 (19) | 120 (12) | 30 (12) | 54 (13) | 12 (6) | 33 (12.8) | 18 (9) | 115 (34.8) | 35 (13.5) | 151 (13.9) | 201 (9.8) |
| Vasculitis# | 5 (1.4) | 4 (1.5) | 2 (0.8) | 25 (2.2) | – | – | 14 (1.5) | 6 (2.3) | – | – | 2 (1.2) | 8 (4) | – | – | – | 42 (2) |
| EG-HX | 13 (3.6) | 7 (2.7) | – | 73 (7.2) | 6 (0.8) | 15 (3) | 37 (3.8) | 7 (2.7) | 8 (2) | – | 2 (0.8) | – | 1 (0.3) | – | – | 28 (1.3) |
| Exogenous etiology | ||||||||||||||||
| EAA (HP) | 47 (13) | 32 (12) | 25 (11) | 50 (4.3) | 38 (5.1) | 34 (7) | 25 (2.6) | 7 (2.7) | 32 (7) | 36 (18) | – | 3 (1.5) | 21 (6.4) | – | 513 (47.3) | 82 (4) |
| Drug¶ | 12 (3.3) | 12 (5) | 6 (2.6) | 21 (1.8) | – | 21 (4) | 17 (1.8) | 4 (1.5) | 20 (5) | 4 (2) | 6 (2.3) | 10 (5) | 4 (1.2) | 3 (1.2) | – | 71 (3.5) |
| Pneumoconiosis° | 19 (5) | 18 (6.8) | 6 (2.6) | – | 55 (7.4) | – | 20 (2) | 8 (3.1) | – | – | 36 (13.9) | 21 (10.4) | – | 3 (1.2) | – | 241 (11.8) |
| Variable etiology | ||||||||||||||||
| Non specific fibrosis | 33 (9.1) | 27 (10) | 12 (5.1) | – | 69 (9.3) | – | 82 (8.5) | 40 (15) | 62 (14) | 12 (6) | 43 (16.7) | 28 (13.9) | 6 (1.8) | – | – | – |
| Others | 13 (3.8) | 10 (3.8) | – | 124 (11) | 76 (10) | 9 (2) | 15 (1.5) | 6 (2.3) | 101 (25) | 9 (4) | 44 (17) | 33 (16.2) | 5 (1.5) | – | 187 (17.3) | 58 (2.7) |
[i] n: number of subjects. Data are presented as n (%), unless otherwise stated.
RENIA: Registry of Interstitial Pneumopathies of Andalusia; SEPAR: Sociedad Española de Neumología y Cirugía Torácica; EXCITING-ILD: Exploring Clinical and Epidemiological Characteristics of Interstitial Lung Diseases; IPF: idiopathic pulmonary fibrosis; IIP: idiopathic interstitial pneumonia; COP: cryptogenic organizing pneumonia; BOOP: bronchiolitis obliterans organizing pneumonia (not necessarily cryptogenic); (C)EP: (chronic) eosinophilic pneumonia; CTD: connective tissue disease; EG: eosinophilic granuloma; HX: histiocytosis X; EAA (HP): extrinsic allergic alveolitis (hypersensitivity pneumonitis).
* If there is data available from IIP, it will be shown separately, after the IPF data. The IPF is part of the IIP.
# Goodpasture’s, granulomatosis with polyangiitis (Wegener’s), Chrug-Strauss, etc.
¶ Radiation was also included in the Italian, SEPAR, US, India, and Turkey registries.
° Coal worker’s pneumoconiosis was excluded in the Flemish, Italian and SEPAR registries. The American and Turkish registries include occupational exposition. The Indian study (1997) includes only silicosis.
Table 2
Common Types of Hypersensitivity Pneumonitis According to Major Classes of Antigens.
| Class of antigens | Specific antigens | Sources | Type of disease |
|---|---|---|---|
| Organic particulate matters | |||
| Microbes | |||
| Bacteria | Saccharopolyspora rectivirgula, Thermoactinomyces vulgaris | Moldy hay, grain | Farmer’s lung |
| Fungus | Aspergillus species | Moldy hay, grain Moldy compost and mushrooms | Farmer’s lung Mushrooms worker’s lung |
| Trichosporon cutaneum | Contaminated houses | Japanese summer-type HP | |
| Penicillium species | Moldy cork Moldy cheese or cheese casings | Suberosis Cheese washer’s lung | |
| Alternaria species | Contaminated wood pulp or dust | Woodworker’s lung | |
| Mycobacteria | Mycobacterium avium-intracellulare | Mold on ceiling, tub water Mist from pool water, sprays and fountains | Hot tub lung Swimming pool lung |
| Proteins | |||
| Animal proteins | Proteins in avian droppings and serum and on feathers | Parakeets, budgerigars, pigeons, parrots, cockatiels, ducks | Pigeon breeder’s lung, bird fancier’s lung |
| Avian proteins | Feather beds, pillow, duvets | Feather duvet lung | |
| Silkworm proteins | Dust from silkworm larvae and cocoons | Silk production HP | |
| Plant’s proteins | Grain flour (wheat, rye, oats, maize) | Flour dust | Flour dust alveolitis |
| Legumes (soy) | Legumes (soy), flour dust | Soya dust alveolitis | |
| Wood (cabreuva, cedar, mahagony, pine, ramin, umbrella pine) | Wood particles | Wood fiber alveolitis | |
| Inorganic particulate matters | |||
| Chemicals products | Diisocyanates, trimellitic anhydride | Polyurethane foams, spray paints, dyes, glues | Chemical worker’s lung |
[i] HP: hypersensitivity pneumonitis.

Figure 2
Mechanisms by which Air Pollution Exposure Could Trigger Intertidal Lung Diseases. Modified from Chest 2015;147(4):1161–1167 [63].
PM2.5: particulate matter <2.5 um in aerodynamic diameter; PM10: particulate matter <10 um in aerodynamic diameter.
