Idiopathic Interstitial Pneumonias

  • Paul J. LeeEmail author
  • Jeffrey P. Kanne
Part of the Respiratory Medicine book series (RM)


The idiopathic interstitial pneumonias are a distinct group of clinicopathologic entities. High-resolution computed tomography (HRCT) plays a critical role in the evaluation and management of patients. In the appropriate clinical setting, characteristic HRCT findings may be diagnostic, obviating the need for open lung biopsy. In more challenging or complicated cases, consensus among the clinician, radiologist, and pathologist may be required. This chapter describes and depicts the characteristic HRCT features of usual interstitial pneumonia, nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, respiratory bronchiolitis, respiratory bronchiolitis associated interstitial lung disease, desquamative interstitial pneumonia, and lymphoid interstitial pneumonia.


Interstitial lung disease (ILD) High-resolution computed tomography (HRCT) Usual interstitial pneumonia (UIP) Nonspecific interstitial pneumonia (NSIP) Cryptogenic organizing pneumonia (COP) Respiratory bronchiolitis (RB) Respiratory bronchiolitis–interstitial lung disease (RB-ILD) Desquamative interstitial pneumonia (DIP) Lymphoid interstitial pneumonia (LIP) 


Over 200 causes of interstitial lung disease have been described. Environmental and occupational exposures, systemic diseases, and genetic causes can all result in a variety of interstitial lung diseases. The idiopathic interstitial pneumonias (IIP) refer to a group of distinct clinicopathologic entities without known causes [1]. Classification of IIP has undergone several iterations as understanding of these entities evolves [2, 3, 4]. The most common IIP is idiopathic pulmonary fibrosis (IPF), which accounts for approximately 40% of all idiopathic interstitial lung disease [5]. While idiopathic forms do exist, the other IIPs more commonly result from exposures, such as tobacco smoke or connective tissue disease.

High-resolution computed tomography (HRCT) plays a key role in assessing the patient with known or suspected interstitial lung disease. While the primary function of HRCT is to distinguish patients with usual interstitial pneumonia (UIP), which is associated with IPF, from those without UIP, HRCT findings can often suggest other causes of diffuse lung disease. Although surgical biopsy is advocated in patients with suspected IIP who do not have a definite UIP pattern on HRCT, consensus between clinicians and radiologists with expertise in interstitial lung disease may suffice to establish a diagnosis.

Usual Interstitial Pneumonia

IPF is uncommon with an incidence of 6.8–16.3 per 100,000 persons in the USA [6]. Men are affected twice as often as women, and the incidence increases with increasing age. While the exact cause of IPF remains unknown, environmental factors such as occupational dusts and fumes [7], cigarette smoke [8], and Epstein-Barr virus infection [9] may be contributory. Prognosis remains poor with fewer than 50% of patients surviving 5 years following diagnosis [10].

IPF is characterized histologically by UIP. The UIP pattern on HRCT consists of reticular opacities, traction bronchiectasis, and architectural distortion in a patchy subpleural and bibasilar distribution (Fig. 12.1) [11, 12]. These findings may be asymmetric but not unilateral. Honeycombing is a critical component of a confident, radiologic diagnosis of UIP (Figs. 12.2, 12.3). Honeycombing consists of clustered cystic spaces stacked upon a subpleural base. These cystic spaces have well-defined walls and typically range from 3 to 10 mm in size but may extend up to 2.5 cm in diameter [1, 5, 13]. Centrilobular and paraseptal emphysema may coexist with CT findings of UIP and should not be mistaken for honeycombing (Fig. 12.4) [8, 14, 15, 16, 17, 18]. Mild mediastinal lymphadenopathy is also common, typically involving the right paratracheal and subcarinal stations. The degree of lymphadenopathy typically correlates with the extent of parenchymal reticulation, although short axis lymph node measurements rarely exceed 1.5–2.0 cm in the absence of another superimposed cause [19, 20]. Ground-glass opacities can be associated with areas of fibrosis and may even represent regions of fibrosis. However, ground-glass opacities should account for a relatively minor component of parenchymal abnormality.
Fig. 12.1

Usual interstitial pneumonia. Transverse (a) and coronal reformatted (b) low-dose HRCT images show subpleural and basal reticulation (arrows)

Fig. 12.2

Usual interstitial pneumonia. Transverse (a) and coronal reformatted (b) low-dose HRCT images show subpleural and basal predominant reticulation with subpleural honeycombing (arrowheads)

Fig. 12.3

Usual interstitial pneumonia. Transverse HRCT image shows extensive, symmetric honeycombing (arrowheads) in the lung bases

Fig. 12.4

Combined pulmonary fibrosis and emphysema. Transverse (a) and coronal reformatted (b) HRCT images show upper lobe predominant bullous emphysema (arrows) and basal predominant subpleural honeycombing (arrowheads)

Studies have shown that the positive predictive value of a confident CT diagnosis of UIP by expert pulmonary radiologists ranges from 90 to 100%. However, these studies also show that a confidant diagnosis is not made in 25–50% of histologically proven cases of UIP [21, 22, 23, 24, 25, 26, 27]. Silva et al. demonstrated that the findings of basal honeycombing, the absence of subpleural sparing, and the absence of centrilobular nodules best distinguish UIP from chronic hypersensitivity pneumonitis and nonspecific interstitial pneumonia (NSIP) [28]. CT findings that should suggest alternative etiologies include micronodules; air trapping; non-honeycomb cysts; consolidation; a peribronchovascular distribution of fibrosis, and significant pleural calcifications, plaques, or effusion [5].

Complications associated with IPF include acute exacerbation (Fig. 12.5), pulmonary hypertension [29, 30, 31], pulmonary thromboembolism, coronary artery disease, bronchogenic carcinoma (Fig. 12.6), pneumothorax (Fig. 12.7), pneumomediastinum, and infection. Opportunistic organisms in this setting include Mycobacteria, Pneumocystis jiroveci, and Aspergillus species [32, 33]. Acute exacerbation occurs in approximately 5–10% of patients on an annual basis. Associated CT findings include new ground-glass opacities and consolidation in a peripheral, diffuse, or multifocal random distribution (Fig. 12.8). It is unclear whether these acute exacerbations represent rapid advancement of the primary disease or superimposed infection, infarction, or other pathology difficult to separate from the primary process [34, 35, 36, 37, 38, 39]. Pulmonary hypertension is commonly associated with IPF, although CT-derived vascular measurements may be of limited predictive value [40]. Bronchogenic carcinoma (Fig. 12.9) develops in approximately 10–15% of those with relatively longstanding IPF and usually manifests as new focal consolidation or nodule in a region of severe fibrosis [41]. The appropriate role of routine screening for these complications has not been defined [5].
Fig. 12.5

Acute exacerbation of UIP. a HRCT image shows subpleural predominant reticulation with mild traction bronchiectasis (arrows) and bronchiolectasis (arrowheads). A small amount of ground-glass opacity is present. b HRCT 9 days later shows development of extensive ground-glass opacity

Fig. 12.6

UIP with bronchogenic carcinoma. HRCT image shows a large nodule (arrow) on a background of subpleural reticulation and honeycombing. Transthoracic needle biopsy showed non-small cell lung carcinoma

Fig. 12.7

NSIP associated with systemic sclerosis. HRCT image shows extensive ground-glass opacity. There is mild bronchial dilation (arrowheads). Note the patulous and fluid-filled esophagus (arrow)

Fig. 12.8

NSIP associated with mixed connective tissue disease. HRCT image shows mild ground-glass opacity and reticulation in the lower lobes associated with mild traction bronchiectasis (arrowheads). Note the patulous esophagus (thin arrow). Mild subpleural sparing is evident (wide arrows)

Fig. 12.9

NSIP associated with undefined connective tissue disease. HRCT image shows ground-glass opacity with reticulation in the lower lungs. Note the rim of relative subpleural sparing (arrows)

Nonspecific Interstitial Pneumonia

Nonspecific interstitial pneumonia (NSIP) was first used to classify patients with interstitial lung disease and surgical biopsies not fitting into any well-defined histologic pattern [3]. Currently, idiopathic NSIP is recognized as a distinct clinicopathologic entity associated with a better prognosis than IPF. Patients with idiopathic NSIP tend to be lifetime non-smoking females of Asian ethnicity [42]. However, NSIP is most commonly associated with connective tissue disease such as systemic sclerosis or may be the sequela of a drug reaction.

While the HRCT findings of NSIP have proven to be more variable than was initially suggested [43, 44, 45, 46], the majority of cases have ground-glass opacities and findings of fibrosis including fine reticulation, traction bronchiectasis, and volume loss in a symmetric mid to lower lung distribution (Figs. 12.7, 12.8) [47, 48]. The severity and distribution of these parenchymal abnormalities are more uniform than in UIP. Distribution along the axial plane may be peripheral, peribronchovascular, or diffuse. When present and in the appropriate setting, a thin rim of subpleural sparing is a rather specific finding for NSIP and should heighten diagnostic confidence (Fig. 12.9) [43]. As with IPF, mild mediastinal lymphadenopathy is common, particularly at the right paratracheal and subcarinal stations, and correlates with the degree of parenchymal involvement. Given the high association of NSIP with underlying collagen vascular diseases and other disorders, associated abnormalities may be sought on available imaging studies.

Although NSIP is characterized by two histologic subtypes, cellular and fibrotic, there are generally no imaging features to reliably distinguish between the two. As would be expected, reticulation is more commonly seen with fibrotic subtype, although the cellular subtype may also demonstrate fine reticulation. In our experience, a posterior basilar and anterior apical distribution of reticulation, when present in the appropriate setting, is relatively specific for the fibrotic subtype. However, with regard to the extent of ground-glass opacity, consolidation, or traction bronchiectasis, there is no reliable separation in CT findings between the two subtypes [10, 49, 50, 51].

Additional findings on HRCT tend to be nonspecific or should suggest alternative etiologies. Relatively mild honeycombing may be seen in NSIP, but significant honeycombing is more commonly associated with a histologic diagnosis of UIP. The reported prevalence of consolidation in NSIP varies widely and may often represent superimposed infection or organizing pneumonia [10, 44, 48, 49, 51, 52, 53, 54, 55, 56, 57]. New consolidation or ground-glass opacities in an acutely ill patient with NSIP may represent an acute exacerbation (Fig. 12.10). While such exacerbations occur less commonly than in patients with IPF, the annual incidence of NSIP acute exacerbations may approach 5% [58]. With appropriate treatment, some findings of NSIP may be reversible on follow-up examination [59].
Fig. 12.10

Acute exacerbation of NSIP. a HRCT image shows multiple foci of peripheral ground-glass opacity (arrows). b HRCT image one month later shows extensive consolidation in both lungs. The patient presented with acute hypoxia and dyspnea

Cryptogenic Organizing Pneumonia

Organizing pneumonia (OP) refers to the histologic pattern described as polypoid plugs of loose organizing connective tissue within the alveoli. This pattern may be idiopathic but is more commonly associated with one of numerous underlying etiologies, including connective tissue disease and drug reaction. Formerly termed bronchiolitis obliterans organizing pneumonia (BOOP), cryptogenic OP (COP) refers to an idiopathic clinical syndrome characterized by organizing pneumonia. The newer terminology reflects the primary histologic features while avoiding confusion with small airways disease (bronchiolitis). Patients with COP are often initially diagnosed with community acquired pneumonia, and further workup is often pursued after failure of antibiotic treatment.

Characteristic HRCT findings of OP include unilateral or, more commonly, bilateral, patchy areas of consolidation, characteristically involving the lower lobes. Consolidation is present on HRCT in at least 90% of patients and follows a subpleural or peribronchovascular distribution in approximately 50% of patients (Fig. 12.11) [60, 61]. Foci of consolidation may be migratory on serial imaging. Common associated findings include ground-glass opacities in a random distribution (60%) (Fig. 12.12), air bronchograms (Fig. 12.13), and mild bronchial dilation. Lung volumes are typically preserved, and architectural distortion is generally absent. Small pleural effusions may occur in a small minority of patients. The reverse-halo sign (Fig. 12.14) was first described in the setting of OP as a relatively specific finding. This sign refers to a central region of ground-glass opacity surrounded by a > 2 mm crescent or ring of denser consolidation. Kim et al. reported the reverse-halo sign in 19% of biopsy proven cases of OP [62]. However, a reverse halo sign has since been reported in numerous separate entities [62]. Reticulation and honeycombing are uncommon to rare but when present are associated with an increased risk of progressive disease [63, 64, 65].
Fig. 12.11

Cryptogenic organizing pneumonia. HRCT image shows peripheral consolidation (arrows) in the lower lobes with a few foci of ground-glass opacity (arrowheads)

Fig. 12.12

Cryptogenic organizing pneumonia. HRCT image shows scattered foci of ground-glass opacity (arrows) in both lungs. Consolidation is more common than ground-glass opacity in COP

Fig. 12.13

Cryptogenic organizing pneumonia. HRCT image shows multiple foci of peripheral and peribronchial consolidation with air bronchograms (arrows)

Fig. 12.14

Cryptogenic organizing pneumonia. HRCT image shows foci of consolidation (wide arrows) and ground-glass opacity (arrowhead) and a reverse halo sign (thin arrow), the latter characterized by central ground-glass opacity and a rim of consolidation

With treatment, clinical and radiologic response is often striking. Symptomatic improvement may be expected within 1–2 days, while radiologic resolution occurs over several weeks (Fig. 12.15) [66]. Although relapse is not common, it does not worsen the long-term prognosis.
Fig. 12.15

Resolving cryptogenic organizing pneumonia. a HRCT image shows scattered of lung consolidation (arrows) in a peripheral and peribronchial distribution. b HRCT image obtained 2 weeks later shows patchy ground-glass opacity (arrows) where consolidation once was

Respiratory Bronchiolitis, Respiratory Bronchiolitis–Interstitial Lung Disease, and Desquamative Interstitial Pneumonia

Respiratory bronchiolitis (RB), RB-associated interstitial lung disease (RB-ILD), and desquamative interstitial pneumonia (DIP) are included in the most recent IIP classification despite being almost invariably associated with cigarette smoking. RB, RB-ILD, and DIP represent a spectrum of smoking-related interstitial lung disease characterized by the accumulation of pigmented macrophages in the respiratory bronchioles and alveoli [67]. With RB, pigmented macrophages are limited primarily to the respiratory bronchioles whereas macrophage accumulation is more extensive with RB-ILD and diffuse in DIP. By definition, patients with RB are asymptomatic while those with RB-ILD and DIP may present with dyspnea, cough, and abnormal pulmonary function tests [68].

Characteristic HRCT findings of RB include mild, poorly defined centrilobular nodules in a mid and upper lung distribution (Fig. 12.16) with scattered, patchy ground-glass opacities [69]. Associated findings include occasional lobular air trapping and emphysema. With RB-ILD, the extent of poorly defined centrilobular nodules is greater (Fig. 12.17), and more extensive patchy ground-glass opacity may be present (Fig. 12.18) [70]. With smoking cessation, these parenchymal abnormalities are reversible to some degree [71].
Fig. 12.16

Respiratory bronchiolitis. HRCT image shows scattered, poorly defined ground-glass attenuation nodules (arrows) in the upper lobes of this cigarette smoker

Fig. 12.17

RB-ILD. HRCT image shows diffuse, poorly defined centrilobular nodules (arrowheads) in the upper lobes of this heavy cigarette smoker who presented with dyspnea and cough

Fig. 12.18

RB-ILD. HRCT image shows patchy ground-glass opacities in the upper lobes of this heavy cigarette smoker who presented with dyspnea

DIP is rare and associated with more severe and diffuse parenchymal abnormality [72]. HRCT findings consist of diffuse ground-glass opacities with peripheral and lower lung predominance (Fig. 12.19) [12]. Reticular opacities are common but are often relatively mild and limited to a bibasilar distribution (Fig. 12.20). Small well-defined cysts may occur in areas of ground-glass opacity [73]. Centrilobular nodules are uncommon, while CT findings of respiratory bronchiolitis tend to be less severe than in RB-ILD [74]. DIP on CT may be indistinguishable from NSIP, acute or subacute hypersensitivity pneumonitis, and infections such as Pneumocystis jiroveci [67].
Fig. 12.19

Desquamative interstitial pneumonia in a heavy smoker. Low-dose HRCT shows extensive ground-glass opacity in the periphery of the lung bases. The HRCT findings of DIP can overlap those of NSIP

Fig. 12.20

Desquamative interstitial pneumonia in a heavy smoker. HRCT image shows patchy ground-glass opacity in the lower lobes. Mild subpleural reticulation (arrow) is present

Acute Interstitial Pneumonia

Acute interstitial pneumonia (AIP) is diagnostically reserved for diffuse alveolar damage of unknown origin. Histologically, AIP is indistinguishable from acute respiratory distress syndrome (ARDS) secondary to shock or sepsis. CT findings in the early exudative phase include dependent, subpleural consolidation and ground-glass opacities in a geographic, patchy, symmetric, and bilateral distribution (Fig. 12.21). Multiple foci of lobular sparing contribute to a geographic pattern. Greater than 50% of the lung parenchyma is typically involved [75].
Fig. 12.21

Acute interstitial pneumonia. Low-dose HRCT image shows diffuse ground-glass opacity with superimposed septal thickening in the anterior lungs and consolidation in the dependent lungs

In the subacute, organizing stage, traction bronchiectasis and architectural distortion progress with the duration of illness (Fig. 12.22). These findings are characteristically prominent relative to any degree of reticulation or honeycombing [76]. Pleural effusions are found in approximately 30%. While typically indistinguishable from ARDS, AIP is more commonly lower lobe predominant in distribution with a greater prevalence of fibrotic changes [77, 78]. As with UIP and NSIP, the degree of fibrotic changes positively correlates with a worsened prognosis. ARDS, conversely, is more commonly associated with interlobular septal thickening. Differential considerations for an AIP/ARDS pattern include pulmonary edema, widespread infection, pulmonary hemorrhage, and acute eosinophilic pneumonia [67, 76, 79].
Fig. 12.22

Organizing phase of acute interstitial pneumonia. HRCT image shows ground-glass opacity and septal thickening (arrowheads) with dependent consolidation. Traction bronchiectasis (arrows) suggests developing fibrosis, although this is sometimes reversible

If a patient with AIP recovers, consolidation and ground-glass opacities typically clear in a progressive fashion, while residual areas of reticulation and hypoattenuation remain, particularly in the nondependent lung [76].

Lymphoid Interstitial Pneumonia

Lymphoid interstitial pneumonia (LIP) is a rare diagnosis most commonly seen in patients with Sjögren syndrome. Additional associations include other connective tissue and autoimmune diseases as well as AIDS, the latter particularly in children. Despite being a lymphoproliferative disorder, LIP is included in the most recent classification system as an interstitial lung disease, given its inclusion in associated differential diagnoses of diffuse lung disease and its histologic pattern, which is characterized by a polyclonal lymphocytic and plasma cell infiltration of the alveolar septum [1].

The common HRCT findings of LIP include bilateral ground-glass opacity, thin-walled perivascular cysts located predominantly within the lower lobes, and poorly defined centrilobular nodules (Figs. 12.23, 12.24, 12.25). The cysts tend to range from 1 mm to 30 mm in diameter and are typically fewer in number compared with lymphangioleiomyomatosis. Less common findings include perilymphatic nodules and septal and bronchovascular thickening [67, 80, 81, 82]. Nodules or calcifications may develop in cysts, reflecting amyloid deposition.
Fig. 12.23

Lymphoid interstitial pneumonia and Sjögren syndrome. HRCT image shows numerous thin-walled cysts (arrows) with adjacent vessels (arrowheads)

Fig. 12.24

Lymphoid interstitial pneumonia and Sjögren syndrome. HRCT image shows scattered thin-walled cysts (arrows) and scattered ground-glass attenuation opacities (arrowheads)

Fig. 12.25

Lymphoid interstitial pneumonia and Sjögren syndrome. HRCT image shows extensive ground-glass opacity, subpleural nodules (arrowheads), and scattered thin-walled cysts


In summary, HRCT is critical to evaluating patients with known or suspected diffuse lung disease. In patients with the appropriate clinical presentation, a highly confident diagnosis of UIP on HRCT is sufficient to establish a diagnosis of IPF. HRCT may also suggest the presence of other interstitial or diffuse lung diseases. Finally, consensus among the clinician, radiologist, and pathologist may be required to establish a diagnosis when the pieces of the diagnostic puzzle are not straightforward.


  1. 1.
    American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med. 2002;165(2):277–304.Google Scholar
  2. 2.
    Liebow AA, Carington CB. The interstitial pneumonias. In: Simon M, Potchen EJ, LeMay M, editors. Frontiers in pulmonary radiology. New York: Grune and Stratton; 1969. p. 102–41.Google Scholar
  3. 3.
    Katzenstein AL. Katzenstein and askin’s surgical pathology of non-neoplastic lung disease. Philadelphia: WB Saunders; 1997.Google Scholar
  4. 4.
    Müller NL, Colby TV. Idiopathic interstitial pneumonias: high-resolution CT and histologic findings. Radiographics. 1997;17:1016–22.PubMedGoogle Scholar
  5. 5.
    Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788–824.PubMedCrossRefGoogle Scholar
  6. 6.
    Raghu G, Weycker D, Edelsberg J, et al. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2006;174:810–6.PubMedCrossRefGoogle Scholar
  7. 7.
    Baumgartner KB, Samet JM, Coultas DB, et al. Occupational and environmental risk factors for idiopathic pulmonary fibrosis: a multicenter case-control study. Collaborating Centers. Am J Epidemiol. 2000;152:307–15.PubMedCrossRefGoogle Scholar
  8. 8.
    Baumgartner KB, Samet JM, Stidley CA, et al. Cigarette smoking: a risk factor for idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1994;150:670–5.Google Scholar
  9. 9.
    Kelly BG, Lok SS, Hasleton PS, et al. A rearranged form of Epstein-Barr virus DNA is associated with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2002;166:510–3.PubMedCrossRefGoogle Scholar
  10. 10.
    Travis WD, Matsui K, Moss J, et al. Idiopathic nonspecific interstitial pneumonia: prognostic significance of cellular and fibrosing patterns: survival comparison with usual interstitial pneumonia and desquamative interstitial pneumonia. Am J Surg Pathol. 2000;24:19–33.PubMedCrossRefGoogle Scholar
  11. 11.
    Nishimura K, Kitaichi M, Izumi T, et al. Usual interstitial pneumonia: histologic correlation with high-resolution CT. Radiology. 1992;182:337–42.PubMedGoogle Scholar
  12. 12.
    Johkoh T, Muller NL, Cartier Y, et al. Idiopathic interstitial pneumonias: diagnostic accuracy of thin-section CT in 129 patients. Radiology. 1999;211:555–60.PubMedGoogle Scholar
  13. 13.
    American Thoracic Society; European Respiratory Society. Idiopathic pulmonary fibrosis: diagnosis and treatment: international consensus statement. Am J Respir Crit Care Med. 2000;161:646–64.Google Scholar
  14. 14.
    Iwai K, Mori T, Yamada N, et al. Idiopathic pulmonary fibrosis: epidemiologic approaches to occupational exposure. Am J Respir Crit Care Med. 1994;150:670–5.PubMedGoogle Scholar
  15. 15.
    Enomoto T, Usuki J, Azuma A, et al. Diabetes mellitus may increase risk for idiopathic pulmonary fibrosis. Chest. 2003;123:2007–11.PubMedCrossRefGoogle Scholar
  16. 16.
    Steele MP, Speer MC, Loyd JE, et al. Clinical and pathologic features of familial interstitial pneumonia. Am J Respir Crit Care Med. 2005;172:1146–52.PubMedCrossRefGoogle Scholar
  17. 17.
    Miyake Y, Sasaki S, Yokoyama T, et al. Occupational and environmental factors and idiopathic pulmonary fibrosis in Japan. Ann Occup Hyg. 2005;49:259–65.PubMedCrossRefGoogle Scholar
  18. 18.
    Taskar VS, Coultas DB. Is idiopathic pulmonary fibrosis an environmental disease? Proc Am Thorac Soc. 2006;3:293–8.PubMedCrossRefGoogle Scholar
  19. 19.
    Hwang JH, Misumi S, Sahin H, et al. Computed tomographic features of idiopathic fibrosing interstitial pneumonia: comparison with pulmonary fibrosis related to collagen vascular disease. J Comput Assist Tomogr. 2009;33:410–5.PubMedCrossRefGoogle Scholar
  20. 20.
    Souza CA, Muller NL, Lee KS, et al. Idiopathic interstitial pneumonias: prevalence of mediastinal lymph node enlargement in 206 patients. AJR. 2006;186:995–9.PubMedCrossRefGoogle Scholar
  21. 21.
    Maher TM. The diagnosis of idiopathic pulmonary fibrosis and its complications. Expert Opin Med Diagn. 2008;2:1317–31.CrossRefGoogle Scholar
  22. 22.
    Mathieson JR, Mayo JR, Staples CA, et al. Chronic diffuse infiltrative lung disease: comparison of diagnostic accuracy of CT and chest radiography. Radiology. 1989;171:111–6.PubMedGoogle Scholar
  23. 23.
    Raghu G, Mageto YN, Lockhart D, et al. The accuracy of the clinical diagnosis of new-onset idiopathic pulmonary fibrosis and other interstitial lung disease: A prospective study. Chest. 1999;116:1168–74.PubMedCrossRefGoogle Scholar
  24. 24.
    Hunninghake GW, Zimmerman MB, Schwartz DA, et al. Utility of lung biopsy for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2001;164:193–6.PubMedGoogle Scholar
  25. 25.
    Grenier P, Valeyre D, Cluzel P, et al. Chronic diffuse interstitial lung disease: diagnostic value of chest radiography and high-resolution CT. Radiology. 1991;179:123–32.PubMedGoogle Scholar
  26. 26.
    Lee KS, Primack SL, Staples CA, et al. Chronic infiltrative lung disease: comparison of diagnostic accuracies of radiography and low- and conventional-dose thin-section CT. Radiology. 1994;191:669–73.PubMedGoogle Scholar
  27. 27.
    Swensen SJ, Aughenbaugh GL, Myers JL. Diffuse lung disease: diagnostic accuracy of CT in patients undergoing surgical biopsy of the lung. Radiology. 1997;205:229–34.PubMedGoogle Scholar
  28. 28.
    Silva C, Muller NL, Lynch DA, et al. Chronic hypersensitivity pneumonitis: differentiation from idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia by using thin-section CT. Radiology. 2008;246(1):288–97.PubMedGoogle Scholar
  29. 29.
    Mejia M, Carrillo G, Rojas-Serrano J, et al. Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension. Chest. 2009;136:10–5.PubMedCrossRefGoogle Scholar
  30. 30.
    Nadrous HF, Pellikka PA, Krowka MJ, et al. The impact of pulmonary hypertension on survival in patients with idiopathic pulmonary fibrosis. Chest. 2005;128:616S–7S.PubMedCrossRefGoogle Scholar
  31. 31.
    Lettieri CJ, Nathan SD, Barnett SD, et al. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest. 2006;129:746–52.PubMedCrossRefGoogle Scholar
  32. 32.
    Martinez FJ, Safrin S, Weycker D, et al. The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med. 2005;142:963–7.PubMedGoogle Scholar
  33. 33.
    Panos RJ, Mortenson RL, Niccoli SA. Clinical deterioration in patients with idiopathic pulmonary fibrosis: causes and assessment. Am J Med. 1990;88:396–404.PubMedCrossRefGoogle Scholar
  34. 34.
    Papiris SA, Manali ED, Roussos C, et al. Clinical review: idiopathic pulmonary fibrosis acute exacerbations–unravelling Ariadne’s thread. Crit Care. 2010;14(6):246.PubMedCrossRefGoogle Scholar
  35. 35.
    Collard HR, Moore BB, Flaherty KR, et al. Idiopathic Pulmonary Fibrosis Clinical Research Network Investigators, Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2007;176:636–43.PubMedCrossRefGoogle Scholar
  36. 36.
    Kim DS, Park JH, Park BK, et al. Acute exacerbation of idiopathic pulmonary fibrosis: frequency and clinical features. Eur Respir J. 2006;27:143–50.PubMedCrossRefGoogle Scholar
  37. 37.
    Parambil JG, Myers JL, Ryu JH. Histopathologic features and outcome of patients with acute exacerbation of idiopathic pulmonary fibrosis undergoing surgical lung biopsy. Chest. 2005;128:3310–5.PubMedCrossRefGoogle Scholar
  38. 38.
    Rice AJ, Wells AU, Bouros D, et al. Terminal diffuse alveolar damage in relation to interstitial pneumonias: an autopsy study. Am J Clin Pathol. 2003;119:709–14.PubMedCrossRefGoogle Scholar
  39. 39.
    Churg A, Muller NL, Silva CIS, et al. Acute exacerbation (acute lung injury of unknown cause) in UIP and other forms of fibrotic interstitial pneumonias. Am J Surg Pathol. 2007;31:277–84.PubMedCrossRefGoogle Scholar
  40. 40.
    Alhamed EH, Al-Boukai AA, Shaik SA, et al. Prediction of Pulmonary Hypertension in Patients with or without Interstitial Lung Disease: Reliability of CT Findings. Radiology May 2011; e-publication ahead of print.Google Scholar
  41. 41.
    Bouros D, Hatzakis K, Labrakis H, et al. Association of malignancy with diseases causing interstitial pulmonary changes. Chest. 2002;121:1278–89.PubMedCrossRefGoogle Scholar
  42. 42.
    Travis WD, Hunninghake G, King TE Jr, et al. Idiopathic nonspecific interstitial pneumonia: report of an American Thoracic Society project. Am J Respir Crit Care Med. 2008;177:1338–47.PubMedCrossRefGoogle Scholar
  43. 43.
    Cottin V, Donsbeck AV, Revel D, et al. Nonspecific interstitial pneumonia: individualization of a clinicopathologic entity in a series of 12 patients. Am J Respir Crit Care Med. 1998;158:1286–93.PubMedGoogle Scholar
  44. 44.
    Kim TS, Lee KS, Chung MP, et al. Nonspecific interstitial pneumonia with fibrosis: high-resolution CT and pathologic findings. AJR. 1998;171:1645–50.PubMedGoogle Scholar
  45. 45.
    Park CS, Jeon JW, Park SW, et al. Nonspecific interstitial pneumonia/fibrosis: clinical manifestations, histologic and radiologic features. Korean J Intern Med. 1996;11:122–32.PubMedGoogle Scholar
  46. 46.
    Park JS, Lee KS, Kim JS, et al. Nonspecific interstitial pneumonia with fibrosis: radiographic and CT findings in seven patients. Radiology. 1995;195:645–8.PubMedGoogle Scholar
  47. 47.
    Elliot TL, Lynch DA, Newell JD Jr, et al. High-resolution computed tomography features of nonspecific interstitial pneumonia and usual interstitial pneumonia. J Comput Assist Tomogr. 2005;29:339–45.PubMedCrossRefGoogle Scholar
  48. 48.
    Jeong YJ, Lee KS, Muller NL, et al. Usual interstitial pneumonia and non-specific interstitial pneumonia: serial thin-section CT findings correlated with pulmonary function. Korean J Radiol. 2005;6:143–52.PubMedCrossRefGoogle Scholar
  49. 49.
    Flaherty KR, Travis WD, Colby TV, et al. Histopathologic variability in usual and nonspecific interstitial pneumonias. Am J Respir Crit Care Med. 2001;164:1722–7.PubMedGoogle Scholar
  50. 50.
    Tsubamoto M, Muller NL, Johkoh T, et al. Pathologic subgroups of nonspecific interstitial pneumonia: differential diagnosis from other idiopathic interstitial pneumonias on high-resolution computed tomography. J Comput Assist Tomogr. 2005;29:793–800.PubMedCrossRefGoogle Scholar
  51. 51.
    Sumikawa H, Johkoh T, Ichikado K, et al. Usual interstitial pneumonia and chronic idiopathic interstitial pneumonia: analysis of CT appearance in 92 patients. Radiology. 2006;241:258–66.PubMedCrossRefGoogle Scholar
  52. 52.
    Kligeman SF, Groshong S, Brown KK, et al. Nonspecific Interstitial Pneumonia: Radiologic, Clinical, and Pathologic Considerations. RadioGraphics. 2009;29:73–87.CrossRefGoogle Scholar
  53. 53.
    MacDonald SL, Rubens MB, Hansell DM, et al. Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparative appearances at and diagnostic accuracy of thin-section CT. Radiology. 2001;221:600–5.PubMedCrossRefGoogle Scholar
  54. 54.
    Flaherty KR, Toews GB, Travis WD, et al. Clinical significance of histological classification of idiopathic interstitial pneumonia. Eur Respir J. 2002;19:275–83.PubMedCrossRefGoogle Scholar
  55. 55.
    Nicholson AG, Colby TV, du Bois RM, Hansell DM, Wells AU. The prognostic significance of the histologic pattern of interstitial pneumonia in patients presenting with the clinical entity of cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med. 2000;162:2213–7.PubMedGoogle Scholar
  56. 56.
    Flaherty KR, Thwaite EL, Kazerooni EA, et al. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax. 2003;58:143–8.PubMedCrossRefGoogle Scholar
  57. 57.
    Johkoh T, Muller NL, Colby TV, et al. Nonspecific interstitial pneumonia: correlation between thin-section CT findings and pathologic subgroups in 55 patients. Radiology. 2002;225:199–204.PubMedCrossRefGoogle Scholar
  58. 58.
    Park IN, Kim DS, Shim TS, et al. Acute exacerbation of interstitial pneumonia other than idiopathic pulmonary fibrosis. Chest. 2007;132:214–20.PubMedCrossRefGoogle Scholar
  59. 59.
    Nishiyama O, Kondoh Y, Taniguchi H, et al. Serial high resolution CT findings in nonspecific interstitial pneumonia/fibrosis. J Comput Assist Tomogr. 2000;24:41–6.PubMedCrossRefGoogle Scholar
  60. 60.
    Muller NL, Guerry-Force ML, Staples CA, et al. Differential diagnosis of bronchiolitis obliterans with organizing pneumonia and usual interstitial pneumonia: clinical, functional, and radiologic findings. Radiology. 1987;162:151–6.PubMedGoogle Scholar
  61. 61.
    Kim SJ, Lee KS, Ryu YH, et al. Reversed halo sign on high-resolution CT of cryptogenic organizing pneumonia: diagnostic implications. AJR. 2003;180:1251–4.PubMedGoogle Scholar
  62. 62.
    Walker C, Tan-Lucien M, Chung JH. Reversed halo sign. J Thoracic Imaging. 2011;26:3.CrossRefGoogle Scholar
  63. 63.
    Bouchardy LM, Kuhlman JE, Ball WC Jr, et al. CT findings in bronchiolitis obliterans organizing pneumonia (BOOP) with radiographic, clinical, and histologic correlation. J Comput Assist Tomogr. 1993;17:352–7.PubMedCrossRefGoogle Scholar
  64. 64.
    Cordier JF, Loire R, Brune J. Idiopathic bronchiolitis obliterans organizing pneumonia: definition of characteristic clinical profiles in a series of 16 patients. Chest. 1989;96:999–1004.PubMedCrossRefGoogle Scholar
  65. 65.
    Lee J, Lynch D, Sharma S, et al. Organizing pneumonia: prognostic implication of high-resolution CT features. J Comput Assist Tomogr. 2003;27:260–5.PubMedCrossRefGoogle Scholar
  66. 66.
    Lynch DA, Travis WD, King TE, et al. Idiopathic interstitial pneumonias: CT features. Radiology. 2005;236:10–21.PubMedCrossRefGoogle Scholar
  67. 67.
    Myers JL, Veal CF Jr, et al. Respiratory bronchiolitis causing interstitial lung disease. A clinicopathologic study of six cases. Am Rev Respir Dis. 1987;135:880–4.PubMedGoogle Scholar
  68. 68.
    Davies G, Wells AU, du Bois RM. Respiratory bronchiolitis associated with interstitial lung disease and desquamative interstitial pneumonia. Clin Chest Med. 2004;25:717–26. vi.PubMedCrossRefGoogle Scholar
  69. 69.
    Remy-Jardin M, Remy J, Gosselin B, et al. Lung parenchymal changes secondary to cigarette smoking: pathologic-CT correlations. Radiology. 1993;186:643–51.PubMedGoogle Scholar
  70. 70.
    Holt R, Schmidt R, Godwin J, et al. High resolution CT in respiratory bronchiolitis-associated interstitial lung disease. J Comput Assist Tomogr. 1993;17:46–50.PubMedCrossRefGoogle Scholar
  71. 71.
    Park JS, Brown KK, Tuder RM, et al. Respiratory bronchiolitis-associated interstitial lung disease: radiologic features with clinical and pathologic correlation. J Comput Assist Tomogr. 2002;26:13–20.PubMedCrossRefGoogle Scholar
  72. 72.
    Ryu JH, Colby TV, Hartman TE, et al. Smoking-related interstitial lung diseases: a concise review. Eur Respir J. 2001;17:122–32.PubMedCrossRefGoogle Scholar
  73. 73.
    Koyama M, Johkoh T, Honda O, et al. Chronic cystic lung disease: diagnostic accuracy of high-resolution CT in 92 patients. AJR. 2003;180:827–35.PubMedGoogle Scholar
  74. 74.
    Hartman TE, Primack SL, Swensen SJ, et al. Desquamative interstitial pneumonia: thin-section CT findings in 22 patients. Radiology. 1993;187:787–90.PubMedGoogle Scholar
  75. 75.
    Ichikado K, Johkoh T, Ikezoe J, et al. Acute interstitial pneumonia: high-resolution CT findings correlated with pathology. AJRl. 1997;168:333–8.PubMedGoogle Scholar
  76. 76.
    Johkoh T, Muller NL, Taniguchi H, et al. Acute interstitial pneumonia: thin-section CT findings in 36 patients. Radiology. 1999;211:859–63.PubMedGoogle Scholar
  77. 77.
    Tomiyama N, Muller NL, Johkoh T, et al. Acute respiratory distress syndrome and acute interstitial pneumonia: comparison of thin-section CT findings. J Comput Assist Tomogr. 2001;25:28–33.PubMedCrossRefGoogle Scholar
  78. 78.
    Swigris JJ, et al. Acute interstitial pneumonia and acute exacerbations of idiopathic pulmonary fibrosis. Semin Respir Crit Care Med. 2006;27(6):659–67.PubMedCrossRefGoogle Scholar
  79. 79.
    Thannickal VJ. Idiopathic interstitial pneumonia: a clinicopathological perspective. Semin Respir Crit Care Med. 2006;27(6):569–73.PubMedCrossRefGoogle Scholar
  80. 80.
    Jonkoh T. Imaging of idiopathic interstitial pneumonias. Clin Chest Med. 2008;29.1:133–47.Google Scholar
  81. 81.
    Swigris JJ, Berry GJ, Raffin TA, et al. Lymphoid interstitial pneumonia: a narrative review. Chest. 2002;122(6):2150–64.PubMedCrossRefGoogle Scholar
  82. 82.
    Das S, Miller RF. Lymphocytic interstitial pneumonitis in HIV infected adults. Sex Transm Infect. 2002;79:88–93.CrossRefGoogle Scholar

Copyright information

© Humana Press, a part of Springer Science+Business Media, LLC 2012

Authors and Affiliations

  1. 1.Department of RadiologyUniversity of Wisconsin School of Medicine and Public HealthMadisonUSA
  2. 2.Department of RadiologyUniversity of Wisconsin School of Medicine and Public HealthMadisonUSA

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