Computed Tomographic Features of Idiopathic Fibrosing Interstitial Pneumonia: Comparison With Pulmonary Fibrosis Related to Collagen Vascular Disease

Objective: To compare the computed tomographic (CT) features of idiopathic fibrosing interstitial pneumonia with those of pulmonary fibrosis related to collagen vascular disease (CVD). Methods: We reviewed the CT scans of 177 patients with diffuse interstitial pulmonary fibrosis, of which 97 had idiopathic fibrosing interstitial pneumonia and 80 had CVD. The CT images were systematically scored for the presence and extent of pulmonary and extrapulmonary abnormalities. Computed tomographic diagnosis of usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia (NSIP) was assigned. Results: A CT pattern of UIP was identified in 59 (60.8%) of patients with idiopathic fibrosing interstitial pneumonia compared with 15 (18.7%) of those patients with CVD; conversely, the CT diagnosis of NSIP was made in 51 (64%) of patients with CVD compared with 36 (37%) of patients with idiopathic disease (P < 0.01). In 113 patients who had lung biopsy, the CT diagnoses of UIP and NSIP were concordant with the histologic diagnoses in 36 of 50 patients and 34 of 41 patients, respectively. Pleural effusions, esophageal dilation, and pericardial abnormalities were more frequent in patients with CVD than in patients with idiopathic fibrosing interstitial pneumonia. Conclusions: Compared with patients with CVD, those patients with an idiopathic fibrosing interstitial pneumonia showed a higher prevalence of a UIP pattern and lower prevalence of an NSIP pattern as determined by CT. Identification of coexisting extrapulmonary abnormalities on CT can support a diagnosis of CVD.

[1]  N. Müller,et al.  Fleischner Society: glossary of terms for thoracic imaging. , 2008, Radiology.

[2]  D. Lynch,et al.  Pericardial abnormalities predict the presence of echocardiographically defined pulmonary arterial hypertension in systemic sclerosis-related interstitial lung disease. , 2007, Chest.

[3]  David A Lynch,et al.  Idiopathic interstitial pneumonias: CT features. , 2005, Radiology.

[4]  D. Lynch,et al.  High-Resolution Computed Tomography Features of Nonspecific Interstitial Pneumonia and Usual Interstitial Pneumonia , 2005, Journal of computer assisted tomography.

[5]  M. Tarkka,et al.  Open lung biopsy of patients with rheumatoid arthritis , 1990, Clinical Rheumatology.

[6]  A. Nicholson,et al.  CT features of lung disease in patients with systemic sclerosis: comparison with idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. , 2004, Radiology.

[7]  D. Lynch,et al.  Rheumatoid arthritis-related lung diseases: CT findings. , 2004, Radiology.

[8]  A. Nicholson,et al.  Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. , 2002, American journal of respiratory and critical care medicine.

[9]  T. Colby,et al.  The major histopathologic pattern of pulmonary fibrosis in scleroderma is nonspecific interstitial pneumonia. , 2002, Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG.

[10]  Paul J. Friedman,et al.  American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors , 2002, American journal of respiratory and critical care medicine.

[11]  D. Schroeder,et al.  Polymyositis-dermatomyositis-associated interstitial lung disease. , 2001, American journal of respiratory and critical care medicine.

[12]  D. Lynch,et al.  Utility of a lung biopsy for the diagnosis of idiopathic pulmonary fibrosis. , 2001, American journal of respiratory and critical care medicine.

[13]  R. D. du Bois,et al.  Cryptogenic fibrosing alveolitis/idiopathic pulmonary fibrosis. , 2001, The European respiratory journal. Supplement.

[14]  R. Ueda,et al.  Non-specific interstitial pneumonia as pulmonary involvement of systemic sclerosis , 2001, Annals of the rheumatic diseases.

[15]  A. Nicholson,et al.  Nonspecific interstitial pneumonia: variable appearance at high-resolution chest CT. , 2000, Radiology.

[16]  A. Nicholson,et al.  The prognostic significance of the histologic pattern of interstitial pneumonia in patients presenting with the clinical entity of cryptogenic fibrosing alveolitis. , 2000, American journal of respiratory and critical care medicine.

[17]  W. Travis,et al.  Idiopathic nonspecific interstitial pneumonia: prognostic significance of cellular and fibrosing patterns: survival comparison with usual interstitial pneumonia and desquamative interstitial pneumonia. , 2000, The American journal of surgical pathology.

[18]  A. Nicholson,et al.  A histologic pattern of nonspecific interstitial pneumonia is associated with a better prognosis than usual interstitial pneumonia in patients with cryptogenic fibrosing alveolitis. , 1999, American journal of respiratory and critical care medicine.

[19]  M Ando,et al.  Idiopathic interstitial pneumonias: diagnostic accuracy of thin-section CT in 129 patients. , 1999, Radiology.

[20]  T. Colby.  Pulmonary pathology in patients with systemic autoimmune diseases. , 1998, Clinics in chest medicine.

[21]  K. S. Lee,et al.  Nonspecific interstitial pneumonia with fibrosis: high-resolution CT and pathologic findings. , 1998, AJR. American journal of roentgenology.

[22]  S. Swensen,et al.  Diffuse lung disease: diagnostic accuracy of CT in patients undergoing surgical biopsy of the lung. , 1997, Radiology.

[23]  D. Hansell,et al.  Cryptogenic fibrosing alveolitis and the fibrosing alveolitis of systemic sclerosis: morphological differences on computed tomographic scans. , 1997, Thorax.

[24]  M. Maniati,et al.  Idiopathic pulmonary fibrosis and pulmonary fibrosis in diffuse systemic sclerosis: two fibroses with different prognoses. , 1997, Respiration; international review of thoracic diseases.

[25]  D. Hansell,et al.  Fibrosing alveolitis associated with systemic sclerosis has a better prognosis than lone cryptogenic fibrosing alveolitis. , 1994, American journal of respiratory and critical care medicine.

[26]  J E Aldrich,et al.  Chronic infiltrative lung disease: comparison of diagnostic accuracies of radiography and low- and conventional-dose thin-section CT. , 1994, Radiology.

[27]  A. Katzenstein,et al.  Nonspecific Interstitial Pneumonia/Fibrosis: Histologic Features and Clinical Significance , 1994, The American journal of surgical pathology.

[28]  C. Selby,et al.  Accuracy of the typical computed tomographic appearances of fibrosing alveolitis. , 1993, Thorax.

[29]  J. Roca,et al.  Interstitial pulmonary fibrosis with and without associated collagen vascular disease: results of a two year follow up. , 1992, Thorax.

[30]  W. Thurlbeck,et al.  Quantitative assessment of lung pathology in idiopathic pulmonary fibrosis. The BAL Cooperative Group Steering Committee. , 1991, The American review of respiratory disease.

[31]  J M Simpson,et al.  Simple method of estimating severity of pulmonary fibrosis on a numerical scale. , 1988, Journal of clinical pathology.

[32]  T. Medsger,et al.  Role of inflammation in the lung disease of systemic sclerosis: comparison with idiopathic pulmonary fibrosis. , 1986, The Journal of laboratory and clinical medicine.

[33]  C. Carrington,et al.  Lung biopsy in rheumatoid arthritis. , 1985, The American review of respiratory disease.

[34]  F L Bookstein,et al.  Normal mediastinal lymph nodes: number and size according to American Thoracic Society mapping. , 1985, AJR. American journal of roentgenology.

[35]  G Gamsu,et al.  CT-determined pulmonary artery diameters in predicting pulmonary hypertension. , 1984, Investigative radiology.

[36]  R. Rudd,et al.  Cryptogenic fibrosing alveolitis. Relationships of pulmonary physiology and bronchoalveolar lavage to response to treatment and prognosis. , 1981, The American review of respiratory disease.

[37]  R. Matthay,et al.  INTERSTITIAL LUNG DISEASE IN POLYMYOSITIS AND DERMATOMYOSITIS: ANALYSIS OF SIX CASES AND REVIEW OF THE LITERATURE , 1976, Medicine.