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Nonspecific Interstitial Pneumonia
Tyler H. Ternes, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Nonspecific interstitial pneumonia (NSIP)
            • Definitions

              • Type of idiopathic interstitial pneumonia (IIP)
              • Chronic fibrosing IIPs
                • Idiopathic pulmonary fibrosis (IPF)
                • Idiopathic NSIP
              • Rare IIPs
                • Lymphoid interstitial pneumonia
                • Idiopathic pleuroparenchymal fibroelastosis
              • Smoking-related IIPs
                • Desquamative interstitial pneumonia
                • Respiratory bronchiolitis-interstitial lung disease
              • Acute or subacute IIPs
                • Cryptogenic organizing pneumonia (OP)
                • Acute interstitial pneumonia
              • NSIP may be idiopathic or may be associated with connective tissue/autoimmune disease, drug toxicity, hypersensitivity pneumonitis, and other etiologies

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        • Image Interpretation Pearls

                                          • Reporting Tips

                                            Selected References

                                            1. Johkoh T et al: Chest CT diagnosis and clinical management of drug-related pneumonitis in patients receiving molecular targeting agents and immune checkpoint inhibitors: a position paper from the Fleischner Society. Radiology. 298(3):550-66, 2021
                                            2. Hatabu H et al: Expanding applications of pulmonary mri in the clinical evaluation of lung disorders: Fleischner Society position paper. Radiology. 297(2):286-301, 2020
                                            3. Teoh AKY et al: Nonspecific interstitial pneumonia. Semin Respir Crit Care Med. 41(2):184-201, 2020
                                            4. Graney BA et al: Interstitial pneumonia with autoimmune features. Ann Am Thorac Soc. 16(5):525-33, 2019
                                            5. Zhan X et al: Differentiating usual interstitial pneumonia from nonspecific interstitial pneumonia using high-resolution computed tomography: the "straight-edge sign". J Thorac Imaging. 33(4):266-70, 2018
                                            6. Chung JH et al: CT findings, radiologic-pathologic correlation, and imaging predictors of survival for patients with interstitial pneumonia with autoimmune features. AJR Am J Roentgenol. 208(6):1229-36, 2017
                                            7. Huo Z et al: Organizing pneumonia components in non-specific interstitial pneumonia (NSIP): a clinicopathological study of 33 NSIP cases. Histopathology. 68(3):347-55, 2016
                                            8. Buzan MT et al: T2 mapping of CT remodelling patterns in interstitial lung disease. Eur Radiol. 25(11):3167-74, 2015
                                            9. Fischer A et al: An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J. 46(4):976-87, 2015
                                            10. Sverzellati N et al: American Thoracic Society-European Respiratory Society classification of the idiopathic interstitial pneumonias: advances in knowledge since 2002. Radiographics. 35(7):1849-71, 2015
                                            11. Todd NW et al: Organizing pneumonia/non-specific interstitial pneumonia overlap is associated with unfavorable lung disease progression. Respir Med. 109(11):1460-8, 2015
                                            12. Travis WD et al: An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 188(6):733-48, 2013
                                            13. Lee HY et al: High-resolution CT findings in fibrotic idiopathic interstitial pneumonias with little honeycombing: serial changes and prognostic implications. AJR Am J Roentgenol. 199(5):982-9, 2012
                                            14. Raghu G 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. 183(6):788-824, 2011
                                            15. Lee JW et al: Cryptogenic organizing pneumonia: serial high-resolution CT findings in 22 patients. AJR Am J Roentgenol. 195(4):916-22, 2010
                                            16. Kligerman SJ, et al: Nonspecific interstitial pneumonia: radiologic, clinical, and pathologic considerations. Radiographics: 29(1):73-87, 2009
                                            17. Silva CI et al: Nonspecific interstitial pneumonia and idiopathic pulmonary fibrosis: changes in pattern and distribution of disease over time. Radiology. 247(1):251-9, 2008
                                            18. Silva CI et al: Chronic hypersensitivity pneumonitis: differentiation from idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia by using thin-section CT. Radiology. 246(1):288-97, 2008
                                            19. Yi CA et al: 3-T MRI for differentiating inflammation- and fibrosis-predominant lesions of usual and nonspecific interstitial pneumonia: comparison study with pathologic correlation. AJR Am J Roentgenol. 190(4):878-85, 2008
                                            20. Kim EY, Lee KS, Chung MP, Kwon OJ, Kim TS, Hwang JH. Nonspecific interstitial pneumonia with fibrosis: serial high-resolution CT findings with functional correlation. AJR 1999;173:949–53.
                                            21. Park JS et al: Nonspecific interstitial pneumonia with fibrosis: radiographic and CT findings in seven patients. Radiology. 195(3):645-8, 1995
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Pathology

                                                    • Clinical Issues

                                                      TERMINOLOGY

                                                      • Abbreviations

                                                        • Nonspecific interstitial pneumonia (NSIP)
                                                      • Definitions

                                                        • Type of idiopathic interstitial pneumonia (IIP)
                                                        • Chronic fibrosing IIPs
                                                          • Idiopathic pulmonary fibrosis (IPF)
                                                          • Idiopathic NSIP
                                                        • Rare IIPs
                                                          • Lymphoid interstitial pneumonia
                                                          • Idiopathic pleuroparenchymal fibroelastosis
                                                        • Smoking-related IIPs
                                                          • Desquamative interstitial pneumonia
                                                          • Respiratory bronchiolitis-interstitial lung disease
                                                        • Acute or subacute IIPs
                                                          • Cryptogenic organizing pneumonia (OP)
                                                          • Acute interstitial pneumonia
                                                        • NSIP may be idiopathic or may be associated with connective tissue/autoimmune disease, drug toxicity, hypersensitivity pneumonitis, and other etiologies

                                                      IMAGING

                                                      • General Features

                                                        • Radiographic Findings

                                                          • CT Findings

                                                            • MR Findings

                                                              • Imaging Recommendations

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Staging, Grading, & Classification

                                                                      • Microscopic Features

                                                                        CLINICAL ISSUES

                                                                        • Presentation

                                                                          • Demographics

                                                                            • Natural History & Prognosis

                                                                              • Treatment

                                                                                DIAGNOSTIC CHECKLIST

                                                                                • Consider

                                                                                  • Image Interpretation Pearls

                                                                                    • Reporting Tips

                                                                                      Selected References

                                                                                      1. Johkoh T et al: Chest CT diagnosis and clinical management of drug-related pneumonitis in patients receiving molecular targeting agents and immune checkpoint inhibitors: a position paper from the Fleischner Society. Radiology. 298(3):550-66, 2021
                                                                                      2. Hatabu H et al: Expanding applications of pulmonary mri in the clinical evaluation of lung disorders: Fleischner Society position paper. Radiology. 297(2):286-301, 2020
                                                                                      3. Teoh AKY et al: Nonspecific interstitial pneumonia. Semin Respir Crit Care Med. 41(2):184-201, 2020
                                                                                      4. Graney BA et al: Interstitial pneumonia with autoimmune features. Ann Am Thorac Soc. 16(5):525-33, 2019
                                                                                      5. Zhan X et al: Differentiating usual interstitial pneumonia from nonspecific interstitial pneumonia using high-resolution computed tomography: the "straight-edge sign". J Thorac Imaging. 33(4):266-70, 2018
                                                                                      6. Chung JH et al: CT findings, radiologic-pathologic correlation, and imaging predictors of survival for patients with interstitial pneumonia with autoimmune features. AJR Am J Roentgenol. 208(6):1229-36, 2017
                                                                                      7. Huo Z et al: Organizing pneumonia components in non-specific interstitial pneumonia (NSIP): a clinicopathological study of 33 NSIP cases. Histopathology. 68(3):347-55, 2016
                                                                                      8. Buzan MT et al: T2 mapping of CT remodelling patterns in interstitial lung disease. Eur Radiol. 25(11):3167-74, 2015
                                                                                      9. Fischer A et al: An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J. 46(4):976-87, 2015
                                                                                      10. Sverzellati N et al: American Thoracic Society-European Respiratory Society classification of the idiopathic interstitial pneumonias: advances in knowledge since 2002. Radiographics. 35(7):1849-71, 2015
                                                                                      11. Todd NW et al: Organizing pneumonia/non-specific interstitial pneumonia overlap is associated with unfavorable lung disease progression. Respir Med. 109(11):1460-8, 2015
                                                                                      12. Travis WD et al: An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 188(6):733-48, 2013
                                                                                      13. Lee HY et al: High-resolution CT findings in fibrotic idiopathic interstitial pneumonias with little honeycombing: serial changes and prognostic implications. AJR Am J Roentgenol. 199(5):982-9, 2012
                                                                                      14. Raghu G 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. 183(6):788-824, 2011
                                                                                      15. Lee JW et al: Cryptogenic organizing pneumonia: serial high-resolution CT findings in 22 patients. AJR Am J Roentgenol. 195(4):916-22, 2010
                                                                                      16. Kligerman SJ, et al: Nonspecific interstitial pneumonia: radiologic, clinical, and pathologic considerations. Radiographics: 29(1):73-87, 2009
                                                                                      17. Silva CI et al: Nonspecific interstitial pneumonia and idiopathic pulmonary fibrosis: changes in pattern and distribution of disease over time. Radiology. 247(1):251-9, 2008
                                                                                      18. Silva CI et al: Chronic hypersensitivity pneumonitis: differentiation from idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia by using thin-section CT. Radiology. 246(1):288-97, 2008
                                                                                      19. Yi CA et al: 3-T MRI for differentiating inflammation- and fibrosis-predominant lesions of usual and nonspecific interstitial pneumonia: comparison study with pathologic correlation. AJR Am J Roentgenol. 190(4):878-85, 2008
                                                                                      20. Kim EY, Lee KS, Chung MP, Kwon OJ, Kim TS, Hwang JH. Nonspecific interstitial pneumonia with fibrosis: serial high-resolution CT findings with functional correlation. AJR 1999;173:949–53.
                                                                                      21. Park JS et al: Nonspecific interstitial pneumonia with fibrosis: radiographic and CT findings in seven patients. Radiology. 195(3):645-8, 1995