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Diffuse Parenchymal Lung Disease: A Clinical Overview

  • Venerino Poletti
Chapter

Abstract

Diffuse parenchymal lung disease is a generic term including a variety of disorders characterized by accumulation/infiltration of extracellular substances, fluids, or cells in the structures of the secondary pulmonary lobule. Pathophysiology is different according to the large number of disorders grouped together. However five basic mechanisms may be considered: infections, malignancy, granulomatous inflammation, nongranulomatous inflammation/fibrosis, and finally alveolar stem cell exhaustion and bronchiolar proliferation/fibrosis. The clinical features may be helpful for diagnosis, but the imaging aspects are a keystone in the diagnostic workup. Invasive procedures are needed only in a minority although still significant number of cases.

Keywords

Secondary pulmonary lobule High-resolution CT scan Idiopathic pulmonary fibrosis (IPF) Sarcoidosis Bronchoalveolar lavage Transbronchial lung biopsy Transbronchial cryobiopsy Surgical lung biopsy 

References

  1. 1.
    Colby TV, Carrington CB. Interstitial lung disease. In: Thurlbeck WM, Churg AM, editors. Pathology of the lung. New York: Thieme; 1995. p. 589–737.Google Scholar
  2. 2.
    Nagaishi C. Functional anatomy and histology of the lung. Baltimore: University Park Press; 1972. p. 26–41.Google Scholar
  3. 3.
    Martignoni G, Pea M, Righellin D, et al. Molecular pathology of lymphangioleiomyomatosis and other perivascular epithelioid cell tumors. Arch Pathol Lab Med. 2010;134:33–40.PubMedGoogle Scholar
  4. 4.
    Durham BH. Molecular characterization of the histiocytosis: neoplasia of dendritic and macrophages. Semin Cell Dev Biol. 2019;86:62–76.CrossRefGoogle Scholar
  5. 5.
    Milne P, Bigley V, Bacon CM, et al. Hematopoietic origin of Langerhans cell histiocytosis and Erdheim Chester disease in adults. Blood. 2017;130:167–75.CrossRefGoogle Scholar
  6. 6.
    Korsten P, Tampa B, Konig MF, Nikiphorou E. Sarcoidosis and autoimmune diseases: differences, similarities and overlaps. Curr Opin Pulm Med. 2018;24:504–12.CrossRefGoogle Scholar
  7. 7.
    Sood AK, Funkhouser W, Handly B, Weston B, Wu EY. Granulomatous-lymphocytic interstitial lung disease in 22q11.2 deletion syndrome: case report and literature review. Curr Allergy Asthma Rep. 2018;18:14.CrossRefGoogle Scholar
  8. 8.
    Atzeni F, Gerardi MC, Barillaro G, Masala IF, Benucci M, Sarzi-Puttini P. Interstitial lung disease in systemic autoimmune rheumatic diseases: a comprehensive review. Expert Rev Clin Immunol. 2018;14:69–82.CrossRefGoogle Scholar
  9. 9.
    Chilosi M, Caliò A, Rossi A, et al. Epithelial to mesenchymal transition-related proteins ZEB1, beta-catenin, and beta-tubulin III in idiopathic pulmonary fibrosis. Mod Pathol. 2017;30:26–36.CrossRefGoogle Scholar
  10. 10.
    Chilosi M, Carloni A, Rossi A, Poletti V. Premature lung aging and cellular senescence in the pathogenesis of idiopathic pulmonary fibrosis and COPD/emphysema. Transl Res. 2013;162:156–73.CrossRefGoogle Scholar
  11. 11.
    Kaur A, Mathai SK, Schwartz DA. Genetics in idiopathic pulmonary fibrosis. Pathogenesis, prognosis and treatment. Front Med. 2017;4:154.CrossRefGoogle Scholar
  12. 12.
    Hoffman TW, van Moorsel CHM, Borie R, Crestani B. Pulmonary phenotypes associated with genetic variation in telomere-related genes. Curr Opin Pulm Med. 2018;24:269–80.PubMedGoogle Scholar
  13. 13.
    Allen RJ, Porte J, Braybrooke R, et al. Genetic variants associated with susceptibility to idiopathic pulmonary fibrosis in people of European ancestry: a genome-wide association study. Lancet Respir Med. 2017;5:869–80.CrossRefGoogle Scholar
  14. 14.
    Dal Piaz G. Computed tomography of diffuse lung diseases and solitary pulmonary nodules. In: Leslie KO, Wick MR, editors. Practical pulmonary pathology: a diagnostic approach. 3rd ed. Amsterdam: Elsevier; 2018. p. 35–98.CrossRefGoogle Scholar
  15. 15.
    Cagle PT, Kerr KM. Pulmonary pathology. Berlin: Springer; 2018.CrossRefGoogle Scholar
  16. 16.
    Costabel U, du Bois RM, Egan JJ, Herth F, Bolliger CT. Diffuse parenchymal lung disease. Basel: Karger; 2007.CrossRefGoogle Scholar
  17. 17.
    Hetzel J, Maldonado F, Ravaglia C, et al. Transbronchial cryobiopsies for the diagnosis of diffuse parenchymal lung diseases. Expert statement from the cryobiopsy working group on safety and utility and a call for standardization of the procedure. Respiration. 2018;95:188–200.CrossRefGoogle Scholar
  18. 18.
    Poletti V, Casoni GL, Gurioli C, Ryu JH, Tomassetti S. Lung cryobiopsies: a paradigm shift in diagnostic bronchoscopy? Respirology. 2014;19:645–54.CrossRefGoogle Scholar
  19. 19.
    Ravaglia C, Wells AU, Tomassetti S, Gurioli C, Gurioli C, Dubini A, Cavazza A, Colby TV, Piciucchi S, Puglisi S, Bosi M, Poletti V. Diagnostic yield and risk/benefit analysis of trans-bronchial lung cryobiopsy in diffuse parenchymal lung diseases: a large cohort of 699 patients. BMC Pulm Med. 2019;19(1):16.Google Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Venerino Poletti
    • 1
    • 2
  1. 1.Department of Diseases of the ThoraxOspedale Morgagni-PierantoniForlìItaly
  2. 2.Department of Respiratory Diseases and AllergyAarhus University HospitalAarhusDenmark

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