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Approach to the adult with interstitial lung disease: Clinical evaluation

Talmadge E King, Jr, MD
Section Editor
Kevin R Flaherty, MD, MS
Deputy Editor
Helen Hollingsworth, MD


The diffuse parenchymal lung diseases, often collectively referred to as the interstitial lung diseases (ILDs), are a heterogeneous group of disorders that are classified together because of similar clinical, radiographic, physiologic, or pathologic manifestations (algorithm 1) [1-6]. The descriptive term "interstitial" reflects the pathologic appearance that the abnormality begins in the interstitium, but the term is somewhat misleading, as most of these disorders are also associated with extensive alteration of alveolar and airway architecture.

An overview of the clinical findings that can aid in the diagnosis of ILD will be presented here (algorithm 2). The individual causes of ILD and the diagnostic testing that is helpful in evaluating patients with ILD are discussed separately. (See "Idiopathic interstitial pneumonias: Clinical manifestations and pathology" and "Clinical manifestations and diagnosis of pulmonary sarcoidosis" and "Pulmonary toxicity associated with systemic antineoplastic therapy: Clinical presentation, diagnosis, and treatment" and "Approach to the adult with interstitial lung disease: Diagnostic testing" and "Role of bronchoalveolar lavage in diagnosis of interstitial lung disease" and "Role of lung biopsy in the diagnosis of interstitial lung disease".)


The diffuse parenchymal lung diseases are divided into those that are associated with known causes and those that are idiopathic. The treatment choices and prognosis vary among the different causes and types of ILD, so ascertaining the correct diagnosis is important.

A variety of infections can cause interstitial opacities on chest radiograph, including fungal pneumonias (eg, coccidioidomycosis, cryptococcosis, Pneumocystis jirovecii), atypical bacterial pneumonias, and viral pneumonias. These infections often occur in immunocompromised hosts and are discussed separately. (See "Approach to the immunocompromised patient with fever and pulmonary infiltrates".)

The most common identifiable causes of ILD are exposure to occupational and environmental agents, especially to inorganic or organic dusts (table 1A-B), drug-induced pulmonary toxicity, and radiation-induced lung injury. (See "Asbestos-related pleuropulmonary disease" and "Chronic beryllium disease (berylliosis)" and "Silicosis" and "Classification and clinical manifestations of hypersensitivity pneumonitis (extrinsic allergic alveolitis)" and "Diagnosis of hypersensitivity pneumonitis (extrinsic allergic alveolitis)" and "Pulmonary toxicity associated with systemic antineoplastic therapy: Clinical presentation, diagnosis, and treatment" and "Radiation-induced lung injury".)

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Literature review current through: Nov 2017. | This topic last updated: Jul 11, 2017.
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  1. Schwartz M, King Jr TE. Interstitial Lung disease, 5th ed, People's Medical Clearing House, Shelton, CT 2011.
  2. 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; 183:788.
  3. American Thoracic Society, European Respiratory Society. 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, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med 2002; 165:277.
  4. King TE Jr, Pardo A, Selman M. Idiopathic pulmonary fibrosis. Lancet 2011; 378:1949.
  5. 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.
  6. Bradley B, Branley HM, Egan JJ, et al. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax 2008; 63 Suppl 5:v1.
  7. Pierson DM, Ionescu D, Qing G, et al. Pulmonary fibrosis in hermansky-pudlak syndrome. a case report and review. Respiration 2006; 73:382.
  8. Tian X, Yi ES, Ryu JH. Lymphocytic interstitial pneumonia and other benign lymphoid disorders. Semin Respir Crit Care Med 2012; 33:450.
  9. Donaghy M, Rees AJ. Cigarette smoking and lung haemorrhage in glomerulonephritis caused by autoantibodies to glomerular basement membrane. Lancet 1983; 2:1390.
  10. Garcia CK, Raghu G. Inherited interstitial lung disease. Clin Chest Med 2004; 25:421.
  11. Marshall RP, Puddicombe A, Cookson WO, Laurent GJ. Adult familial cryptogenic fibrosing alveolitis in the United Kingdom. Thorax 2000; 55:143.
  12. Diaz de Leon A, Cronkhite JT, Katzenstein AL, et al. Telomere lengths, pulmonary fibrosis and telomerase (TERT) mutations. PLoS One 2010; 5:e10680.
  13. Alder JK, Chen JJ, Lancaster L, et al. Short telomeres are a risk factor for idiopathic pulmonary fibrosis. Proc Natl Acad Sci U S A 2008; 105:13051.
  14. Grutters JC, du Bois RM. Genetics of fibrosing lung diseases. Eur Respir J 2005; 25:915.
  15. 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.
  16. Rouhani FN, Brantly ML, Markello TC, et al. Alveolar macrophage dysregulation in Hermansky-Pudlak syndrome type 1. Am J Respir Crit Care Med 2009; 180:1114.
  17. Camus P, Bonniaud P, Fanton A, et al. Drug-induced and iatrogenic infiltrative lung disease. Clin Chest Med 2004; 25:479.
  18. Camus P. Drug-induced interstitial lung disease. In: Interstitial Lung Disease, 4th ed, King TE Jr, Schwarz MI (Eds), B.C. Decker, Hamilton, ON, Canada 2003. p.485.
  19. Fernández AB, Karas RH, Alsheikh-Ali AA, Thompson PD. Statins and interstitial lung disease: a systematic review of the literature and of food and drug administration adverse event reports. Chest 2008; 134:824.
  20. Glazer CS, Newman LS. Occupational interstitial lung disease. Clin Chest Med 2004; 25:467.
  21. Selman M, Pardo A, King TE Jr. Hypersensitivity pneumonitis: insights in diagnosis and pathobiology. Am J Respir Crit Care Med 2012; 186:314.
  22. Sirajuddin A, Kanne JP. Occupational lung disease. J Thorac Imaging 2009; 24:310.
  23. Sauler M, Gulati M. Newly recognized occupational and environmental causes of chronic terminal airways and parenchymal lung disease. Clin Chest Med 2012; 33:667.
  24. Schwartzstein RM. Are you fluent in the 'language' of dyspnea? J Respir Dis 1996; 17:322.
  25. Bohadana A, Izbicki G, Kraman SS. Fundamentals of lung auscultation. N Engl J Med 2014; 370:744.
  26. Spicknall KE, Zirwas MJ, English JC 3rd. Clubbing: an update on diagnosis, differential diagnosis, pathophysiology, and clinical relevance. J Am Acad Dermatol 2005; 52:1020.