Interpretation of lung biopsy results in interstitial lung disease
- Talmadge E King, Jr, MD
Talmadge E King, Jr, MD
- Editor-in-Chief — Pulmonary and Critical Care Medicine
- Section Editor — Interstitial Lung Disease
- Dean, School of Medicine
- Vice Chancellor, Medical Affairs
- University of California San Francisco
- Section Editors
- Kevin R Flaherty, MD, MS
Kevin R Flaherty, MD, MS
- Section Editor — Interstitial Lung Disease
- Associate Professor of Medicine
- University of Michigan Health System
- Praveen N Mathur, MB, BS
Praveen N Mathur, MB, BS
- Section Editor — Interventional Pulmonology
- Professor of Clinical Medicine
- Indiana University School of Medicine
- Andrew Nicholson, MD
Andrew Nicholson, MD
- Section Editor — Pulmonary Pathology
- Professor of Respiratory Pathology
- Imperial College School of Medicine, London
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). When the results of clinical evaluation, laboratory testing, imaging studies including high resolution computed tomography (HRCT), and pulmonary function testing do not allow the clinician to make a confident diagnosis of a given type or stage of ILD, lung biopsy with careful examination of lung tissue is often necessary (algorithm 2).
The clinicopathologic interpretation of lung biopsy results in adults with ILD will be reviewed here. The clinical evaluation, diagnostic testing, radiographic patterns, role of bronchoalveolar lavage, and role of lung biopsy in ILD are discussed separately. (See "Approach to the adult with interstitial lung disease: Clinical evaluation" and "Approach to the adult with interstitial lung disease: Diagnostic testing" and "High resolution computed tomography of the lungs" and "Basic principles and technique of bronchoalveolar lavage" and "Role of bronchoalveolar lavage in diagnosis of interstitial lung disease" and "Role of lung biopsy in the diagnosis of interstitial lung disease".)
CLINICIAN - PATHOLOGIST INTERACTION
Histologic abnormalities alone are rarely specific in ILD. More often, the findings are "characteristic" or "consistent with" a specific diagnosis. "Nonspecific" reaction patterns are common to several diseases that cause inflammation and/or fibrosis in the lungs . Small tissue samples and substantial inter-observer variability among pathologists further complicate interpretation [2-4]. As a result, most cases require careful clinical and pathological correlation to arrive at the most accurate final diagnosis [5-8].
With a multidisciplinary approach, surgical lung biopsy produces a definitive diagnosis in more than 90 percent of cases of ILD occurring in immunocompetent hosts [5,9].
Data provided to the pathologist by the clinician at the time of biopsy should include:
- Colby TV, Churg AC. Patterns of pulmonary fibrosis. In: 1986 Pathology Annual, Sommers SC, Rosen PP, Fechner RE (Eds), Appleton-Century-Crofts, Norwalk, CT 1986. p.277.
- Nicholson AG, Addis BJ, Bharucha H, et al. Inter-observer variation between pathologists in diffuse parenchymal lung disease. Thorax 2004; 59:500.
- 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.
- Nicholson AG, Colby TV, du Bois RM, et al. 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.
- Flaherty KR, King TE Jr, Raghu G, et al. Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis? Am J Respir Crit Care Med 2004; 170:904.
- Hunninghake GW, Zimmerman MB, Schwartz DA, et al. Utility of a lung biopsy for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2001; 164:193.
- Leslie KO, Colby TV, Lynch DA. Anatomic distribution and histopathologic patterns of interstitial lung disease. In: Interstitial Lung Disease, 5th, Schwartz MI, King TE, Jr. (Eds), People's Medical Publishing House, Shelton, CT 2011. p.35.
- Walsh SL, Wells AU, Desai SR, et al. Multicentre evaluation of multidisciplinary team meeting agreement on diagnosis in diffuse parenchymal lung disease: a case-cohort study. Lancet Respir Med 2016; 4:557.
- Wagner JD, Stahler C, Knox S, et al. Clinical utility of open lung biopsy for undiagnosed pulmonary infiltrates. Am J Surg 1992; 164:104.
- Wells AU, Hansell DM, Nicholson AG. What is this thing called CFA? Thorax 2007; 62:3.
- Churg A, Muller NL, Flint J, Wright JL. Chronic hypersensitivity pneumonitis. Am J Surg Pathol 2006; 30:201.
- Katzenstein AL, Fiorelli RF. Nonspecific interstitial pneumonia/fibrosis. Histologic features and clinical significance. Am J Surg Pathol 1994; 18:136.
- Craig PJ, Wells AU, Doffman S, et al. Desquamative interstitial pneumonia, respiratory bronchiolitis and their relationship to smoking. Histopathology 2004; 45:275.
- Yousem SA, Colby TV, Gaensler EA. Respiratory bronchiolitis-associated interstitial lung disease and its relationship to desquamative interstitial pneumonia. Mayo Clin Proc 1989; 64:1373.
- Niewoehner DE, Kleinerman J, Rice DB. Pathologic changes in the peripheral airways of young cigarette smokers. N Engl J Med 1974; 291:755.
- Myers JL, Veal CF Jr, Shin MS, Katzenstein AL. Respiratory bronchiolitis causing interstitial lung disease. A clinicopathologic study of six cases. Am Rev Respir Dis 1987; 135:880.
- Colby TV, Lombard C, Yousem SA, et al. Atlas of Pulmonary Surgical Pathology, WB Saunders, Philadelphia 1991. p.380.
- Nicholson AG. Lymphocytic interstitial pneumonia and other lymphoproliferative disorders in the lung. Semin Respir Crit Care Med 2001; 22:409.
- Katzenstein AL, Myers JL, Mazur MT. Acute interstitial pneumonia. A clinicopathologic, ultrastructural, and cell kinetic study. Am J Surg Pathol 1986; 10:256.
- Olson J, Colby TV, Elliott CG. Hamman-Rich syndrome revisited. Mayo Clin Proc 1990; 65:1538.
- Fulmer JD, Katzenstein AL. The interstitial lung diseases. In: Pulmonary and Critical Care Medicine, Bone RC (Ed), Mosby Year Book, St. Louis 1993. p.M1.
- Fasano MB, Sullivan KE, Sarpong SB, et al. Sarcoidosis and common variable immunodeficiency. Report of 8 cases and review of the literature. Medicine (Baltimore) 1996; 75:251.
- Popa V, Colby TV, Reich SB. Pulmonary interstitial disease in Ig deficiency. Chest 2002; 122:1594.
- Villanueva AG, Mark EJ. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 24-1996. A 54-year-old woman with infiltrative lung disease and mild dyspnea. N Engl J Med 1996; 335:417.
- Kusagaya H, Fujisawa T, Enomoto N, et al. Co-occurrence of Pneumoperitoneum and Pneumothorax in a Patient with Pleuroparenchymal Fibroelastosis. Am J Respir Crit Care Med 2015; 191:1200.
- Watanabe K, Nagata N, Kitasato Y, et al. Rapid decrease in forced vital capacity in patients with idiopathic pulmonary upper lobe fibrosis. Respir Investig 2012; 50:88.
- Reddy TL, Tominaga M, Hansell DM, et al. Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J 2012; 40:377.
- Beynat-Mouterde C, Beltramo G, Lezmi G, et al. Pleuroparenchymal fibroelastosis as a late complication of chemotherapy agents. Eur Respir J 2014; 44:523.
- Beasley MB, Franks TJ, Galvin JR, et al. Acute fibrinous and organizing pneumonia: a histological pattern of lung injury and possible variant of diffuse alveolar damage. Arch Pathol Lab Med 2002; 126:1064.
- Hariri LP, Unizony S, Stone J, et al. Acute fibrinous and organizing pneumonia in systemic lupus erythematosus: a case report and review of the literature. Pathol Int 2010; 60:755.
- Damas C, Morais A, Moura CS, Marques A. Acute fibrinous and organizing pneumonia. Rev Port Pneumol 2006; 12:615.
- Bhatti S, Hakeem A, Torrealba J, et al. Severe acute fibrinous and organizing pneumonia (AFOP) causing ventilatory failure: successful treatment with mycophenolate mofetil and corticosteroids. Respir Med 2009; 103:1764.
- Balduin R, Giacometti C, Saccarola L, et al. Acute fibrinous and organizing pneumonia in a patient with collagen vascular disease "stigma". Sarcoidosis Vasc Diffuse Lung Dis 2007; 24:78.
- Yokogawa N, Alcid DV. Acute fibrinous and organizing pneumonia as a rare presentation of abacavir hypersensitivity reaction. AIDS 2007; 21:2116.
- Piciucchi S, Dubini A, Tomassetti S, et al. A case of amiodarone-induced acute fibrinous and organizing pneumonia mimicking mesothelioma. Am J Respir Crit Care Med 2015; 191:104.
- Churg A, Myers J, Suarez T, et al. Airway-centered interstitial fibrosis: a distinct form of aggressive diffuse lung disease. Am J Surg Pathol 2004; 28:62.
- Fukuoka J, Franks TJ, Colby TV, et al. Peribronchiolar metaplasia: a common histologic lesion in diffuse lung disease and a rare cause of interstitial lung disease: clinicopathologic features of 15 cases. Am J Surg Pathol 2005; 29:948.
- 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.
- Daniil ZD, Gilchrist FC, Nicholson AG, 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. Am J Respir Crit Care Med 1999; 160:899.
- CLINICIAN - PATHOLOGIST INTERACTION
- INTERPRETATION OF HISTOPATHOLOGIC PATTERNS
- Interstitial pneumonias
- - Usual interstitial pneumonia
- - Nonspecific interstitial pneumonia
- - Desquamative interstitial pneumonia
- - Respiratory bronchiolitis
- - Organizing pneumonia
- - Lymphoid interstitial pneumonia
- - Diffuse alveolar damage
- Granulomatous lung diseases
- - Sarcoidosis
- - Chronic beryllium disease
- - Hypersensitivity pneumonitis
- - Langerhans cell granulomatosis
- - Erdheim-Chester disease
- - Foreign body granulomatosis
- - Diseases with associated vascular inflammation
- - Bronchocentric granulomatosis
- Other diffuse parenchymal lung diseases
- - Eosinophilic pneumonia
- - Pulmonary hemorrhage syndromes
- - Pulmonary alveolar proteinosis
- - Pulmonary amyloidosis
- - Smooth muscle proliferation
- - Honeycomb lung
- Rare histopathologic interstitial pneumonia patterns
- Unclassifiable interstitial pneumonia
- SUMMARY AND RECOMMENDATIONS