Interstitial lung disease: A clinical overview and general approach

Interstitial lung disease: A clinical overview and general approach
By Prof. Ramadan Nafae Professor and Head of Chest Department Zagazig , Faulty of Medicine

Items: Definition Epidemiology Classification Pathogenesis Diagnosis
Treatment Final comments

HAMMAN and RICH were the first to describe (in and 1944) four patients who died of rapidly progressive lung disease characterized by diffuse interstitial pneumonia and fibrosis. Interstitium Refers to the microscopic anatomic space bounded by the basement membrane of epithelial and endothelial cells. Within this interstitial space, fibroblast like cells (mesenchymal and connective tissue cells) and extracellular matrix components (interstitial collagens, elastin, proteoglycans) are present

It is clear that the disease is not restricted to the interstitium as it involves epithelial, endothelial and mesenchymal cells, macrophages and recruited inflammatory cells, secreted proteins, and aberration of matrix component within the alveolar space. In addition, the disease process extends into the alveolar space, acini, bronchiolar lumen and bronchioles.

ILD is a heterogeneous syndrome with the following common clinical features:
Exertional dyspnea Bilateral diffuse infiltrates on chest radiographs Physiological abnormalities with a restrictive lung defect, decreased diffusing capacity (DLco) and abnormal alveolar- arterial oxygen gradient (PAO2 – PaO2) at rest or with exertion. Absence of pulmonary infection and neoplasm. Histopathology with varing degrees of fibrosis and inflammation with or without evidence of granulomatous or secondary vascular changes in the pulmonary parenchyma.

Epidemiology It is more frequent than previously recognized.
Incidence ranges from 3 to 26 per per year. The prevalence of preclinical and undiagnosed ILD in the community is 10 times that of clinically recognized. Among these, IPF is the most common, representing at least 30% of the incident cases.

Diffuse Parenchymal Lung Disease (DPLD)
DPLD of known cause, eg, drugs or association, eg, collagen vascular disease Idiopathic interstitial pneumonias Granulomatous DPLD, eg, sarcoidosis Other forms of DPLD, eg, LAM, HX, etc Idiopathic pulmonary fibrosis IIP other than idiopathic pulmonary fibrosis Desquamative interstitial pneumonia Respiratory bronchiolitis interstitial lung disease Differential Diagnosis of IPF Diffuse parenchymal lung diseases (DPLDs) include disorders of known cause (eg, drug-related, collagen vascular disease) and those of unknown etiology (ie, IIPs), granulomatous DPLD (eg, sarcoidosis), and other forms of DPLD (eg, angioleiomyomatoisis (LAM), pulmonary Langerhans’ cell histiocytosis/histiocytosis X [HX]), and eosinophilic pneumonia. The idiopathic interstitial pneumonias can be distinguished from other forms of diffuse parenchymal lung disease by clinical methods including history, physical examination, chest radiology, laboratory studies, and pathology. Within the idiopathic interstitial pneumonias, it is important to distinguish between idiopathic pulmonary fibrosis and the other interstitial pneumonias, which include desquamative interstitial pneumonia (DIP), respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), acute interstitial pneumonia (AIP), cryptogenic organizing pneumonia (COP), nonspecific interstitial pneumonia (NSIP; a provisional term), and lymphocytic interstitial pneumonia (LIP). American Thoracic Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med. 2002;165: Acute interstitial pneumonia Cryptogenic organizing pneumonia Nonspecific interstitial pneumonia (provisional) Lymphocytic interstitial pneumonia ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2002;165:

ATS/ERS Classification of Idiopathic Interstitial Pneumonias
Histologic Pattern Clinical/Radiologic/Pathologic Diagnosis Usual interstitial pneumonia Idiopathic pulmonary fibrosis/cryptogenic fibrosing alveolitis Nonspecific interstitial pneumonia Organizing pneumonia Cryptogenic organizing pneumonia Diffuse alveolar damage Acute interstitial pneumonia Respiratory bronchiolitis Respiratory bronchiolitis interstitial lung disease Desquamative interstitial pneumonia Lymphoid interstitial pneumonia ATS/ERS Classification of Idiopathic Interstitial Pneumonias This table summarizes the recent ATS/ERS classification of the idiopathic interstitial pneumonias, with the requisite histopathologic correlate listed for each. It is important to note that, while a histopathologic pattern of UIP is required for a diagnosis of IPF, other diseases are associated with a UIP pattern. Therefore, the presence of UIP on biopsy does not definitively lead to a clinical diagnosis of IPF. American Thoracic Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med. 2002;165: Nicholson, AG, Addis BJ, Bharucha H, et al. Interobserver variation between pathologists in diffuse parenchymal lung disease. Thorax. 2004;59:

IIP Classification Radiology Distribution Pathology Diagnosis IPF/UIP
Fibrosis, HC Basilar, peripheral Temporal heterog., FF, fibrotic and normal lung, microscopic HC NSIP GGO +/- fibrosis Diffuse interstitial inflammation +/- fibrosis COP GGO, nodules, consolidation Patchy upper lungs, small airways, alveolar Granulation tissue plugs in alveolar ducts and alveoli AIP GGO, consolidation Diffuse, random Hyaline membranes, immature fibroblasts in alveolar spaces and interstitium to variable degree RB-ILD Bronchiectasis, GGO Upper lungs, bronchocentric Respiratory bronchiolitis surrounded by Ms in alveoli DIP Basilar, peripheral, alveolar Alveolar Ms in air spaces diffusely in the biopsy LIP GGO, nodules, cysts Patchy Lymphoid hyperplasia IIP Classification A joint statement of the American Thoracic Society (ATS) and European Respiratory Society (ERS) was produced to standardize the classification of the idiopathic interstitial pneumonias (IIPs) and to establish a uniform set of definitions and criteria for the diagnosis of IIPs. The classification of IIPs included 7 clinical/radiologic/pathologic entities that are listed in this slide in order of relative frequency: Idiopathic pulmonary fibrosis/usual interstitial pneumonia Nonspecific interstitial pneumonia Cryptogenic organizing pneumonia Acute interstitial pneumonia Respiratory bronchiolitis-associated interstitial lung disease Desquamative interstitial pneumonia Lymphoid interstitial pneumonia Histologic, lung distribution, and pathologic characteristics of these conditions are summarized in this table. The diagnostic process will be addressed in more detail later in this section. HC, honeycombing; GGO, ground glass opacity; FF, fibrotic foci; M, macrophage American Thoracic Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med. 2002;166: Thannickal VJ, Toews GB, White ES, Lynch JP 3rd, Martinez FJ. Mechanisms of pulmonary fibrosis. Annu Rev Med. 2004;55: Strollo DC. Imaging of idiopathic interstitial lung diseases. Concepts and conundrums. Am J Respir Cell Mol Biol. 2003;29(3 Suppl):S10-S18.

Inhaled environmental agents (fumes, dust, smoke) Delivery &
Four proposed mechanisms and potential variations in lung responses to inhaled agents Inhaled environmental agents (fumes, dust, smoke) Delivery & persistence Genetic predisposition Alveolar epithelial cell injury Biochemical Wound healing (inflammation, coagulation, epithelial/endothelial repair) Immunologic Fibrotic Chronic airflow obstruction Normal Pulmonary fibrosis

Recent Hypothesis: Inflammatory hypothesis Epithelial Cell Apoptosis
Angiogenesis Abnormal Matrix Turnover Th1 versus Th2 Cytokines Growth Factor Production Altered Fibroblast Phenotypes Myofibroblast Recruitment and Maintenance

AGE GENETIC FACTORS ENVIRONMENTAL FACTORS NATURE OF INJURY
LUNG INJURY AGE GENETIC FACTORS ENVIRONMENTAL FACTORS NATURE OF INJURY – Etiologic agent – Recurrent vs single – Endothelial vs epithelial Histopathologic Pattern Histopathological Patterns of IIPs A recent review of the mechanisms of pulmonary fibrosis by Thannickal and colleagues explained how histopathologic changes in the lung can be quite diverse, with overlapping patterns of inflammation and fibrosis. “Histopathologic patterns of idiopathic interstitial pneumonias (IIPs) represent a spectrum of tissue reactions with varying degrees of inflammation and fibrosis. This reaction pattern probably depends on multiple factors, including age, genetic susceptibility, environmental factors, and perhaps the nature of the injurious agent.” Thannickal VJ, Toews GB, White ES, Lynch JP 3rd, Martinez FJ. Mechanisms of pulmonary fibrosis. Annu Rev Med. 2004;55: DIP RB-ILD LIP COP NSIP AIP UIP Inflammation Fibrosis Thannickal VJ, et al. Annu Rev Med. 2004;55:

Approach to the Diagnosis of ILD
Clinical History Physical Laboratory PFTs Radiology Chest X-ray HRCT Pathology Surgical lung biopsy Primary care physicians Pulmonologists Radiologists Pathologists Approach to the Diagnosis of IPF The current recommended approach to diagnosing IPF is multidimensional and multidisciplinary, comprising clinical, radiologic, and pathologic assessments and relying on the expertise of pulmonologists, pathologists, radiologists, and primary care physicians. Clinical evaluation begins with a thorough and extensive medical history including signs and symptoms, family history, and medication use. Dyspnea is the most prominent presenting symptom of IPF. Physical exam may reveal “velcro-like” crackles, most prominent at the lung bases, in more than 80% of patients. As the disease progresses, rales, clubbing (in 25% to 50% of patients), or cyanosis may develop. A routine laboratory evaluation is often helpful to rule out other causes of diffuse parenchymal lung disease. Pulmonary function tests typically reveal a restrictive pattern (reduced vital capacity and total lung capacity) and impaired gas exchange (reduced DLCO). Resting arterial blood gases may be normal; however, exercise typically results in an O2 desaturation. Chest x-rays may reveal bilateral reticular opacities with a basal predominance. Importantly, however, a normal chest x-ray does not exclude a diagnosis of IPF. Usual interstitial pneumonia is the histopathological pattern associated with the clinical entity of IPF, and can best be identified through surgical lung biopsy (thoracotomy or VATS). Multidimensional and multidisciplinary

ILD presents a clinical conundrum as;
1st at least 150 clinical entities and situation are associated with ILD. 2nd difficulty to determine the best specific diagnostic approach. 3rd a conclusive cause cannot be ascertained (even after lung biopsy) in a significant portion of patients. Finally even when a specific diagnosis is made, an effective therapeutic regimen is not available for many patients with ILD.

Diagnosis History The patient's age, cigarette-smoking status and sex may provide useful clues. Thorough medical history that must include a review of environmental factors, occupations, exposures, medication, and drug usage and family medical history.

Age: Infancy and childhood: Follicular bronchiolitis
Cellular interstitial pneumonia Acute idiopathic pulmonary hemorrhage of infancy

Age (cont.): Before age 40:
Familial idiopathic pulmonary fibrosis Metabolic storage disorders Hermansky pudalic syndrome Other inherited interstitial lung diseases Collagen vascular disease- associated ILD LAM Pulmonary Langerhans’cell granulomatosis Sarcoidosis After age 50: IPF 1 in 500 people over the age of 75 yrs.

Race: Sarcoidosis occurs folds among blacks.

Gender : Gender clearly affects the way patients present with pulmonary fibrosis: Men tend to present later in the disease, whereas women tend to present earlier. Women : Collagen vascular disease- associated ILD LAM Tuberous sclerosis Men: Pneumoconiosis

History (cont.) Smoking – related ILD :
Desquamative interstitial pneumonia. RBILD. Pulmonary Langerhans’ cell histiocytosis. IPF. Rheumatoid arthritis associated ILD. Acute eosinophilic pneumonia.

Smoking (cont.) Cigarette smoking is associated with a 1.6- to 2.3- fold excess risk of pulmonary fibrosis. The recognition that theses diseases are related to smoking is not just a matter of cinematic the cornerstone of therapy for theses patients is smoking cessation, in absence of which, immunosuppressive therapy may have no effect whatsoever.

ILD by onset and duration:
Acute onset (days to weeks): AIP Acute pneumonitis from collagen vascular disease (especially SLE) COP Drugs DAH Eosinophilic lung disease Hypersensitivity pneumonitis

ILD by onset and duration (cont.):
Subacute (weeks to months): Collagen vascular disease- associated ILD COP Drugs Subacute hypersensitivity pneumonitis Chronic (months to years): Chronic hypersensitivity pneumonitis Collagen vascular diseaes- associated ILD IPF and NSIP Occupation – related lung diseases.

History (cont.) Farming or exposure to known causes of hypersensitivity pneumonitis including birds, drugs, humidifiers. History of aspiration, dysphagia, arthritis, recurrent sinusitis, pneumothorax, muscle and skin symptoms, dry and gritty eyes, dry mouth and hemoptysis.

Physical examination Physical examination of the respiratory system is rarely helpful in the diagnostic evaluation of interstitial lung diseases. The classical “Velcro rales” or inspiratory crackles, occur not only in most patients with IPF but also in many other interstitial lung diseases.

Clubbing : Eighty percent of patients with clubbing have a respiratory disorder. Among patients with ILD clubbing is found in % of patients with IPF and 50% of patients with DIP and 75% of patients with ILD from rheumatoid arthritis.

Physical examination (cont.)
Extrathoracic findings can be insightful e.g. Skin abnormalities, peripheral lymphadenopathy and hepatosplenomegally are commonly associated with sarcoidosis. Iridocyclitis, uveitis or conjunctivitis may be associated with sarcoidosis.

Physical examination Characteristic skin rashes and lesions occur in collagen vascular diseases, disseminated histocytosis-X, tuberous sclerosis and neurofibromatosis. Signs of arthritis may be associated with sarcoidosis or collagen vascular diseases Sclerdactyly, Raynaud's phenomenon and telangiectatic lesions are characteristics features of scleroderma and CREST syndrome. Epilepsy, mental retardation in tuberous sclerosis. Diabetes insipidus in Langerhans cell granulomatosis

Chest Radiographic pattern
First review previous chest radiographs as this allows the clinician to ascertain the onset, progression, chronicity and stability of patient's disease. A rare patient with ILD will present with a normal chest radiograph. When radiographic abnormalities are noted, their distribution and appearance are useful in narrowing the differential diagnosis of ILD.

Radiographic Clues (cont.)
Mid/upper lung field disease: sarcoidosis, silicosis, ankylosing spondylitis, histiocytosis X. Lower lung field predominance: asbestosis, idiopathic pulmonary fibrosis, collagen vascular disease. Kerley B lines: congestive heart failure, lymphangitic carcinoma, LAM. Pleural plaques/ thickening: asbestosis.

Pleural effusion: congestive heart failure, lupus, rheumatoid arthritis, LAM, drug induced. Hilar adenopathy: sarcoidosis (bilateral and symmetrical), lymphangitic carcinoma (unilateral). Preserved lung volumes: sarcoidosis, histiocytosis X, LAM. Thin walled cysts (better seen on HRCT): histiocytosis X, LAM.

Photographic negative of pulmonary edema: Chronic eosinophilic pneumonia. Recurrent pneumothorax: Langerhans’ cell granulomatosis. LAM Tuberous sclerosis. Neurofibromatosis.

Computed tomography and high-resolution CT images
CT and HRCT scans are more sensitive and have a greater ability to detect anatomic abnormalities than do chest radiograph. Its impressive sensitivity help both in ruling out a diagnosis of ILD and in defining the parenchymal, pleural and mediastinal abnormalities in these disorders. It helps the surgeon to identify areas of non-fibrotic, active disease and relatively unaffected areas to guide appropriate site selection for biopsy.

HRCT (Cont.) HRCT helps in identifying "active and reversible inflammation" (ground glass attenuation) and irreversible fibrotic manifestations (traction bronchiectasis, bronchiolectasis and honeycombing). Extensive fibrotic changes suggest end or advanced stage disease with limited potential for both invasive diagnostic and therapeutic approaches which could be toxic.

HRCT has the potential for differentiating sarcoidosis, lymphangitic carcinomatosis and bronchiolitis. The presence of cystic images within the parenchyma raises the possibilities of three major cystic ILD; LAM, Tuberous sclerosis and Langerhans cell granulomatosis In LAM and Tuberous sclerosis, the cysts are numerous, thin walled, typically less than 2 mm in diameter and distributed throughout the pulmonary parenchyma. In Langerhans cell granulomatosis cysts are bizar shaped and distributed predominantly in the upper lobes.

In acute hypersensitivity pneumonitis HRCT show multifocal diffuse ground glass attenuation despite a normal chest radiograph. Smokers with symptomatic RBILD typically have patchy ground glass attenuation on HRCT. IPF is characterized by patchy subpleural and basilar fibrosis. A normal HRCT does not exclude the presence of microscopic ILD in a patient with a high pretest probability of the disorder.

Pulmonary physiology testing
Regardless of the cause, a restrictive lung defect and decreased diffusing capacity (DLco) are the predominant physiological abnormalities seen in ILD. Decreased FEV1, FVC, TLC The (PAO2 – PaO2) difference, at rest or with exercise may be normal or increased.

Differential diagnosis by function:
When there is a decrease in MVV out of proportion to the decrease in FEV1 and a decrease in maximal inspiratory pressures, diseases such as polymyositis, scleroderma and SLE should come to mind. A mixed pattern of obstructive and restrictive abnormalities may be present when ILD coexists with COPD or Asthma.

Differential diagnosis by function (cont.):
ILD associated with asthma or recurrent bronchospasm include; Churg-Strauss syndrome, ABPA, Sarcoidosis (endobronchial) and tropical pulmonary interstitial eosinophilia. Resting pulmonary function tests: Document the existence, gauge the severity and provide clues that are useful in the differential diagnosis of ILD. Also they are useful in the monitoring of clinical progression of the disease or response to therapy.

Exercise affords the most sensitive diagnostic and physiological test for ILD. The degree of arterial hypoxemia induced by exercise and the alveolar-arterial difference in P02 (PAO2 – PaO2 gradient) correlate well with the degree of pulmonary fibrosis.

Routine laboratory tests
Include: Complete blood count, leucocytic differential ESR Chemistry profile (calcium, liver function tests, electrolytes, renal function tests) Screening for collagen vascular diseases and urine analysis. When appropriate, creatinine kinase, aldolase, and angiotensin converting enzyme levels should be measured.

Bronchoscopy with transbronchial biopsy
Provide additional information, especially when tissue abnormalities tend to be distributed in peribronchiovascular areas e.g. Sarcoidosis, LAM and Lymphangitic carcinomatosis. It may disclose certain distinctive abnormalities e.g. Tight, uniform, well formed non caseating granulomas of sarcoidosis. Smooth muscle proliferation of LAM. Lymphatic metastasis of malignant cells. Giant cell granulomas are suggestive of hard metal pneumoconiosis. It is diagnostic if an infectious agent or malignancy is detected.

"Bronchoalveolar lavage"

Surgical lung biopsy: Thoracoscopy-Guided and open lung biopsy
Surgical lung biopsy remains the “gold standard” for diagnosis. It is, however, by no means always definitive: the size of specimens, site of biopsy, expertise of pathologists and interobserver differences among pathologists are factors that may preclude a conclusive diagnosis. The site of the biopsy should be chosen on the basis of HRCT findings and ideally be at the interface of involved and less involved lung tissue. A biopsy of more than one site in the lung is more helpful.

Surgical lung biopsy: Thoracoscopy-Guided and open lung biopsy
TGLB or open lung biopsies merit consideration as the final diagnostic step. Which patient are suitable candidates for these procedures? Unexplained dyspnea on exertion or abnormal results on pulmonary function testing favor such interventions (normal chest radiographs or HRCT scans do not negate the need for tissue diagnosis). On the other hand, not all patients with typical clinical features compatible with IPF require surgical lung biopsy for definitive diagnosis.

Diagnostic approach to suspected ILD

American Journal of Respiratory Cell and Molecular Biology VOL

Diagnostic Criteria for IPF in the Absence of a Surgical Lung Biopsy
Major Criteria Exclusion of other known causes of ILD Evidence of restriction and/or impaired gas exchange HRCT: bibasilar reticular abnormalities with minimal ground glass opacities TBB or BAL that does not support an alternative diagnosis Minor Criteria Age > 50 years Insidious onset of otherwise unexplained dyspnea on exertion Duration of illness > 3 months Bibasilar, inspiratory, Velcro® crackles Diagnostic Criteria for IPF in the Absence of a Surgical Lung Biopsy This slide illustrates the diagnostic criteria for IPF in the absence of a surgical lung biopsy. Presence all of the major diagnostic criteria as well as at least 3 of the minor criteria increases the likelihood of a correct clinical diagnosis of IPF. Major diagnostic criteria include: Exclusion of other known causes of interstitial lung disease (ILD) such as certain drug toxicities, environmental exposures, and connective tissue diseases Abnormal pulmonary function studies that include evidence of restriction and/or impaired gas exchange Bibasilar reticular abnormalities with minimal ground glass opacities on HRCT scans Transbronchial lung biopsy or bronchoalveolar lavage showing no features to support an alternative diagnosis Minor diagnostic criteria include: Age > 50 years Insidious onset of otherwise unexplained dyspnea on exertion Duration of illness > 3 months Bibasilar, inspiratory crackles (dry or “Velcro” type in quality) American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International Consensus Statement. Am J Respir Crit Care Med. 2000;161: All major criteria and at least 3 minor criteria must be present to increase the likelihood of an IPF diagnosis ATS/ERS. Am J Respir Crit Care Med. 2000;161:

Treatment The therapeutic regimen used for patients with ILD needs to be tailored to the patient and the disease process (disease-specific intervention). Avoidance of the offending agent or its environment. The use of corticosteroids, alone or in combination with immunosuppressives (azathioprine, cyclophosphamide) is currently recommended for most patients with chronic fibrotic lung disorders. However the clinical response is variable and unpredictable, some ILDs generally have a better prognosis and response more favorably than do others.

Novel therapies in IPF A number of agents that interfere with collagen synthesis have been tested: Pirfenidone (A pyridone molecule) IFN--1b (A glycoprotein) cost > $ per patient per year in USA. IFN-ß-1a. Colchicine D-Penicillamine (A chelating agent). N-acetylcysteine (Antioxidant). Captopril (ACE inhibitor). Bosentan (Endothelin-1 receptor antagonist). Imatinib mesylate (A protein-tyrosine kinase inhibitor).

Novel therapies in Sarcoidosis
Hydroxychloroquine: is effective for control of cutaneous sarcoid and has been successfully used to treat sarcoid-associated hypercalcemia, arthritis, neurological disease, and pulmonary disease. Infliximab and etanercept (TNF-alpha inhibitor). Pentoxifyllin and Thalidomide (TNF-alpha antagonists). Methotrexate (10-25mg / week).

Novel therapies in ILD associated with CT disease
Infliximab. Bosentan.

Novel therapies in ILD due to pulmonary alveolar proteinosis
Granulocyte Macrophage Colony Stimulating Factor (GM-CSF): A cytokine that stimulates the granulocytes, macrophages, dendritic cells, and bone marrow precursors of platelets. Administered by either S.C injection or aerosolized form. It has recently been demonstrated to effectively control disease course and provide a very useful alternative to traditional therapy of whole lung lavage

Treatment of LAM Estrogen-containing medications should be discontinued. Oophorectomy, progesterone, Tamoxifen and luteinizing hormone-releasing hormone analogs have been used with limited success. Lung transplantation offers the only hope for cure despite reports of recurrent disease in the transplanted lung.

Management of pulmonary hypertension complicating ILD
Beraprost sodium: prostacyclin analogue. Sildenafil : phosphodiestrase inhibitor. Bosentan: endothelin-1 antagonist. Theses medications may have beneficial effects that extend beyond vasodilatation, including anti-fibrotic and anti-inflammatory effects

Other measures Plasmapheresis is indicated in intractable and severe cases of alveolar hemorrhage syndrome resistant to corticosteroids and immunosuppressives. Supplemental oxygen is indicated to maintain adequate oxygen saturation. Unless contraindicated, all patients should receive pneumococcal and periodic influenza vaccinations. Other supportive measures such as rehabilitation are indicated in appropriate patients. Lung transplantation is a viable surgical option for selected patients who don't respond to currently available therapeutic regimens.

Final Comments The interstitial lung diseases are a fascinating collection of lung diseases that occur at any age group and may develop as a consequence of an extraordinarily broad collection of systemic diseases. The importance of a careful history and physical examination cannot be overstated, and may obviate many expensive diagnostic tests. The diagnosis and management of interstitial lung diseases often requires active discussion and collaboration between the clinician, surgeon, pathologist and radiologist.

Final Comments Recent studies challenge the dogma that lung biopsy is the gold standard for diagnosing interstitial lung disease. Rather than the lung biopsy per se, the new gold standard for the diagnosis is the combined input from the diagnostic studies (radiology, pathology, and functional testing) and clinical evaluation that allows a confident diagnosis in many situations.

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