Restrictive Lung Disease

Restrictive Lung Disease

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Restrictive lung diseases are characterized by reduced lung volumes, either because of an alteration in lung parenchyma or because of a disease of the pleura, chest wall, or neuromuscular apparatus. Unlike obstructive lung diseases, such as asthma and chronic obstructive pulmonary disease (COPD), which show a normal or increased total lung capacity (TLC), restrictive disease are associated with a decreased TLC. Measures of expiratory airflow are preserved and airway resistance is normal and the forced expiratory volume in 1 second (FEV1)/forced vital capacity (FVC) ratio is increased. If caused by parenchymal lung disease, restrictive lung disorders are accompanied by reduced gas transfer, which may be marked clinically by desaturation after exercise.

The many disorders that cause reduction or restriction of lung volumes may be divided into two groups based on anatomical structures.

The first is intrinsic lung diseases or diseases of the lung parenchyma. The diseases cause inflammation or scarring of the lung tissue (interstitial lung disease) or result in filling of the air spaces with exudate and debris (pneumonitis). These diseases can be characterized according to etiological factors. They include idiopathic fibrotic diseases, connective-tissue diseases, drug-induced lung disease, environmental exposures (inorganic and organic dusts), and primary diseases of the lungs (including sarcoidosis).

The second is extrinsic disorders or extrapulmonary diseases. The chest wall, pleura, and respiratory muscles are the components of the respiratory pump, and they need to function normally for effective ventilation. Diseases of these structures result in lung restriction, impaired ventilatory function, and respiratory failure (eg, nonmuscular diseases of the chest wall, neuromuscular disorders).

The mnemonic “PAINT” has been used to divide the causes of restrictive lung disease into pleural, alveolar, interstitial, neuromuscular, and thoracic cage abnormalities.

Table 1. Causes of Restrictive Lung Disease (Open Table in a new window)

Causes

Examples

Diagnosis

PFT Findings

Pleural

Trapped lung, pleural scarring, large pleural effusions, chronic empyema, asbestosis

Radiography, CT scanning, pleural manometry, pleural biopsy

Low RVa, low TLC, low FVC

Alveolar

Edema, hemorrhage

Radiography, CT scanning, physical examination

Increased DLCOb in hemorrhage (Intrapulmonary hemoglobin absorbs the carbon monoxide, thus increasing the DLCO reading.)

Interstitial

Interstitial lung disease including IPFc, NSIPd, COPe

Radiography, CT scanning, physical examination, echo often shows pulmonary hypertension

Low RV, low FVC, low TLC, decreased DLCO, poor lung compliance

Neuromuscular

Myasthenia gravis, ALSf, myopathy

Physical examination, EMGsg, serology

Low RV, low TLC, low NIFh, low MMVi

Thoracic/extrathoracic

Obesity, kyphoscoliosis, ascites

Physical examination

Low ERVj and FRC in obesity, low VCk, TLC, FRCl in kyphoscoliosis

aResidual volume.

bDiffusion capacity of the lungs for carbon monoxide.

cIdiopathic pulmonary fibrosis.

dNonspecific interstitial pneumonitis.

eCryptogenic organizing pneumonia.

fAmyotrophic lateral sclerosis.

gElectromyography.

hNegative inspiratory force.

iMaximal voluntary ventilation.

jExpiratory reserve volume.

kVital capacity.

lFunctional residual capacity.

Air flows to and from the alveoli as lungs inflate and deflate during each respiratory cycle. Lung inflation is accomplished by a contraction of respiratory, diaphragmatic, and external intercostal muscles, whereas deflation is passive at rest. Functional reserve capacity (FRC) is the volume of air in the lungs when the respiratory muscles are fully relaxed and no airflow is present. The volume of FRC is determined by the balance of the inward elastic recoil of the lungs and the outward elastic recoil of the chest wall. Restrictive lung diseases are characterized by a reduction in FRC and other lung volumes because of pathology in the lungs, pleura, or  structures of the thoracic cage.

The distensibility of the respiratory system is called compliance. Compliance is the volume change produced by a change in the distending pressure. Lung compliance is independent of the thoracic cage, which is a semirigid container. The compliance of an intact respiratory system is an algebraic sum of the compliances of both of these structures. Therefore, it is influenced by any disease of the lungs, pleura, or chest wall.

In cases of intrinsic lung disease, the physiological effects of diffuse parenchymal disorders reduce all lung volumes by the excessive elastic recoil of the lungs,  relative to the outward recoil forces of the chest wall. Expiratory airflow is reduced in proportion to lung volume.

Arterial hypoxemia in disorders of pulmonary parenchyma is primarily caused by ventilation-perfusion mismatching, with further contribution from an intrapulmonary shunt. Decreased diffusion of oxygen rarely contributes to hypoxemia because sufficient time still exists for full equilibration of oxygen or carbon dioxide. However, if transit time is significantly shortened, as with exercise, this can highlight the pathology with significant exercise-induced desaturation.

Collagen-vascular diseases, including scleroderma, polymyositis, dermatomyositis, systemic lupus erythematosus, rheumatoid arthritis, and ankylosing spondylitis, are potential causes of restrictive lung disease.

Other causes may include drugs and other treatments (eg, nitrofurantoin, amiodarone, gold, phenytoin, thiazides, hydralazine, bleomycin, bischloroethylnitrosourea [BCNU or carmustine], cyclophosphamide, methotrexate, radiation). Also see Drug-Induced Pulmonary Toxicity.

Causes related to primary or unclassified diseases may include sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis (LAM), pulmonary vasculitis, alveolar proteinosis, eosinophilic pneumonia, and cryptogenic organizing pneumonia (COP).

Inorganic dust exposure (eg, silicosis, asbestosis, talc, pneumoconiosis, berylliosis, hard metal fibrosis, coal worker’s pneumoconiosis, chemical worker’s lung) may cause restrictive lung disease.

Organic dust exposure can lead to hypersensitivity pneumonitis (eg, farmer’s lung, bird fancier’s lung, bagassosis, and mushroom worker’s lung, humidifier lung, hot tub pneumonitis).

These may include acute interstitial pneumonia, IPF (usually interstitial pneumonitis), lymphocytic interstitial pneumonitis, desquamative interstitial pneumonitis, and nonspecific interstitial pneumonitis.

Nonmuscular diseases of the chest wall, in which kyphosis can be idiopathic or secondary, may cause restrictive lung disease. The most common cause of secondary kyphoscoliosis is neuromuscular disease (eg, polio, muscular dystrophy). Fibrothorax, massive pleural effusion, morbid obesity, ankylosing spondylitis, and thoracoplasty are other causes.

Neuromuscular diseases manifest as respiratory muscle weakness and are due to myopathy or myositis, quadriplegia, or phrenic neuropathy from infectious or metabolic causes.

Pleural diseases, including trapped lung or asbestos-related pleural plaques, are an underrecognized, and potentially treatable, cause of restrictive lung disease.

United States

For intrinsic lung diseases, studies cite an overall prevalence of 3-6 cases per 100,000 persons. The prevalence of idiopathic pulmonary fibrosis (IPF) is 27-29 cases per 100,000 persons. [1, 2] The prevalence for adults aged 35-44 years is 2.7 cases per 100,000 persons. Prevalence exceeded 175 cases per 100,000 persons among patients older than 75 years. Exposure to dust, metals, organic solvents, and agricultural employment is associated with increased risk.

In North America, the prevalence of sarcoidosis is 10-40 cases per 100,000 persons. [3]

The incidence of chronic interstitial lung diseases in persons with collagen-vascular diseases is variable, but it is increasing for most diseases. [4]

Kyphoscoliosis is a common extrinsic disorder. It is associated with an incidence of mild deformities amounting to 1 case per 1000 persons, with severe deformity occurring in 1 case per 10,000 persons. [5]

Other nonmuscular and neuromuscular disorders are rare, but their incidence and prevalence are not well known.

According to the US Centers for Disease Control and Prevention (CDC), 35.9% of Americans older than 20 years are obese, and 69% of Americans are at least overweight (body mass index [BMI] 25-30). [6]

International

In Sweden, the prevalence rate for sarcoidosis is 64 cases per 100,000 persons. In Japan, the prevalence rate of sarcoidosis is 10-40 cases per 100,000 persons. [7] The prevalence of sarcoidosis is difficult to determine.

Pediatric data from England have demonstrated the prevalence of idiopathic scoliosis to be 1 case per 200 patients aged 6-14 years. [8]

The worldwide prevalence of fibrotic lung diseases is difficult to determine because studies have not been performed.

Although a familial variant of IPF exists, a genetic predisposition has not yet been elucidated. [9]

The incidence among black Americans is 35.5 cases per 100,000 persons. In contrast, the incidence among white Americans is 10.9 cases per 100,000 persons. [3]

LAM and lung involvement in tuberous sclerosis occur primarily in premenopausal women, although a handful of cases of LAM have been reported in men. Sporadic LAM has a prevalence of approximately 4.9 cases per 1,000,000 women. Men are more likely to have pneumoconiosis because of occupational exposure, IPF, and collagen-vascular diseases (eg, rheumatoid lung). Worldwide, sarcoidosis is slightly more common in women. [7]

IPF is rare in children. Some intrinsic lung diseases present in patients aged 20-40 years. These include sarcoidosis, collagen-vascular–associated diseases, and pulmonary Langerhans cell histiocytosis (formerly referred to as histiocytosis X). Most patients with IPF are older than 50 years. [10]

The natural history of interstitial lung diseases is variable. It depends on the specific diagnosis and the extent and severity of lung involvement based on high-resolution CT scanning and lung biopsy. [10] IPF is typically a relentless progressive disorder, and patients have a mean survival of 3-6 years after diagnosis. [11] Early recognition of IPF is important for directing patient management and predicting prognosis. [12]

Pulmonary sarcoidosis has a relatively benign self-limiting course, with spontaneous recovery or stabilization in most cases. [13] However, up to 20% of patients develop pulmonary fibrosis and disability. [14]

Prognosis for collagen-vascular diseases, eosinophilic pneumonia, cryptogenic organizing pneumonia (COP), and drug-induced lung disease is generally favorable with treatment. [15, 16, 17]

Patients with chest wall diseases and neuromuscular disorders develop progressive respiratory failure and often succumb during an intercurrent pulmonary infection. [18]

The mortality and morbidity from various causes of restrictive lung disease is dependent on the underlying cause of the disease process.

The median survival time for patients with IPF is less than 3 years. Factors that predict poor outcome include older age, male sex, severe dyspnea, history of cigarette smoking, severe loss of lung function, appearance and severity of fibrosis on radiologic studies, lack of response to therapy, and prominent fibroblastic foci on histopathologic evaluation. [11]

See the image below.

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Causes

Examples

Diagnosis

PFT Findings

Pleural

Trapped lung, pleural scarring, large pleural effusions, chronic empyema, asbestosis

Radiography, CT scanning, pleural manometry, pleural biopsy

Low RVa, low TLC, low FVC

Alveolar

Edema, hemorrhage

Radiography, CT scanning, physical examination

Increased DLCOb in hemorrhage (Intrapulmonary hemoglobin absorbs the carbon monoxide, thus increasing the DLCO reading.)

Interstitial

Interstitial lung disease including IPFc, NSIPd, COPe

Radiography, CT scanning, physical examination, echo often shows pulmonary hypertension

Low RV, low FVC, low TLC, decreased DLCO, poor lung compliance

Neuromuscular

Myasthenia gravis, ALSf, myopathy

Physical examination, EMGsg, serology

Low RV, low TLC, low NIFh, low MMVi

Thoracic/extrathoracic

Obesity, kyphoscoliosis, ascites

Physical examination

Low ERVj and FRC in obesity, low VCk, TLC, FRCl in kyphoscoliosis

aResidual volume.

bDiffusion capacity of the lungs for carbon monoxide.

cIdiopathic pulmonary fibrosis.

dNonspecific interstitial pneumonitis.

eCryptogenic organizing pneumonia.

fAmyotrophic lateral sclerosis.

gElectromyography.

hNegative inspiratory force.

iMaximal voluntary ventilation.

jExpiratory reserve volume.

kVital capacity.

lFunctional residual capacity.

Features

AIP

UIP

NSIP

COP

Pathologic

Temporal appearance

Uniform

Heterogeneous

Uniform

Uniform

Interstitial inflammation

Scant

Scant

Usually prominent

Variable

Collagen fibrosis

No

Patchy

Variable, diffuse

No

Fibroblast proliferation

Diffuse, interstitial

Patchy (fibroblast foci)

Occasional

Patchy, airspace

COP areas

Rare

No

Rare

Honeycomb changes

Rare

Yes

Rare

No

Hyaline membranes

Yes, often focal

No

No

No

Jonathan Robert Caronia, DO Fellow, Department of Pulmonary and Critical Care Medicine, Lenox Hill Hospital, North Shore LIJ Health System

Disclosure: Nothing to disclose.

Chuan Jiang, MD Physician, Lenox Hill Hospital, Northwell Health

Chuan Jiang, MD is a member of the following medical societies: American College of Physicians, Society of Hospital Medicine

Disclosure: Nothing to disclose.

Klaus-Dieter Lessnau, MD, FCCP Clinical Associate Professor of Medicine, New York University School of Medicine; Medical Director, Pulmonary Physiology Laboratory; Director of Research in Pulmonary Medicine, Department of Medicine, Section of Pulmonary Medicine, Lenox Hill Hospital

Klaus-Dieter Lessnau, MD, FCCP is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Medical Association, American Thoracic Society, Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Daniel R Ouellette, MD, FCCP Associate Professor of Medicine, Wayne State University School of Medicine; Chair of the Clinical Competency Committee, Pulmonary and Critical Care Fellowship Program, Senior Staff and Attending Physician, Division of Pulmonary and Critical Care Medicine, Henry Ford Health System; Chair, Guideline Oversight Committee, American College of Chest Physicians

Daniel R Ouellette, MD, FCCP is a member of the following medical societies: American College of Chest Physicians, Society of Critical Care Medicine, American Thoracic Society

Disclosure: Nothing to disclose.

John J Oppenheimer, MD Clinical Professor, Department of Medicine, Rutgers New Jersey Medical School; Director of Clinical Research, Pulmonary and Allergy Associates, PA

John J Oppenheimer, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, New Jersey Allergy, Asthma and Immunology society

Disclosure: Received research grant from: quintiles, PRA, ICON, Novartis: Adjudication<br/>Received consulting fee from AZ for consulting; Received consulting fee from Glaxo, Myelin, Meda for consulting; Received grant/research funds from Glaxo for independent contractor; Received consulting fee from Merck for consulting; Received honoraria from Annals of Allergy Asthma Immunology for none; Partner received honoraria from ABAI for none. for: Atlantic Health System.

Laurie Robin Grier, MD Medical Director of MICU, Professor of Medicine, Emergency Medicine, Anesthesiology and Obstetrics/Gynecology, Fellowship Director for Critical Care Medicine, Section of Pulmonary and Critical Care Medicine, Louisiana State University Health Science Center at Shreveport

Laurie Robin Grier, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Society for Parenteral and Enteral Nutrition, Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Lalit K Kanaparthi, MD Attending Physician, North Florida Lung Associates

Lalit K Kanaparthi, MD is a member of the following medical societies: American College of Chest Physicians, American Medical Association, American Thoracic Society

Disclosure: Nothing to disclose.

Sat Sharma, MD, FRCPC Professor and Head, Division of Pulmonary Medicine, Department of Internal Medicine, University of Manitoba; Site Director, Respiratory Medicine, St Boniface General Hospital

Sat Sharma, MD, FRCPC is a member of the following medical societies: American Academy of Sleep Medicine, American College of Chest Physicians, American College of Physicians-American Society of Internal Medicine, American Thoracic Society, Canadian Medical Association, Royal College of Physicians and Surgeons of Canada, Royal Society of Medicine, Society of Critical Care Medicine, and World Medical Association

Disclosure: Nothing to disclose.

Restrictive Lung Disease

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