Restrictive Lung Disease Clinical Presentation

Updated: Mar 30, 2017
  • Author: Jonathan Robert Caronia, DO; Chief Editor: John J Oppenheimer, MD  more...
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Presentation

History

The initial evaluation of patients should consist of a complete history, including a total review of past systemic conditions. A careful history of occupation, travel, habits, hobbies, exposures, and HIV risk factors is critical to help identify any etiologic agent or trigger.

Duration of illness

Acute disorders last days to weeks and include acute interstitial pneumonitis, eosinophilic pneumonia, and diffuse alveolar hemorrhage.

Subacute disorders lasting weeks to months include sarcoidosis, drug-induced interstitial lung disease, alveolar hemorrhage syndrome, COP, and connective-tissue diseases. [8]

Chronic cases lasting months to years include idiopathic pulmonary fibrosis (IPF), sarcoidosis, and pulmonary Langerhans cell histiocytosis.

Hypersensitivity pneumonitis and cryptogenic organizing pneumonia (COP) may manifest as acute, subacute, or chronic disease.

Smoking history

Pulmonary Langerhans cell histiocytosis, desquamative interstitial pneumonitis, IPF, and respiratory bronchiolitis occur with increased frequency among persons who smoke or those who previously smoked.

Prior medication use

A detailed history of previously used medications is needed to exclude the possibility of drug-induced lung disease. These commonly used drugs are nitrofurantoin, amiodarone, gold, chemotherapeutic agents, procainamide, and hydralazine.

Radiation may also cause pneumonitis and fibrosis.

Family history

Familial associations include IPF, sarcoidosis, and LAM.

Occupational history

Seek a strict chronological listing of the patient's lifelong employment, including specific duties and known exposures.

Inhaled inorganic dust from substances (eg, silica, asbestos, beryllium, hard metals, cobalt) can cause pneumoconiosis. [9, 10]

Inhaled organic dust may cause hypersensitivity pneumonitis.

Environmental exposure

A review of the domestic and work environment of the patient and spouse is invaluable.

Symptoms of intrinsic diseases

Progressive exertional dyspnea is the predominant symptom. Grading the level of dyspnea is useful as a method to gauge the severity of the disease and to follow its course.

A dry cough is common and may be a disturbing sign. A productive cough is an unusual sign in most patients with diffuse parenchymal lung disorders.

Hemoptysis or grossly bloody sputum occurs in patients with diffuse alveolar hemorrhage syndromes and vasculitis.

Wheezing is an uncommon manifestation but can occur in patients with lymphangitic carcinomatosis, chronic eosinophilic pneumonia, and respiratory bronchiolitis.

Chest pain is uncommon in most instances of the disease, but pleuritic chest pain can occur in patients with rheumatoid arthritis, systemic lupus erythematosus, and some drug-induced disorders. [11]

Symptoms of extrinsic disorders

Nonmuscular diseases of the chest wall affect patients with kyphoscoliosis. Patients younger than 35 years tend to be asymptomatic, whereas middle-aged patients develop dyspnea, decreased exercise tolerance, and respiratory infections.

The cause of respiratory failure is often multifactorial and is secondary to spinal deformity, muscle weakness, disordered ventilatory control, sleep disordered breathing, and airway disease.

Neuromuscular disorders occur as the respiratory muscle weakness progresses. Patients develop dyspnea upon exertion, followed by dyspnea at rest, and their condition ultimately advances to respiratory failure.

Patients with neuromuscular diseases develop significant respiratory muscle weakness and may demonstrate fatigue, dyspnea, impaired control of secretions, and recurrent lower respiratory tract infections. In these patients, the central drive is not decreased. [12] Acute and chronic respiratory failure, pulmonary hypertension, and cor pulmonale eventually ensue.

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Physical Examination

Intrinsic disorders

The physical examination in patients with intrinsic lung disorders may yield distinguishing physical findings.

Velcro crackles are common in most patients with interstitial lung disorders.

Inspiratory squeaks or scattered, late, inspiratory high-pitched rhonchi are frequently heard in patients with bronchiolitis.

Cyanosis at rest is uncommon in persons with interstitial lung diseases, and this is usually a late manifestation of advanced disease.

Digital clubbing is common in those with idiopathic pulmonary fibrosis (IPF) and is rare in others (eg, those with sarcoidosis or hypersensitivity pneumonitis). See the image below.

Approximately half of the patients with idiopathic Approximately half of the patients with idiopathic pulmonary fibrosis develop clubbing. Clubbing is commonly seen in patients with asbestosis.

Extrapulmonary findings, including erythema nodosum, suggest sarcoidosis. A maculopapular rash can occur in those with connective-tissue diseases, or it may be drug-induced. Raynaud phenomenon may be present in patients with connective-tissue diseases, and telangiectasia is present in those with scleroderma. Peripheral lymphadenopathy, salivary gland enlargement, and hepatosplenomegaly are signs of systemic sarcoidosis. Uveitis may be observed in those with sarcoidosis and ankylosing spondylitis.

Cor pulmonale occurs in the late stages of pulmonary fibrosis or advanced kyphoscoliosis. Pulmonary hypertension and cor pulmonale become evident when signs include a loud P2, right-sided precordial lift, and right-sided gallop.

Extrinsic disorders

By their very nature, severe kyphoscoliosis and massive obesity are easily recognizable. The pleural disorders are associated with decreased tactile fremitus, dullness upon percussion, and decreased intensity of breath sounds.

In cases of neuromuscular diseases, the physical examination findings may indicate accessory muscles usage, rapid shallow breathing, paradoxical breathing, and other features of systemic involvement.

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Causes

Intrinsic lung diseases

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

Other causes may include drugs and other treatments (eg, nitrofurantoin, amiodarone, gold, phenytoin [Dilantin], 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, LAM, pulmonary vasculitis, alveolar proteinosis, eosinophilic pneumonia, and COP.

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

Organic dust exposure (eg, farmer's lung, bird fancier's lung, bagassosis, and mushroom worker's lung, which all cause hypersensitivity pneumonitis) is another cause.

Idiopathic fibrotic disorders

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

Extrinsic disorders

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.

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Complications

Acute exacerbation in patients with IPF is a recognized complication that occurs unpredictably and presents as worsening dyspnea. Chest radiography demonstrates bilateral mixed alveolar-interstitial infiltrates and CT scan reveals ground-glass opacities and consolidation. The treatment includes high-dose systemic corticosteroids, although these are likely not effective, and the worsening disease portends extremely poor prognosis.

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