Restrictive lung disease

Restrictive lung disease
ICD-9	518.89

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]

Overview

Restrictive lung diseases are a category of respiratory disease characterized by a loss of lung compliance, causing incomplete lung expansion and increased lung stiffness.

Complete Differential Diagnosis for Restrictive Lung Disease

• Abestosis
• Ankylosing spondylitis
• Bagassosis
• Berylliosis
• Bird Fanciers Lung
• Dermatomyositis
• Drug treatments
• Farmers Lung
• Fibrothorax
• Hard Metal Fibrosis
• Idiopathic kyphosis
• Interstitial Pneumonia
• Interstitial pneumonitis
• Lymphangioleiomyomatosis
• Massive Pleural Effusion
• Mushroom Worker Lung
• Myopathy
• Myositis
• Obesity
• Phrenic neuropathy
• Pneumoconiosis
• Polymyositis
• Pulmonary Vasculitis
• Rheumatoid Arthritis
• Sarcoidosis
• Scleroderma
• Silicosis
• Systemic Lupus Erythematosus
• Talc

Pathophysiology

The underlying process is usually pulmonary fibrosis (scarring of the lung). As the disease progresses, the normal lung tissue is gradually replaced by scar tissue interspersed with pockets of air. This can lead to parts of the lung having a honeycomb-like appearance.

Causes and classification

Restrictive lung diseases may be due to specific causes which can be intrinsic to the parenchyma of the lung, or extrinsic to it.

Intrinsic

• Asbestosis caused by long-term exposure to asbestos dust.
• Radiation fibrosis, usually from the radiation given for cancer treatment.
• Certain drugs such as amiodarone, bleomycin and methotrexate.
• As a consequence of another disease such as rheumatoid arthritis.
• Hypersensitivity pneumonitis due to an allergic reaction to inhaled particles.
• Acute respiratory distress syndrome (ARDS), a severe lung condition occurring in response to a critical illness or injury.
• Infant respiratory distress syndrome due to a deficiency of surfactant in the lungs of a baby born prematurely.

• Many cases of restrictive lung disease are idiopathic (have no known cause). Examples are:

• Idiopathic pulmonary fibrosis
• Idiopathic interstitial pneumonia, of which there are several types
• Sarcoidosis
• Eosinophilic pneumonia
• Lymphangioleiomyomatosis
• Pulmonary Langerhans cell histiocytosis
• Pulmonary alveolar proteinosis

• Conditions specifically affecting the interstitium are called interstitial lung diseases.

Extrinsic

Quadriplegia can be a cause of restrictive lung disease.

Clinical Presentation

The main symptoms are:

• Shortness of breath
• Cough

Diagnosis

• In restrictive lung disease, both the FEV1 and FVC are reduced so the FEV1/FVC ratio is normal or even increased in contrast to obstructive lung disease where this ratio is reduced. The values for residual volume and total lung capacity are generally decreased in restrictive lung disease.

• One definition requires a total lung capacity which is 80% of the expected value.

Related Chapters

• Chronic obstructive lung disease
• Treatments for COPD - How to overcome any lung-related illness.

Additional Resources

• Baughman RP, Lower EE. Use of intermittent, intravenous cyclophosphamide for idiopathic pulmonary fibrosis. Chest. Oct 1992;102(4):1090-4.
• Baydur A. Respiratory muscle strength and control of ventilation in patients with neuromuscular disease. Chest. Feb 1991;99(2):330-8.
• Bjoraker JA, Ryu JH, Edwin MK, et al. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. Jan 1998;157(1):199-203.
• Douglas WW, Ryu JH, Swensen SJ, et al. Colchicine versus prednisone in the treatment of idiopathic pulmonary fibrosis. A randomized prospective study. Members of the Lung Study Group. Am J Respir Crit Care Med. Jul 1998; 158(1): 220-5.
• Fishbein MC. Diagnosis: to biopsy or not to biopsy: assessing the role of surgical lung biopsy in the diagnosis of idiopathic pulmonary fibrosis. Chest. Nov 2005;128 (5 Suppl 1): 520S-525S.
• Flaherty KR, Martinez FJ, Travis W, Lynch JP 3rd. Nonspecific interstitial pneumonia (NSIP). Semin Respir Crit Care Med. Aug 2001;22(4): 423-34.
• Flaherty KR, Toews GB, Travis WD, et al. Clinical significance of histological classification of idiopathic interstitial pneumonia. Eur Respir J. Feb 2002; 19(2): 275-83.
• Gay SE, Kazerooni EA, Toews GB, et al. Idiopathic pulmonary fibrosis: predicting response to therapy and survival. Am J Respir Crit Care Med. Apr 1998;157(4 Pt 1):1063-72.
• Goldstein RH, Fine A. Potential therapeutic initiatives for fibrogenic lung diseases. Chest. Sep 1995;108(3):848-55.
• Hunninghake GW, Kalica AR. Approaches to the treatment of pulmonary fibrosis. Am J Respir Crit Care Med. Mar 1995; 151 (3 Pt 1): 915-8.
• Katzenstein AL, Myers JL. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. Am J Respir Crit Care Med. Apr 1998;157(4 Pt 1): 1301-15.
• Martinez FJ, Safrin S, Weycker D, et al. The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med. Jun 21 2005;142(12 Pt 1): 963-7.
• Mathieson JR, Mayo JR, Staples CA, Muller NL. Chronic diffuse infiltrative lung disease: comparison of diagnostic accuracy of CT and chest radiography. Radiology. Apr 1989; 171(1): 111-6.
• Muller NL. Clinical value of high-resolution CT in chronic diffuse lung disease. AJR Am J Roentgenol. Dec 1991;157(6):1163-70.
• Parambil JG, Myers JL, Ryu JH. Histopathologic features and outcome of patients with acute exacerbation of idiopathic pulmonary fibrosis undergoing surgical lung biopsy. Chest. Nov 2005;128(5): 3310-5.
• Peckham RM, Shorr AF, Helman DL. Potential limitations of clinical criteria for the diagnosis of idiopathic pulmonary fibrosis/cryptogenic fibrosing alveolitis. Respiration. Mar-Apr 2004;71(2): 165-9.
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• Remy-Jardin M, Remy J, Giraud F, et al. Computed tomography assessment of ground-glass opacity: semiology and significance. J Thorac Imaging. Fall 1993; 8(4): 249-64.
• Shah NR, Noble P, Jackson RM, et al. A critical assessment of treatment options for idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis. Oct 2005; 22(3): 167-74.
• Turner-Warwick M, Burrows B, Johnson A. Cryptogenic fibrosing alveolitis: response to corticosteroid treatment and its effect on survival. Thorax. Aug 1980;35(8): 593-9.
• Vianello A, Bevilacqua M, Salvador V, et al. Long-term nasal intermittent positive pressure ventilation in advanced Duchennes muscular dystrophy. Chest. Feb 1994; 105(2): 445-8.
• Wagner JD, Stahler C, Knox S, et al. Clinical utility of open lung biopsy for undiagnosed pulmonary infiltrates. Am J Surg. Aug 1992;164(2): 104-7; discussion 108.
• Wells A. Clinical usefulness of high resolution computed tomography in cryptogenic fibrosing alveolitis. Thorax. Dec 1998;53(12):1080-7.
• Winterbauer RH, Hammar SP, Hallman KO, et al. Diffuse interstitial pneumonitis. Clinicopathologic correlations in 20 patients treated with prednisone/azathioprine. Am J Med. Oct 1978;65(4):661-72.
• du Bois RM. Evolving concepts in the early and accurate diagnosis of idiopathic pulmonary fibrosis. Clin Chest Med. Mar 2006;27(1 Suppl 1):S17-25, v-vi.