Restrictive lung disease
| Restrictive lung disease | |
| ICD-9 | 518.89 |
|---|---|
| eMedicine | med/2012 |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
Restrictive lung diseases are a category of respiratory disease characterised by a loss of lung compliance,[1] causing incomplete lung expansion and increased lung stiffness.
Complete Differential Diagnosis for Restrictive Lung Disease
- Abestosis
- Anklosing 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.[2]
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.[3]
Clinical Presentation
The main symptoms are:
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[4].
One definition requires a total lung capacity which is 80% of the expected value.[5]
References
Template:Medical conditions Template:Respiratory pathology
- ↑ Sharma, Sat. "Restrictive Lung Disease". Retrieved 2008-04-19.
- ↑ "eMedicine - Restrictive Lung Disease : Article by Sat Sharma". Retrieved 2008-11-21.
- ↑ Walker J, Cooney M, Norton S (1989). "Improved pulmonary function in chronic quadriplegics after pulmonary therapy and arm ergometry". Paraplegia. 27 (4): 278–83. PMID 2780083. Unknown parameter
|month=ignored (help) - ↑ "www.spirXpert.com".
- ↑ Brack T, Jubran A, Tobin MJ (2002). "Dyspnea and decreased variability of breathing in patients with restrictive lung disease". Am. J. Respir. Crit. Care Med. 165 (9): 1260–4. PMID 11991875. Unknown parameter
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