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Restrictive lung diseases or restrictive ventilatory defects

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Home of Kyle J. Norton for The Better of Living & Living Health Restrictive lung disease is a condition marked most obviously by a reduction in total lung capacity. A restrictive ventilatory defect may be caused by a pulmonary deficit, such as pulmonary fibrosis (abnormally stiff, non-compliant lungs), or by non-pulmonary deficits, including respiratory muscle weakness, paralysis, and deformity or rigidity of the chest wall(1).

A. Symptoms
1.  Dyspnea (shortness of breath)
Patients with obstructive and restrictive ventilatory abnormalities suffer from exercise intolerance and dyspnea. Breathing pattern components (volume, flow, and timing) during incremental exercise may provide further insight in the role played by dynamic hyperinflation in the genesis of dyspnea parameters(2).

2. Wheezing 
Wheezing occurs during breathing, as a result of some parts of the respiratory tree must be narrowed or obstructed due to inflammation of the airways expiration causes of a blockage in the airways or lungs.

3. Chronic coughing 
Chronic cought is a insistently repetitive or continuous cough last longer than eight weeks.
According to the study of 10 patients with ulcerative colitis, all of whom were non-smokers, presented with a productive cough. In six, the chest radiograph was normal and cough was the only symptom; three of these patients had a minor obstructive ventilatory defect on testing. Four patients complained
of exertional dyspnoea and had both an abnormal chest radiograph with bilateral pulmonary shadows and a mixed obstructive and restrictive ventilatory defect(3).

4. Coughing up blood
It may be a result due to bleeding of the upper Respiratory tract or lung tumor.
5. Chest pain
Half of patients with respiratory Diseases are experience symptoms of chest pain(4).

6. Etc.

B. Causes and Risk factors
B.1. Causes
The causes of restrictive lung disease are unknown but some researchers suggested the below
1. Cystic fibrosis 
Recurrence is almost certainly more frequent in cystic fibrosis, and in view of the hazards of complication and the danger of bilateral pneumothoraces pleurodesis has to be considered. Of the various methods available, parietal pleurectomy is undoubtedly associated with the lowest incidence of
recurrence (Bromley, 1967; Heckscher, Larsen, and Lassen, 1966) and does not usually cause a restrictive ventilatory defect (Gobbel et al., 1963), so that provided the procedure is without undue risk it is the method of choice in this disease. In all three cases reported here there were severe obstructive
and restrictive defects of ventilatory capacity, but there were no immediate and serious post-operative complications, probably because of energetic physiotherapy and chemotherapy, according to the study by Brompton Hospital(5).

2. Cigarette smoke and air pollution
Welders exposed for over 10 years showed a prevalence of respiratory abnormalities significantly higher than those exposed for less than 10 years (44.4 vs 13.3%) (P less than 0.01) thereby showing that occupational exposure to welding fumes resulted in increased prevalence of pulmonary impairment in the welders. Smoking also had a contributory role thereby suggesting an interaction between smoking and welding exposure on the prevalence of pulmonary impairment in the welders engaged in brassware industries(6). Other suggested that in the study to investigate symptomatology, lung function, and radiographic change over an average period of 8 to 9 yr, of Sixty-four subjects with asbestos-related diffuse pleural thickening attending the London Medical Boarding Centre for Respiratory Diseases, showed that Chest pain was a common symptom, occurring in over half of the subjects. Approximately one-third of the subjects had a history of pleurisy or pleural effusion. Full long function, available in all cases, showed a highly significant decrement (p restrictive ventilatory defect(7).
3.  Osteogenesis imperfecta (OI)
 Osteogenesis imperfecta (OI) is an inherited connective tissue disorder characterized by bone fragility, multiple fractures and significant chest wall deformities. In the study of seven patients with severe OI type III, 15 with moderate OI type IV and 26 healthy subjects, found that the restrictive respiratory pattern of Osteogenesis Imperfecta is closely related to the severity of the disease and to the sternal deformities. Pectus carinatum characterizes OI type III patients and alters respiratory muscles coordination, leading to chest wall and rib cage distortions and an inefficient ventilator pattern. OI type IV is characterized by lower alterations in the respiratory function(8).

4. Connective tissue diseases
In the study to perform PFT in rheumatoid arthritic patients without pulmonary involvement and to identify variables related to changes in PFT over 5 years of follow-up in 82 patients (21 men, 61 women), found that among the 67 surviving patients, 38 (56.7%) agreed to participate in a follow-up study. The initial PFT revealed normal PFT in only 30 patients (36.6%); an obstructive ventilatory defect in 2 (2.4%), a small airway defect in 12 (17%), a restrictive ventilatory defect in 21 (25.6%), and reduced DLco in 17 (20.7%). Among the 38 patients participating in the 5 year follow-up study, 8 developed respiratory symptoms, one patient had a new obstructive ventilatory defect, one patient developed a restrictive ventilatory defect, and 5 patients had a newly developed small airway defect(9).

5. Etc.

B.2. Risk factors
1. Second hand smoke and genetic influence
Cigarette smoke not only causes Respiratory  Disease, including Restrictive lung disease but also can influence early immune function as a potential to interact with other genetic factors and environmental risk factors to influence disease propensity(10).

2. Obesity
Obesity, particularly severe central obesity, affects respiratory physiology both at rest and during exercise as a result of ventilatory defect due to reductions in expiratory reserve volume, functional residual capacity, respiratory system compliance and impaired respiratory system. In the study by National Taiwan University Hospital, showed that obesity may also impair upper airway mechanical function and neuromuscular strength, and increase oxygen consumption, which in turn, increase the work of breathing and impair ventilatory drive. The combination of ventilatory impairment, excess CO(2) production and reduced ventilatory drive predisposes obese individuals to obesity hypoventilation syndrome(11).

3.  Race
African Americans are at higher risk than white race in development of Restrictive lung disease.

4. Age
As the disease is a result of progression over prolonged period of time, most patients with restrictive lung disease are older than 50 years.

5. Etc.

C. Diseases associated with restrictive lung diseases
1. Heart diseases
In the study to explore the association of COPD and restrictive lung function impairment, respectively, with heart diseases in the general population in a cross-sectional study of 642 randomly selected 22- to 72-year-old subjects in northern Swede, found that there is a strong association between COPD and cardiovascular diseases and indicates a strong association between restrictive lung function and heart diseases. Both obstructive and restrictive lung function impairments were common among subjects with heart diseases and vice versa(12).

2. Ulcerative colitis
There is an association between ulcerative colitis and lung disease in which patients have chronic cough and show hyperplastic and inflammatory changes in their bronchial mucosa, according to the study by
Guy’s Hospital and Brook General Hospital(13).

3. Syringomyelia and syringobulbia
There is a report of three patients with syringomyelia and syringobulbia who developed severe respiratory complications. In neurological examination showed evidence of IXth and Xth cranial nerve involvement with dysphagia and dysphonia, but there were no complaints of serious sleep difficulties. Two patients died during sleep and the other was resuscitated during a nap. All patients showed moderate restrictive ventilatory defects with reduced maximal buccal pressures and one also showed a low ventilatory response to CO2 rebreathing(14).

4. Sarcoidosis
In the study to assess various lung function indices, including C(L,s) and D(L,CO), as markers of functional abnormality in sarcoidosis patients from 830 consecutive patients referred for lung function tests with a diagnosis of sarcoidosis (223 in stage I, 486 in stage II and 121 in stage III), found that
Normal total lung capacity was found in 772 (93%) patients. Of these cases, 24.5% had a low C(L,s) and 21.5% had a low D(L,CO). At least one abnormality was observed in 39.3% of these patients, whereas, in restrictive patients, this figure was 88%. Airway obstruction was present in 11.7% of cases(15).

5. Neuromuscular disease
The earliest sign of respiratory compromise in the patient with neuromuscular disease is nocturnal hypoventilation, which progresses over time to include daytime hypoventilation and eventually the need for full-time mechanical ventilation(16).

6. Other diseases
According to the study by Department of Public Health and Clinical Medicine, Umeå University, in restrictive lung function the prevalence of chronic rhinitis, cardiovascular disease, hyperlipemia and diabetes was higher compared to in Nlf (41.0% vs 32.3%, p = 0.017, 59.0% vs 41.0%, p<0.001, 29.2% vs.12.9%, p = 0.033, 20.9% vs 8.6%, p <0.001). In COPD and heart disease, 62.5% had chronic rhinitis and/or GERD, while in Nlf the corresponding proportion was 42.5%(17).

7. Etc.

D. Diagnosis 
If you are experience some of the above symptoms and if your doctor suspects that you have restrictive lung function, after recording your family history and completing the physical exam, the test which your doctor orders include 
1. Chest CT scan and echocardiography
In the study to examine the frequency and spectrum of diseases associated with isolated reduction in the diffusing capacity of lung for carbon monoxide (D(Lco)) of the 38,095 patients who underwent PFTs during the study period, 179 (0.47%; 95% confidence interval [CI], 0.40%-0.54%) had isolated D(Lco) abnormalities. The 27 patients (15.1%; 95% CI, 10.2%-21.2%) who had also undergone chest CT and echocardiography within 1 month of PFTs form the study cohort reported herein. Their mean D(Lco) was 50% +/- 15% (95% CI, 45%-56%) with average normal pulse oxygen saturation at rest and mild hypoxemia with activity. Thirteen of the 27 patients (48%; 95% CI, 28.7%-68.1%) had underlying emphysema evident on CT. Eleven of these 13 patients had emphysema associated with a restrictive lung process. The 14 patients without emphysema had interstitial lung disease, pulmonary vascular disease, and other isolated findings(18).

2. Utility of cardiopulmonary exercise testing
Restrictive lung disease occurs commonly in patients with neuromuscular disease. Exercise testing is mandatory because pulmonary function tests at rest are not reliable for the diagnostic evaluation and functional characterisation of these patients. Cardiopulmonary exercise testing (CPET) with measurement of gas exchange is the favoured tool. It is an excellent method to investigate exercise dyspnea, describe altered physiological response to exercise and characterise the involved organ systems heart, lung and muscle(19).

3. FEV1/FVC ratio test
The aim of the test is to analyze the  forced expiratory volume in one second (FEV1) and forced vital capacity (FVC). In patients with restrictive lung disease, ratio is higher than 80% as the decline in FVC is more than that of FEV1. In the study use of clinical and spirometry findings in order to distinguish between the restrictive and nonspecific patterns of pulmonary function test results in patients with low FVC and a normal or elevated FEV1/FVC ratio, showed that the most common causes of a nonspecific pattern were obstructive disorders, congestive heart failure, obesity, bronchiolitis, interstitial diseases, and neuromuscular disorders. In patients given a working diagnosis of pulmonary fibrosis, pleural disease, or chest wall disease, the positive predictive value (PPV) for restriction was >or= 90%. In males, an FVC restrictive pattern was found in 84.4% of those with an FVC or= 0% between the FEV1% and the FVC% had a PPV for restriction of 89.5%. After performing logistic regression, we developed a point scale for predicting the restrictive pattern(20).

4. Spirometry
Spirometry measures the flow and volume of air entering and leaving the lungs. It is used to assess ventilatory function and differentiates between normality and diseases causing obstructive and possibly restrictive defects(21).

4. Etc.

E. Preventions
1. Lose weight if you are Obese
Obesity is associated to the increased risk of Restrictive lung disease. According to the study to evaluate the effects of obesity on the pulmonary function of adult women, showed that the alterations evidenced in the components of the vital capacity (inspiratory reserve volume and expiratory reserve volume) suggest damage to the chest mechanics caused by obesity. These factors probably contributed to a reduction of the maximal voluntary ventilation(22).

2.  Quit smoking
A similar trend was observed in the control group indicating that smoking had a deteriorating effect on spirometric tests. The results of the pulmonary function tests showed a predominantly restrictive type of pulmonary impairment (12.3%) followed by a mixed ventilatory defect (8.7%)(23).

3. Prevention of occupational causes of the diseases and second smoke
In the study to investigate symptomatology, lung function, and radiographic change over an average period of 8 to 9 yr, of Sixty-four subjects with asbestos-related diffuse pleural thickening attending the London Medical Boarding Centre for Respiratory Diseases, showed that Chest pain was a common symptom, occurring in over half of the subjects. Approximately one-third of the subjects had a history of pleurisy or pleural effusion. Full long function, available in all cases, showed a highly significant decrement (p restrictive ventilatory defect(24).

4. Traditional Mediterranean diet
Eating plenty of fruit, vegetables and fish keep lungs healthy. Those who follow a diet closest to this ” Mediterranean ” ideal are less likely as their peers with eating habits furthest from this pattern to develop chronic lung disease. A good amount of fresh vegetable juices are a good way to start healing, along with adding a variety of spices such as ginger, onions, and garlic. The emphasis should be on eating as healthy a diet as possible(25).

5. Etc.

F. Treatments
F.1. In conventional medicine perspective
Treatments are depending to the underline causes in diagnosis
F.1.1. Non medication Therapies
1. Pulmonary rehabilitation
Pulmonary rehabilitation programs improve exercise tolerance, muscle strength, and dyspnea in patients with COPD. In the study to assess prospectively the effectiveness and feasibility of pulmonary rehabilitation in patients with restrictive lung diseases by Department of Respiratory Medicine, Ghent University Hospital, showed that patients with RLD respond well after 12 weeks of pulmonary rehabilitation, and even better results were seen after 24 weeks. Clinically significant improvements were obtained in the majority of the patients after 24 weeks(26).

2. Oxygen therapy
Collective experience with pulmonary rehabilitation and disease management has shown that patients with lung diseases including COPD and restrictive lung diseases live a longer and more productive quality of life if they can remain active(27).

3. Continuous positive airway pressure (CPAP)
The aim of CPAP is to provide continuous positive pressure to maintain a continuous level of positive airway pressure of that can improve oxygenation in a patient whose hypoxemia is refractory to oxygen therapy. Evidence suggests that use of CPAP for longer than 6 hours decreases sleepiness, improves daily functioning, and restores memory to normal levels(28).

4. Mechanical ventilation support
The aim of Mechanical ventilation is to mechanically assist or replace spontaneous breathing, if the patient is on his/her way to respiratory failure. High levels of prolonged pressure support ventilation promote diaphragmatic atrophy and contractile dysfunction. Furthermore, similar to controlled mechanical ventilation, pressure support ventilation-induced diaphragmatic atrophy and weakness are associated with both diaphragmatic oxidative stress and protease activation(29).

F.1.2.  Medication therapy
1. Inhaled corticosteroids 
Inhaled corticosteroids act locally in the lungs to inhibit the inflammatory process and support the function to relieve the symptoms of Restrictive lung disease. In the study of nine pregnant women with interstitial and restrictive lung disease between 1981 and 1994, showed that hree patients had severe disease, characterized by vital capacity corticosteroids. One patient had an adverse outcome; she was delivered at 31 weeks and required mechanical ventilation for 72 hours. All other patients were delivered at or beyond 36 weeks with no adverse intrapartum or postpartum complications(30).
2. Immunosuppressive therapy 
The aim of Immunosuppressive therapy is to reduce the immune response. Some researchers suggested that adverse drug reactions should be considered in patients with concomitant lung and liver disease(31).

3. Reducing fluid buildup in the lungs
If the underlined causes of the disease is as a result of cardiac problem.

F.1.3. Surgery
If the underlined causes as a result of  abdominal problems, such as obesity, remove abdominal tumors, repair diaphragmatic hernias, etc. In the study to describe the outcome of surgical treatment for pediatric patients with forced vital capacity (FVC) <40% and severe vertebral deformity, showed that corrective scoliosis surgery in pediatric patients with severe restrictive lung disease is well tolerated, but the management of this population requires extensive experience with the vertebral surgery involved, and a multidisciplinary approach that includes pulmonologists, nutritionists and anesthesiologists. Currently, there is no indication for routine preoperative tracheostomy(32). 

F.2. In herbal medicine perspective
The aim od herbal medicine is to prevent lung infections and relieve its symptoms. Herbs support the lungs, strengthen the immune system and repair tissues, include
1. Herbs support the lungs
In the study of the effect of astragalus membranceus(fisch) bge, codonpsis pilosula and glycyrrhiza uralensis fisch on airway responsiveness, found that after 28 asthmatic patients received the treatment of those herbs, the results showed that FVC value increased obviously (t = 2.217, P herbs (P
2. Herbs enhance immunity 
In the study of the effect of Chinese herbs (Astragalus radix and Ganoderma lucidum) on immune response of carp in fish fed diets containing Astragalus (0.5%), Ganoderma (0.5%) and combination of two herbs (Astragalus 0.5% and Ganoderma 0.5%) for 5 weeks, showed that  feeding non-vaccinated and vaccinated carp with combination of Astragalus and Ganoderma stimulated respiratory burst activity, phagocytosis of phagocytic cells in blood and lysozyme and circulatory antibody titres in plasma in vaccinated carp. Fish challenged with A. hydrophila had variable survival. The best survival (60%) was in vaccinated group fed with both herbs, while almost 90% of control fish (negative control) and 60% of fish vaccinated only (positive control) died(34).

3. Herbs repair tissues
According to the study by Ohio State University’s Heart and Lung Research Institute, Grape-seed extractseemed to aid wound healing in two ways: It helped the body make more of a compound used to regenerate damaged blood vessels, and it also increased the amount of free radicals in the wound site. Free radicals help clear potentially pathogenic bacteria from a wound(35).
F.3. In traditional Chinese medicine perspective
 Pulmonary fibrosis (PF) is a restrictive lung disease that may occur idiopathically or as a complication of many diseases. In the study to test a new Chinese medicine formula DSQRL for the treatment of experimental PF in comparison with prednisone, conducted by Hong Kong Baptist University, Hong Kong, showed that at the end of 30 days treatment, the DSQRL treatment achieved a better outcome (p<0.05) than prednisone in terms of histological examination, bronchoalveolar lavage fluid analysis, hydroxyproline assay and complications(36). In other study by the same university to compare the Chinese medicine formula DSQRL from glucocorticoids, indicated that at the end of 60 days treatment, the DSQRL treatment achieved a significantly better outcome than prednisolone in terms of general behavior, histological examination, hydroxyproline content of the lung and inflammatory cell counts in bronchoalveolar lavage fluid(37).

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