Restrictive lung diseases or restrictive ventilatory defects
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).
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 < 0.001) compared with predicted values in all variables except gas transfer coefficient (Kco) at initial presentation, consistent with a 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).
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