Pathophysiology of Interstitial Pulmonary Fibrosis

Herbert, F. A.

doi:10.1001/archinte.1962.03620230074012

Cited by 42 publications

(4 citation statements)

References 12 publications

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“…These findings are consistent with those generally accepted in pulmonary sarcoidosis (16)(17)(18), systemic sclerosis (19)(20)(21), idiopathic interstitial pulmonary fibrosis (22)(23)(24)(25), and other diseases with pulmonary fibrosis (26)(27)(28)(29). A restrictive ventilatory defect has been the most frequently described early abnormality in these diseases.…”

Section: Discussionsupporting

confidence: 91%

Diffusing capacities and ventilation: perfusion ratios in patients with the clinical syndrome of alveolar capillary block

Arndt¹,

King²,

Briscoe³

1970

J. Clin. Invest.

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A B S T R A C T Studies were performed on 10 patients with the clinical syndrome of alveolar capillary block while each patient was breathing four different inspired oxygen mixtures. The data were interpreted using the principle of the Bohr integral isopleth with which alveolar oxygen tension in the differently ventilated parts of the lung can initially be treated as unknown. It is then possible to determine the distribution of ventilation, of perfusion, of diffusing capacity, of lung volume, and of alveolar and end capillary blood oxygen tension in the variously functioning parts of the lung. In two patients shunts were the major factor interfering with oxygen transfer. In four others inequalities in ventilation: perfusion ratios and in diffusing capacity in different parts of the lung were the factors interfering with oxygen transfer. In four more patients ventilation: perfusion ratios were the same throughout the lung, the only disturbance of oxygen transfer being in the total diffusing capacity or in its distribution between the different parts of the lung.

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Section: Discussionsupporting

confidence: 91%

Diffusing capacities and ventilation: perfusion ratios in patients with the clinical syndrome of alveolar capillary block

Arndt¹,

King²,

Briscoe³

1970

J. Clin. Invest.

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“…Improvement occurred in 41 % of treated patients and was substantial (grade 2) in 16%. This compares with an overall improvement of 34% in 64 patients described in four published reports (Herbert, Nahmias, Gaensler, and MacMahon, 1962;Ford, Giacobine, Madoff, and Sachs, 1964;Livingstone et al, 1964;Ander, 1965). From three of these reports it is possible to estimate that a substantial improvement, corresponding to our grade 2, occurred in 15% of patients.…”

Section: Discussionmentioning

confidence: 52%

The prognosis of cryptogenic fibrosing alveolitis

Stack¹,

Choo-Kang²,

Heard³

1972

Thorax

226

120

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The course of cryptogenic fibrosing alveolitis (CFA) in 96 patients is described. The mean survival from the onset of the first symptom until death was 47-4 months in all the 59 fatal cases, 49-6 months in those dying from CFA only, and 48-4 months in those dying from CFA in whom the course of the illness was not influenced by corticosteroids. There was a wide variation in the natural course of the disorder, varying from death within a year of the onset of symptoms in five patients to prolonged survival with slow or arrested progress in four patients not treated with corticosteroids. Most patients died from CFA but five developed bronchial carcinoma and two died after lung biopsy.The main factor influencing the prognosis was the effectiveness of corticosteroid drugs. Only 16% of the treated patients had a worthwhile (grade 2) response to these drugs and the five-year survival of this group was 67 %. In those patients in whom the course of the inness was uninfluenced by corticosteroids the five-year survival rate was only 20%. The histological appearances on lung biopsy gave a rough guide to the likely effect of corticosteroid therapy.Other factors influencing the prognosis were the degree of dyspnoea on presentation, the vital capacity, and the presence of arterial hypoxaemia. Patients who had had symptoms for more than four years before the first hospital attendance had a relatively good prognosis.By contrast, neither the extent of radiographic abnormality nor the presence of circulating rheumatoid and antinuclear factors influenced subsequent survival.

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“…The term desquamative interstitial pneumonia (DIP) was first proposed by Liebow in 1962 1 , 2 and further cases were subsequently reported 3 ,. 4 It may be an early phase of cryptogenic fibrosing alveolitis (CFA) 5 –9 but some believe it is a different entity from this disease 10 ,.…”

Section: Introductionmentioning

confidence: 99%