Diffuse pulmonary alveolar fibrosis

Scadding, J. G. (1974).Thorax, 29, 271-281. Diffuse pulmonary alveolar fibrosis. The problems of diagnostic categorization of diffuse pulmonary alveolar fibrosis are outlined, and attention is drawn to the different implications of categories defined aetiologically and histopathologically. The pattern of pulmonary insufficiency associated with changes of these sorts is described, and the importance of using a terminology of pulmonary insufficiency which refers unequivocally to disorders of function is emphasized. The usage of the word `interstitial' in the context of inflammations of the lung is critically examined. It seems to be used to imply predominant involvement of alveolar walls, which in other contexts would be regarded as `parenchyma'. This unresolved conflict which accepted anatomical usage can be avoided by general acceptance of complementary conventions that `pneumonia' refers to inflammations of the lung characterized by exudation into alveolar spaces leading to consolidation, and `alveolitis' to those affecting predominantly alveolar walls. Some cases of alveolitis, so defined, can be shown to be due to reactions with inhaled organic dusts in the gas-exchanging part of the lungs in specifically sensitized individuals, constituting a group of aetiologically defined categories `extrinsic allergic alveolitis'. A few are due to ingested toxic substances. Many remain of unknown cause and show a strong and early tendency to alveolar wall fibrosis: they constitute a histopathologically defined category `cryptogenic fibrosing alveolitis'.     

Diffuse pulmonary angiomatosis.

A six year old girl with diffuse pulmonary angiomatosis presented with haemoptysis and diffuse interstitial changes with bilateral pleural effusions on the chest radiograph. The lung lesion as seen on biopsy specimens (and confirmed at necropsy) consisted of bloodless, thin walled, endothelium lined channels, affecting the interstitial septae, pleura, bronchi, and adventitia of large vessels. There was no response to oral corticosteroids or a trial of cyclosphamide. This lesion may be an example of an angiogenic disease.     

Diffuse pulmonary fibrosis and hiatus hernia

Six patients presenting with initially obscure pulmonary fibrosis are described. Reasons are given for attributing the condition to chronic aspiration pneumonitis from gastro-oesophageal reflux in association with hiatus hernia, supported by lung histology in three cases. Study of a further series of 143 cases of hiatus hernia suggests a 4% incidence of this insidious complication in addition to any more acute bronchopulmonary episodes. This possibility should be remembered in the differential diagnosis of any patient with pulmonary fibrosis of doubtful origin, as the advanced condition bears a poor prognosis.     

Diffuse persistent interstitial pulmonary emphysema treated by lobectomy

Persistent interstitial pulmonary emphysema is a rare condition that occurs in preterm infants on mechanical ventilation, characterized by abnormal accumulation of air in the pulmonary interstitium, due to disruption of the basement membrane. Diffuse persistent interstitial pulmonary emphysema is observed when small cysts are noted in all lobes of the lung. The management of infants suffering from diffuse persistent interstitial pulmonary emphysema varies according to severity and stability of the patient, being either conservative treatment or aggressive surgical treatment by pneumonectomy. We report a case of an unstable patient with diffuse persistent interstitial pulmonary emphysema successfully treated by lobectomy as a form of conservative surgical approach.     

Diffuse pulmonary hemorrhage. An uncommon manifestation of Wegener's granulomatosis

We report two cases of Wegener's granulomatosis with the unusual manifestation of diffuse alveolar hemorrahge. One patient with well-documented Wegener's granulomatosis developed alveolar hemorrhage 4 weeks after leukopenia necessitated the discontinuation of cyclophosphamide. The second patient presented with pulmonary hemorrhage and died 10 days after an open-lung biopsy in which histologic features of Wegener's granulomatosis were overshadowed by alveolar hemorrhage. Lung biopsies in both cases showed marked alveolar hemorrhage and pulmonary capillaritis. The importance of recognizing capillaritis and other subtle histologic features of Wegener's granulomatosis are emphasized.     

Diffuse pulmonary infiltrates after bone marrow transplantation: the role of open lung biopsy

Background

Diffuse pulmonary infiltrates is the major complication and cause of mortality after bone marrow transplantation. We analyzed the etiologies and prognostic factors in bone marrow recipients with diffuse pulmonary infiltrates and assessed the role of open lung biopsy in managing this complication.

Methods

Medical records of patients with diffuse pulmonary infiltrates after bone marrow transplantation were reviewed. Possible prognostic factors were analyzed by multivariate logistic regression.

Results

Sixty-eight (20%) of 341 bone marrow recipients had diffuse pulmonary infiltrates and 34 died. Thirty-five underwent open lung biopsy, resulting in therapeutic changes in 22 (63%) and clinical improvement in 16 (46%). The leading diagnoses were idiopathic interstitial pneumonitis (40%) and cytomegalovirus pneumonitis (20%). Cytomegalovirus pneumonitis caused radiographically observable interstitial infiltrates exclusively and was frequently associated with hepatitis. Idiopathic interstitial pneumonitis resulted in either diffuse ground-glass opacity or interstitial infiltrates. Three (9%) patients had miliary tuberculosis. Respiratory failure (p < 0.001) and acute graft-versus-host disease (p = 0.016) were the poor prognostic factors.

Conclusions

Among bone marrow recipients, we found diffuse pulmonary infiltrates in 20% and a mortality rate of 50%. Idiopathic interstitial pneumonitis and cytomegalovirus pneumonitis were the most common causes and should be suspected in patients with diffuse interstitial infiltrates. In endemic areas, miliary tuberculosis should be suspected in bone marrow recipients with diffuse reticulonodular lesions. Respiratory failure and acute graft-versus-host disease were poor prognostic factors. By establishing a correct diagnosis, open lung biopsy led to treatment changes in about two-thirds of these patients.     

Diffuse pulmonary fibrosis and the Hermansky-Pudlak syndrome: clinical course and postmortem findings.

The Hermansky-Pudlak syndrome consists of albinism, platelet function defect, pigment laden macrophases and, on occasions, pulmonary fibrosis. The clinical course and postmortem findings of a patient with pulmonary fibrosis which mimicked cryptogenic fibrosing alveolitis are reported. Histological examination revealed a chronic inflammatory infiltrate of pigment laden microphages.