Follicular bronchitis/bronchiolitis.

Nineteen open lung biopsies demonstrating follicular bronchitis/bronchiolitis were reviewed with special attention to clinical manifestations. Morphologically, follicular bronchitis/bronchiolitis was represented by coalescent reactive germinal centers adjacent to airways in the absence of clinical or pathologic evidence of chronic obstructive pulmonary disease or bronchiectasis. Three clinicopathologic groups were identified: 1) patients with collagen vascular diseases, especially rheumatoid arthritis and Sjögren's syndrome; 2) patients with a familial form of the disease or with immunodeficiency syndromes; and 3) a heterogeneous group of patients with frequent peripheral blood eosinophilia, suggesting a hypersensitivity reaction. Prognosis was related to age at the time of biopsy and, to some extent, to the clinical group. Steroid therapy had inconsistent effects in all groups identified. The differential diagnosis of lymphoid lesions in the lung is also discussed.

Thoracic surgical problems in asbestos-related disorders.

Among 1,577 persons with asbestos exposure followed up from 3 to 30 years, 113 had thoracic surgical procedures for asbestos-related disorders. Twenty-six individuals suspected of having asbestosis with atypical features underwent open-lung biopsy; a different disease was revealed in 14. Most of the 29 patients with mesothelioma had a small thoracotomy for diagnosis only; chemotherapy in half of them proved entirely ineffective. Experience with 23 patients with bronchogenic carcinoma did not differ from that in persons not exposed to asbestos. Problems of causal relationship are discussed. Most of the 68 individuals with benign asbestos pleural effusion had no symptoms, but because of recurrence, 15 were operated on for decortication or for possible mesothelioma. Hyaline plaques often were mistaken for lung, rib, or diaphragmatic tumors, and sometimes mesothelioma was suspected. Operative intervention in the 24 patients with plaques could have been avoided by obtaining a more detailed occupational history and reviewing previous chest roentgenograms, which invariably showed identical or smaller plaques from 2 to 17 years earlier.

Lung biopsy in rheumatoid arthritis.

Forty open lung biopsies from patients with rheumatoid arthritis and possible "rheumatoid lung disease" were reviewed in an attempt to correlate histology with radiologic, physiologic, and prognostic variables. A wide variety of histopathologic features was seen, and primary and secondary patterns of injury were recognized. Five different groups based on histologic patterns were identified: pulmonary rheumatoid nodules, usual interstitial pneumonia (UIP), bronchiolitis obliterans with patchy organizing pneumonia (BOOP), lymphoid hyperplasia, and cellular interstitial infiltrates. The finding of rheumatoid nodules as the primary pattern imparted a uniformly good prognosis, whereas the pattern of UIP indicated a poor one. Patients with BOOP had a more favorable prognosis than did patients with UIP, as did patients with lymphoid hyperplasia and/or nonspecific cellular interstitial infiltrates. Consistent correlations between pulmonary function testing and roentgenographic and histologic findings were not found. The term "rheumatoid lung disease" is of no use as a histologic diagnosis because it encompasses a broad spectrum of morphologic changes that carry significantly different prognoses.

Bronchiolitis obliterans organizing pneumonia.

In 50 of 94 patients with bronchiolitis obliterans we found no apparent cause or associated disease, and the bronchiolitis obliterans occurred with patchy organizing pneumonia. Histologic characteristics included polypoid masses of granulation tissue in lumens of small airways, alveolar ducts, and some alveoli. The fibrosis was uniform in age, suggesting that all repair had begun at the same time. The distribution was patchy, with preservation of background architecture. Clinically, there was cough or flu-like illness for 4 to 10 weeks, and crackles were heard in the lungs of 68 per cent of the patients. Radiographs showed an unusual pattern of patchy densities with a "ground glass" appearance in 81 per cent. Physiologically, there was restriction in 72 per cent of the patients, and 86 per cent had impaired diffusing capacity. Obstruction was limited to smokers. The mean follow-up period was four years. With corticosteroids, there was complete clinical and physiologic recovery in 65 per cent of the subjects; two died from progressive disease. This disorder differs from bronchiolitis obliterans with irreversible obstruction. It was confused most often with idiopathic pulmonary fibrosis. In view of the benign course and therapeutic response, a histologic distinction is important.

Diffuse pleural thickening in an asbestos-exposed population: prevalence and causes.

Two types of pleural reaction have been described in association with asbestos exposure: pleural plaques and diffuse pleural thickening. This study was undertaken to determine the prevalence and causes of diffuse thickening in asbestos-exposed persons. Serial chest radiographs in 1373 exposed individuals and 717 controls were interpreted according to the ILO scheme by two B readers. Diffuse pleural thickening was defined as a smooth, noninterrupted pleural density extending over at least one-fourth of the chest wall, with or without costophrenic angle obliteration. Among the exposed group, plaques and diffuse thickening occurred with almost equal frequency, 16.5% and 13.5%, respectively. Of the 185 cases with diffuse thickening, the radiographic appearance was most often due to the residual of a benign asbestos effusion (31.3%) or confluent plaques (25.4%). The most commonly held explanation of diffuse thickening, an extension of pulmonary fibrosis to the visceral and parietal pleura, was actually infrequent (10.2%). Among the group with diffuse thickening without asbestosis, the forced vital capacity and single-breath diffusing capacity were significantly lower than those of comparable normal persons and those with confluent plaques.

Pulmonary veno-occlusive disease in the middle-aged.

Pulmonary veno-occlusive disease (PVO) is an uncommon cause of pulmonary hypertension which is difficult to diagnose without histology. We report 3 cases of histologically confirmed PVO. Our patients were all middle-aged or older, and 1 had spontaneous improvement in his symptoms suggesting that PVO may occur more commonly among older adults than previously believed and that indolent forms of the disease may exist. As new therapeutic approaches to the various entities causing pulmonary hypertension are developed, it is increasingly important to determine precisely which condition is present in a given patient.

Chronic diffuse infiltrative lung disease. Newer approaches.

A new scheme for description of diffuse infiltrative lung diseases using the graphic terminology of the International Labour Office Classification is described. Conventions for grading the type (rounded, or "pqr," and irregular, or "stu"), severity (profusion in 12 steps), localization of opacities, and pleural disease were retained. Modifications included (a) a third group of opacities, called "xyz," corresponding to reticulonodular patterns; and (b) "ground glass" (alveolar) patterns, subdivided into seven types by character and location. In a study of 365 cases proven by open biopsy, when this scheme was used without any knowledge of clinical data, the first two radiologic diagnostic choices corresponded with the principal histologic diagnosis in 50 per cent of cases. This classification provides an understandable and quantifiable system of communication and a tool for teaching, clinical research, and epidemiologic studies.

Diffuse infiltrative lung disease: a new scheme for description.

A scheme was devised for semiquantitative description of the diffuse infiltrative lung diseases using the graphic terminology of the International Labour Office and Union Internationale Contre le Cancer (ILO/UC) classification. Conventions for grading the type (rounded or "pqr" and irregular or "stu"), severity (profusion in 12 steps), localization of opacities, and pleural disease were retained. Modifications included: (a) a third group of opacities, called "xyz," corresponding to reticulonodular patterns; (b) "ground glass" (alveolar) patterns, subdivided into 7 types by character and location; (c) notations for severity of emphysema; and (d) hilar node enlargement. This initial study concerned diagnostic aspects and therefore was limited to 365 cases proved by open biopsy. When this scheme was used without any knowledge of clinical data, the first two radiologic diagnostic choices corresponded with the principal histologic diagnosis in 50% of cases. The biopsy diagnosis was mentioned among the first three choices in 78% of cases. It is concluded that this classification provides an understandable and quantifiable system of communication and a tool for teaching, clinical research, and epidemiologic studies.

Pulmonary lymphomas: current concepts.

The shift from a clinical definition (aggressive behavior) to an immunologic definition (clonal proliferations of cells) of lymphomas has led to a reinterpretation of most pulmonary pseudolymphomas as indolent lymphomas. The recognition of the diverse histologic appearance of lymphomas and the acceptance of extranodal lymphomas support the view that most cases of lymphomatoid granulomatosis are malignant lymphomas. Criteria for the recognition and classification of pulmonary lymphoid lesions are discussed.

Intravascular bronchioloalveolar tumor (IV-BAT).

Intravascular bronchioloalveolar tumor (IV-BAT) is a recently described, histologically distinct, malignant, primary lung neoplasm. Originally it was believed to arise from type II alveolar cells which invade blood vessels within the lung, and hence its name. More recent ultrastructural studies suggest an origin from mesenchymal cells, and therefore the tumor may be more appropriately considered a sarcoma. The onset of the disease may be insidious, and its clinical course may vary from slow to rapid progression. A history is present of a young woman with slowly enlarging multiple ill defined pulmonary nodules. Open lung biopsy was diagnostic of IV-BAT. The clinical course, differential diagnosis and pathogenesis of this tumor are briefly discussed.

A radiographic classification for sarcoidosis: physiologic correlation.

The chest roentgenogram is frequently used to judge severity and course of sarcoidosis. The only widely used method for staging, suggested by Siltzbach, does not provide for such judgments. Therefore, we devised a scheme for objective description of type and quantity of opacities based on the ILO/UC Classification for the Pneumoconioses. We added a "reticulonodular" category (x y z) to the present "rounded (p q r) and "linear-irregular" (s t u) categories. We retained the 11 point scale for profusion (severity) and added notations to describe ground glass (alveolar) patterns, size of nodes, and hilar retractions. Among 211 patients, x y z (35%) and p q r (33%) opacities predominated while s t u opacities (19%) were unusual. Radiographic severity correlated best with vital capacity (rs = -0.49) and the diffusing capacity (rs = -0.32). With the Siltzbach classification these correlations were not as good (rs = -0.27 and -0.19). Siltzbach Stage III (fibrosis) was a distinct group with poor function and frequent airway obstruction. There was no correlation between radiographic appearance and pathologic severity because the latter grading, on a scale from 0 to 10, never exceeded 3. Sequential studies in 64 patients showed that, when individuals are used as their own controls, overall profusion correlated highly with physiologic changes over time.

Pulmonary lymphomas simulating lymphomatoid granulomatosis.

Twenty cases of malignant lymphoma presenting in the lung and 10 cases with secondary pulmonary involvement were studied. All cases shared the feature of prominent vascular infiltration by lymphoid cells, and in the 20 cases presenting with pulmonary involvement, this feature led to confusion with lymphomatoid granulomatosis. Both the primary and secondary lymphomas showed similar histologic features including vascular infiltration, extensive necrosis, and foci of a histologically polymorphous and benign infiltrate. The diagnosis of lymphoma was based on the identification of monomorphous foci of atypical lymphoid cells except in the cases of Hodgkin's disease. The malignant cells were occasionally focal and microscopic and surrounded by an extensive histologically benign infiltrate. Examination of several blocks was often required in such cases before a diagnosis of lymphoma could be made. The clinical and radiologic findings at presentation were nonspecific. Radiologic findings included unilateral or bilateral nodules and infiltrates. The prognosis of the 20 patients who presented with pulmonary lymphoma was poor; half were dead in less than 2 years.

Comparison of transbronchial and open biopsies in chronic infiltrative lung diseases.

To ascertain the diagnostic reliability of transbronchial biopsy (TBB) a prospective comparison with open lung biopsy was made. All patients with undiagnosed diffuse infiltrative (interstitial) lung disease had a TBB. When this was characteristic of tumor, sarcoidosis, or infection, no additional biopsy was deemed necessary. In all other patients, an open biopsy was performed. Between January 1976 and May 1980, among 176 patients with interstitial disease, 60 (34.1%) required lung biopsy. Transbronchial biopsy was contraindicated in 7, leaving 53 in the study group. Among these 53 patients TBB was diagnostic in 20 (37.7%), and the histologic findings in the remaining 33 patients who lacked definitive tissue diagnoses were reported as normal lung, 10 patients; nonspecific abnormalities, 11 patients; interstitial pneumonia, 6; interstitial fibrosis, 3; inadequate specimen, 3. Open biopsy in these 33 patients resulted in specific diagnoses in 92%, and these diagnoses bore little relationship to the original TBB diagnoses. We concluded that TBB diagnoses of interstitial pneumonia, chronic inflammation, nonspecific reaction, and fibrosis are unreliable and often entirely misleading. In this group, an open biopsy is required to reach a specific histologic diagnosis.

Pulmonary involvement of malignant histiocytosis: a clinicopathologic spectrum.

We described five patients in whom pulmonary malignant histiocytosis was histologically confirmed during life. Pulmonary symptoms dominated the clinical presentation in three patients, and one patient had a 5-year history of pulmonary malignant histiocytosis controlled by steroids. Radiologic features were nonspecific. Most patients had bilateral reticulonodular or fluffy infiltrates. Recurrent pulmonary histiocytosis in one patient was manifest by bilateral pulmonary nodules. Pathologically, pulmonary infiltration by malignant histiocytosis followed the normal lymphatic pathways of the lung along bronchovascular rays, in interlobular septa, and within the pleura. Unusual features included marked septal edema and fibrosis out of proportion to the degree of infiltration, and in one case, the marked predilection fo the infiltrate to occlude small airways ("malignant histiocytosis bronchiolitis"). Three patients had microscopic nondestructive nodules adjacent to lymphatics. A pulmonary recurrent in one patient was composed of large monomorphous nodules with central necrosis and prominent vascular infiltration by malignant cells. The pulmonary infiltrate of malignant histiocytosis was often heterogeneous, and included variable numbers of lymphocytes and plasma cells intermingled with alveolar macrophages and metaplastic alveolar lining cells. The cytologic features of the infiltrate varied from benign to pleomorphic and obviously malignant. Histologic features which may obscure the correct diagnosis, as they did initially in three of our cases, include: malignant histiocytic bronchiolitis, marked septal edema and fibrosis; the heterogeneous cellular infiltrate; and in one case, benign cytologic features. The most valuable initial clue to the correct diagnosis was the tendency of the infiltrates to follow lymphatics of the lung.