The alphabet soup revisited: the chronic interstitial pneumonias in the 1990s.

Liebow classified the idiopathic interstitial pneumonias as usual (UIP), desquamative (DIP), bronchiolitis obliterans (BIP), lymphoid (LIP), and giant cell (GIP) interstitial pneumonias. This classification was modified to exclude LIP and GIP. UIP, the most common type, is characterized by synchronous foci of inflammation, collagen deposition, and fibrosis with interspersed normal lung. It usually affects men 40-60 years old and manifests radiologically with bilateral, basilar irregular opacities and volume loss. In most cases, a confident diagnosis can be made at high-resolution computed tomography because of characteristic subpleural irregular linear opacities, ground-glass opacities, honeycombing, and traction bronchiectasis. DIP affects younger patients and is characterized by diffuse intraalveolar macrophage aggregation. Typical radiologic features include bilateral, basilar ground-glass opacities and preserved lung volumes. BIP, renamed bronchiolitis obliterans with organizing pneumonia, affects middle-aged patients and manifests with multifocal plugs of immature fibroblasts in the air spaces. Typical radiologic features include bilateral consolidations and normal lung volumes. Recently described entities include acute (AIP) and nonspecific (NIP) interstitial pneumonias and respiratory bronchiolitis with interstitial lung disease (RB-ILD). AIP is a rapidly progressive, often fatal, illness characterized by diffuse alveolar damage and manifests with clinical and radiologic features of adult respiratory distress syndrome. NIP is a heterogeneous group of fibrosing disorders that cannot be otherwise classified. RB-ILD is a disease of smokers with a good prognosis.

Lymphoid interstitial pneumonia: clinicopathological and immunopathological findings in 18 cases.

Eighteen patients with lymphoid interstitial pneumonia (LIP) were studied. The diagnosis was established by the microscopic finding of interstitial infiltrates of lymphocytes and plasma cells. Forty-seven% of patients also had germinal centres, while 72% showed interstitial giant cells. Cases studied by the immunoperoxidase technique showed the interstitial plasma cells to be polytypic. The median age of patients was 56 years; most had cough, dyspnea, or chest pain. Chest X-rays showed either patchy interstitial infiltrates (usually bilateral) or poorly defined nodules. Ten patients had hypergammaglobulinemia; one had hypogammaglobulinemia. Two patients had Sjögren's syndrome, two had biopsy-proven chronic active hepatitis, and two had a clinical diagnosis of primary biliary cirrhosis. Follow-up examination of 14 patients showed clearing of symptoms, X-ray infiltrates or stable infiltrates in 4 cases each. Five patients died (mean survival, 41 months), one of whom succumbed to disseminated lymphoma and a second to respiratory failure. Our results support the hypothesis that LIP is a non-neoplastic cellular proliferation in which lymphoma may supervene. The high incidence (22%) of chronic liver disease has not previously been noted.

Lymphomatoid granulomatosis: a clinicopathologic study of 42 patients.

We studied the histological and clinicopathological findings in 42 patients who had lymphomatoid granulomatosis (LYG). In addition to small round lymphocytes, small to intermediate lymphocytes with serpentine nuclei, large immature mononuclear lymphoid cells, abundant histiocytes, and vascular invasion by the cell infiltrate were observed in all cases. Fifty percent of lesions had occasional "atypical" cells with multi-lobed nuclei. Three of four follow-up autopsies showed large cell lymphoma, while one other autopsy and the single repeat biopsy showed increased numbers of large immature mononuclear lymphoid cells. Patients were most frequently men 40-60 yr old who had a history of pulmonary symptoms, such as cough or chest pain, and who showed multiple bilateral lung nodules without hilar adenopathy in the chest x-ray. Thirteen patients (38%) died of disease, 11 of them within 12 mth of initial diagnosis. The presence of neurological signs and symptoms, increased mitoses, or increased numbers of atypical multi-nucleated cells in the initial biopsy were not statistically significant predictors of survival.

Amyloidosis presenting in the lower respiratory tract. Clinicopathologic, radiologic, immunohistochemical, and histochemical studies on 48 cases.

We studied 48 cases of amyloidosis localized to the lower respiratory tract. Fourteen cases were classified as tracheobronchial amyloidosis. Twenty-eight cases showed solitary or multiple nodules, and six cases had a diffuse interstitial parenchymal pattern. Almost all patients with tracheobronchial and diffuse interstitial amyloidosis had respiratory symptoms (usually dyspnea), whereas most with nodular amyloidosis were asymptomatic. Nodular parenchymal and, less frequently, tracheobronchial amyloidosis had contiguous cellular infiltrates of plasma cells, lymphocytes, and giant cells. Immunohistochemical staining of specimens from 18 cases showed these plasma cells to be polytypic, except for two tracheobronchial lesions that had a disproportionate number of lambda light chain-bearing plasma cells. Permanganate oxidation of specimens from 19 cases showed patterns in keeping with immunoglobulin-derived amyloid in 12. Local deposits of amyloid in lung may arise from deposition of circulating precursor proteins.

Pulmonary manifestations of juvenile laryngotracheal papillomatosis.

Juvenile laryngotracheal papillomatosis spreads to involve the lungs in less than 1% of cases, and when this occurs, the prognosis is poor. In seven such cases, the lung lesions, which appeared either solid or cystic on radiographs, proved to be benign squamous cell proliferations or papillomas, with central cavities containing debris or air. They seemed to grow centrifugally, using the alveolar walls as scaffolding with central coalescence and lung destruction. Papillomas spread inferiorly from the larynx by direct extension as far as the major bronchi, but rarely beyond. However, the parenchymal lesions were widely scattered, and some were subpleural. This discrepancy suggests that fragments become detached, particularly during endoscopic resection, and are carried down the airways by airflow. Those that lodge proximal to the respiratory bronchioles may be removed by mucociliary action and cough. Those that travel more distally are poorly cleared and may grow. If enough lung parenchyma is destroyed, the patient can develop symptoms of restrictive lung disease in addition to signs of upper airway obstruction.

Primary non-Hodgkin's lymphoma and pseudolymphoma of lung: a study of 161 patients.

The authors studied 161 cases of primary non-Hodgkin's lymphomas and pseudolymphomas of lung. Small lymphocytic proliferations, which they believe to be lymphomas, constituted 31.6 per cent of cases; plasmacytoid lymphocytic and small cleaved follicular center cell lymphomas (Lukes-Collins system), 22.4 and 11.8 per cent of cases, respectively; and the remaining follicular center cell lymphomas and B-immunoblastic sarcomas, 5.6 per cent of cases. Pseudolymphomas constituted 14 per cent of cases. Most patients were elderly and asymptomatic; in most cases a solitary nodule or infiltrate was observed on a chest radiograph. Radiographic evidence of effusion was found in both lymphomas and pseudolymphomas, but hilar adenopathy was restricted to lymphomas. A few peribronchial reactive germinal centers and intralesional giant cells/granulomas were seen frequently in unequivocal lymphomas, so their presence cannot be used to exclude neoplasia. A generally monomorphic cell population and invasion of bronchial cartilage or visceral pleura are suggestive of malignancy, whereas primitive cytologic appearance and invasion of lymph nodes or parietal pleura are pathognomonic of malignancy. Diffusely admixed mature lymphocytes and plasma cells with numerous reactive follicles suggest pseudolymphomas. Immunologic determination of clonality may be diagnostically definitive. Most localized lesions in lung were treated by surgical resection, whereas in cases of extensive pulmonary disease, biopsies were performed and patients were treated by chemotherapy or irradiation. Both lymphomas and pseudolymphomas recurred, most often within three years. Pseudolymphoma recurred only in lung. When distant spread of lymphoma occurred, it commonly involved extranodal sites. Only 18 of 101 patients with lymphoma died with or of tumor, and no patient with pseudolymphoma died of disease. Neither histologic subtype among the "small cell" lymphoid lymphomas nor the presence of regional node involvement was prognostically significant, but pleural effusion on the initial chest radiograph was a significant predictor of both recurrence and mortality.

Bronchocentric granulomatosis: roentgenographic manifestations.

The roentgenographic manifestations of bronchocentric granulomatosis were reviewed in 15 patients. Nine patients had mass lesions. Four had alveolar infiltrates and 2 presented with reticulonodular infiltrates . Correlation of these radiographic patterns with the pathologic findings helps explain the varied morphologic appearance of this disease on the chest radiographs. Upper lobe involvement and unilateral disease were predominant. Seven of 15 patients (47%) presented with solitary mass lesions. Pleural reaction was occasionally noted. Hilar adenopathy and cavitation were infrequent. There was little correlation between radiographic pattern and clinical presentation.

Pericardial tuberculoma. An unusual cause of mediastinal mass.

Pericardial tuberculoma can appear as a solitary mediastinal mass along the left-heart border. The authors discuss two cases and cite three additional cases to demonstrate that, although rare, pericardial tuberculoma merits consideration in the differential diagnosis of mediastinal masses, particularly in patients with histories of tuberculosis.