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A symptomless 60 year old man with a heavy smoking history presented with a nodule in the right lung, which was found by an annual chest X-ray. Chest CT showed a 1 cm speculated nodule in the right S8. Bronchoscopic transbronchial biopsy to the nodule yielded no definite diagnosis. Since the nodule was highly suggestive of lung cancer, wedge resection was performed by video-assisted thoracoscopic surgery. Pathological findings of the specimen showed that the nodule consisted of a mixed population of histiocytoid cells with eosinophils. The nuclei and cytoplasms of the histiocytoid cells were stained positively for S-100 protein. Pulmonary eosinophilic granuloma (PEG) was diagnosed. Common radiographic findings of PEG should present with a mixture of multiple nodular shadows and cystic lesions. PEG presenting as a solitary nodule is rare.

CASE 1. A 55-year-old man was admitted because of an abnormality in chest radiographs. Chest HRCT showed multiple cystic lesions with thick and thin walls, and nodules; and strongly suggested pulmonary eosinophilic granuloma (EG). Open lung biopsy revealed granuloma formation and a fibrotic area consistent with EG. CASE 2. A 28-year-old woman was admitted because of chest pain and dyspnea. Chest radiography revealed bilateral pneumothorax. Chest HRCT showed multiple cystic lesions with thick walls involving the entire lung. As lung biopsy revealed, proliferative lesions and cavitation containing S-100 protein-positive histiocytes, we diagnosed this case as pulmonary EG in the active stage. CASE 3. A 32-year-old woman was admitted because of dyspnea. Chest CT showed bullous changes in the lung. As an open lung biopsy revealed, honeycomb changes with S-100 protein-positive histiocytes, this case was diagnosed as the regressive phase of EG. It is known that the chest radiography and HRCT findings of EG are characteristic and vary with the stage, so a surgical lung biopsy is necessary for diagnosis of EG. It is considered that the prognosis of the active phase of EG involving the entire lung is poor.

A 19-yr-old male presented with left lung parahilar consolidation and clinical signs of pneumonia. These symptoms were secondary to an endobronchial mass in the apical segment of the lower lobe. At thoracotomy an apical segment bronchial sleeve resection successfully extirpated what later proved to be an endobronchial eosinophilic granuloma. To the authors' knowledge this is the first case report of an endobronchial eosinophilic granuloma in an adult.

One clinical case of pulmonary eosinophilic granuloma were followed-up during 10 months of chemotherapy. Clinical and radiological symptoms of disease were described. Series of 4 HRCT examinations revealed improvement of pulmonary changes.

A case of pulmonary eosinophilic granuloma that underwent spontaneous remission is presented. A 23-year old man presented with dry cough and fever. Chest X-ray film revealed diffuse reticulo-nodular infiltrates in the middle and upper lung fields. Chest CT and HRCT showed multiple cystic lesions with thick walls and small nodules predominantly in the inner zone. Based on radiographic findings, pulmonary eosinophilic granuloma was suspected. Bronchoalveolar lavage cell data showed lymphocyte and eosinophil alveolitis with no increase of CD 1 lymphocytes. The symptoms and radiographic findings improved markedly within 4 months after the onset of symptoms without treatment and upon cessation of smoking. Chest CT and HRCT showed that the cystic walls were thinner and that the small nodules had decreased. Thoracoscopic lung biopsy revealed granulomatous lesions consisting of CD 1 and S-100 protein positive histiocytes with infiltration of eosinophils and fibrous lesions. Pulmonary eosinophilic granuloma was diagnosed. There has been no recurrence for 1 year.

A 32-year-old man was hospitalized due to right pneumothorax. Chest X-ray showed interstitial shadows predominantly in the upper and middle lung fields. Eosinophilic granuloma was diagnosed by transbronchial lung biopsy. He had a history of paraesthesia in the right arm for 6 months prior to the pneumothorax. Initially, head CT showed an iso-dense area enhanced with contrast medium in the left temporal lobe. Two weeks after the onset of paraesthesia, head CT showed an iso-dense lesion surrounded by low density area. Subjective symptoms disappeared in a month, and no abnormalities on head CT were seen at 2 months. It is highly suspected; therefore, that the present case of eosinophilic granuloma was associated with a brain lesion.

A case of pulmonary eosinophilic granuloma which was diagnosed by TBLB was presented. A 24-year-old male was admitted complaining of cough and fever of unknown etiology. He had a history of fever which had disappeared spontaneously 9 months previously. Laboratory examinations including blood gas analysis and pulmonary function tests showed no significant abnormalities. Chest roentgenogram showed faint linear opacities. Computed tomography revealed multiple small cysts and small nodular lesions mainly in the upper lung field. CT findings were strongly suggested pulmonary eosinophilic granuloma. TBLB was performed and 6 specimens were obtained, of which 4 showed granulomatous lesions consisting of histiocytosis X cell and inflammatory cells with infiltration of eosinophils. Furthermore, granulomatous lesions were positive for S-100 protein staining. The effectiveness of open lung biopsy has been emphasized in the diagnosis of pulmonary eosinophilic granuloma, however, TBLB is also useful for diagnosis, especially in the active or early stage of this disease.

S-100 protein immunostaining has been advocated to identify the characteristic Langerhans' cells in the histologic diagnosis of PEG. Reliable demonstration of an increased number of Langerhans' cells is essential in difficult biopsy cases, since occasional Langerhans' cells can be found in other pulmonary lesions. We examined the S-100 protein labeling pattern in three cases of PEG and in a variety of controls. Non-Langerhans' histiocytes were labeled for lysozyme antigen on the same histologic sections using a combined ABC and PAP technique. This verified that the S-100 protein-negative histiocytes were indeed a separate population from the S-100 protein-positive histiocytes and did not represent Langerhans' cells which failed to label with antiserum to S-100 protein. This technique confirms the usefulness of S-100 protein staining in the diagnosis of PEG and offers a means to verify the reliability of the S-100 protein labeling in questionable cases.

The clinical signs associated with heartworm disease are the result of changes in the pulmonary arterial system. These clinical signs are the result of either pulmonary hypertension or lung parenchymal disease associated with vascular changes. An increase in pulmonary arterial pressure produces an increase in right ventricular afterload, which may lead to exercise intolerance, syncope, and right-sided congestive heart failure. Coughing, dyspnea, and hemoptysis are the results of pulmonary parenchymal disease.

The field of research bronchoalveolar lavage has grown exponentially in the past decade. Despite this growth, the clinical role of lavage is uncertain. The authors review the clinical areas in which lavage has made its greatest impact as a diagnostic test (e.g. eosinophilic pneumonia, eosinophilic granuloma, alveolar proteinosis, infection, and neoplasia). The importance of the test early in the evaluation of undiagnosed diffuse alveolar diseases is considered. Finally we discuss the possibility that urea marks the recovered lining fluid volume and thus serves as a standard denominator. Results from our laboratory indicate that it does not. We feel that lavage is clinically useful and should be employed in the evaluation of undiagnosed diseases. Although it has provided data about the pathogenesis of chronic inflammatory diseases, its role in the longitudinal evaluation of these illnesses remains to be proven.

A 26 year-old male presented with a spontaneous pathological fracture of the right femur caused by an osteolytic lesion. A chest X-ray demonstrated the coexistence of interstitial pulmonary fibrosis. Fibrocaseous tuberculosis and widespread malignancy were ruled out by appropriate investigations. The differential diagnosis, as based on the histological examination of the bone tumour, rested between hyperparathyroidism complicated by pulmonary disease and eosinophilic granuloma with lung manifestations. Hyperparathyroidism was ruled out by the absence of clinical and biochemical evidence of a disturbance of calcium metabolism. The diagnosis of histiocytosis-X was established by the histological appearance of the lung biopsy.

One hundred cases of eosinophilic granuloma diagnosed by open lung biopsy were reviewed. There were 60 women and 40 men, ages 18 to over 60 years. Radiographs and clinical histories were contributed by physicians referring the cases for pathological consultation. Clinical follow-up information was obtained in 60 cases. The outcome was generally benign; the 16 asymptomatic patients remained well; 17 others had complete remission of symptoms, 22 had persistent symptoms, though half had partial improvement; 4 patients had progressive disease despite treatment, but only 1 patient died (of bilateral pneumothoraces complicating severe fibrosis). The more severe manifestations were found in young men, who had a higher incidence of pneumothorax, fibrosis and honeycombing, and diabetes insipidus. Many previously reported cases have had a less favorable outcome; milder cases of primary pulmonary histiocytosis have probably been overlooked in the past. In this series the prognostic value of histologic, clinical, or radiologic findings was limited. The diagnosis can often be strongly suggested radiologically, though infectious granulomata must be excluded; it is arguable whether exclusion of sarcoidosis is clinically important. The effectiveness of treatment with adrenal corticosteroids could not be assessed because of lack of controls; some individuals appeared to benefit, but relapse was very unusual in any case. The etiology of EG remains obscure, but in view of the paucity of patients with a history of allergy or asthma, and the lack of eosinophilia in any case, hypersensitivity seems unlikely. Smoking was far more common among these patients (97% altogether; 80% current) than in the general population (about 35%), an unexplained finding.

Three adult patients with chronic bilateral pulmonary infiltrates due to eosinophilic granuloma are described. The diagnosis was based on histologically proven antecedent or concomitant osseous eosinophilic granuloma in all cases. Electron microscopy was performed in two cases, showing abundant Lx-bodies in both. Pulmonary tissue was obtained in the same two cases, yielding eosinophilic granulomas in one and non-specific septal infiltrates in the other. In the third case the assumption of pulmonary eosinophilic granuloma was made by clinical analogy, thus, avoiding lung biopsy. The course was characterized by lasting benefit from skeletal surgery in all patients and by refractoriness of pulmonary lesions to immunodepressants given to two patients. Progression of pulmonary disease was minimal or none during the observation period of four, three, and three years, respectively. Diffuse pulmonary infiltrates may be a manifestation of a covert osseous eosinophilic granuloma. Skeletal screening by x-ray or scintigraphy should be part of the work-up of otherwise unexplained diffuse lung lesions. It may obviate the need for lung biopsy.

Pulmonary eosinophilic granuloma diagnosed by lung biopsy is described; the case demonstrates the remission and relapse that may occur.

Histiocytosis X is a disease of unknown origin which usually affects multiple organs, including the lung. The age of onset, the clinical course and the pattern of spread allow a distinction to be made between 3 varieties: Letterer-Siwe, Hand-Schüller-Christian and eosinophilic granuloma. The latter form, in adult patients, may predominantly or solely affect the lungs. The authors reviewed clinical, radiographic and CT findings of 7 adult patients with pulmonary eosinophilic granuloma, picked out of a series of 265 cases of interstitial lung pathology, diagnosed since 1973. Typical pulmonary involvement is bilateral, symmetrical and predominates in the upper areas. Honeycomb pattern was found in 1 patient at the onset of symptoms, and in 2 cases during the follow-up, without severe reduction in pulmonary volumes. Pneumothorax was observed in 3 cases and bone lesions in 2. CT added new and important informations such as presence, size and wall thickness of "cystic" lesions. New laboratory tests and bronchoalveolar lavage demonstrated minor diagnostic usefulness than radiological findings. The authors conclude by discussing such problems as prognostic factors and differential diagnosis.

15 cases of histiocytosis X and 274 cases of histologically confirmed sarcoidosis were diagnosed during the investigation period from 1969 to 1975. Data of 12 adults with primary pulmonary histiocytosis X were evaluated in extenso. The necessary histological verification of diagnosis was only possible by open lung biopsy. Already in early stage small excavations were found by tomography in half of the cases. Eelvation of serum copper and of the index of leukocyte alkaline phosphatase was striking. In a single case antinuclear antibodies were proven. An intra patient comparison verifies corticosteroids suppressing the disease. On the occasion of a second lung biopsy in one case could be seen that after treatment no more histiocytosis-specific substrate was existing. Exacerbation and relapse during and after continuous long-term therapy were not observed. The features of histiocytosis X and sarcoidosis are set side by side in order to show differences and relations.

The authors used immunohistochemical staining for S-100 protein to search for Langerhans cells in 7 cases of pulmonary eosinophilic granuloma (EGL) and in 18 cases of other pulmonary processes (including reactive eosinophilic pleuritis, chronic interstitial pneumonias, eosinophilic pneumonia, and nonspecific scars), which can produce diagnostic confusion with EGL. Qualitatively, Langerhans cells were found in almost every disease. However, cases of active or resolving EGL showed greater than 75 such cells/10 high-power fields (hpf), often appearing as densely packed aggregates (a virtually diagnostic feature), while all other conditions, including completely scarred EGL, showed fewer than 35 Langerhans cells/10 hpf, and the cells were scattered through the parenchyma. The authors conclude the following: (1) Langerhans cells participate in many types of inflammatory process in the lung, and hence the mere presence of Langerhans cells is not diagnostic of EGL; (2) S-100 staining with quantitation of Langerhans cells is a useful adjunct in the diagnosis of active and resolving EGL.

The cases are described of two patients with pulmonary eosinophilic granuloma who presented with spontaneous pneumothorax. Prolonged air leak and recurrence of the pneumothorax after initial re-expansion of the lung occurred in both patients. In one of the patients the pneumothorax was simultaneously bilateral and almost complete.Thoracotomy, with aggressive and total pleurodesis, is recommended in the management of these patients to prevent recurrence and complications. Lung biopsy should be performed in all patients undergoing thoracotomy for recurrent pneumothorax.

The histologic and clinical features of pulmonary histiocytosis X are discussed. The majority of patients are in the third and fourth decades of life. They present with nonspecific respiratory complaints and bilateral reticulonodular infiltrates that can be seen on chest radiographs. The histologic lesions of pulmonary histiocytosis X are scattered, discrete nodules that frequently center on small airways. The initially cellular nodules are replaced progressively by fibrous tissue. The cell infiltrates in active lesions contain variable numbers of eosinophils and histiocytes resembling Langerhans cells of the epidermis (Hx cells). In most patients the disease resolves or stabilizes, leaving few, if any, significant residues. A minority of patients develop progressive pulmonary disease that is ultimately fatal. The cause of pulmonary histiocytosis X is unknown, but two recent series suggest a relationship to cigarette smoking.