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A new classification of the idiopathic interstitial pneumonia has recently been proposed, defining idiopathic pulmonary fibrosis (IPF) more rigorously and in contrast to further subentities. The new classification is of prognostic and clinical relevance. Diagnosis requires a combined clinical, radiological and pathological effort. In patients with characteristic clinical and HR-CT findings, the diagnosis of IPF can be made with sufficient confidence without surgical lung biopsy. Recent evidence suggests that the primary pathogenetic event in IPF involves epithelial injury and abnormal wound heeling. This explains the ineffectiveness of the usual anti-inflammatory therapy in the majority of patients. Since no antifibrotic drugs are available at present, the recommended standard therapy is a combination of prednisone with azathioprine or cyclophosphamide. New antifibrotic molecules are already tested in clinical trials.

History and Admission Findings: A 36-year-old Korean had since childhood suffered from a chronic progressive lung disease, marked by dyspnoea, productive cough and recurrent sinusitis. He was in a reduced general condition on admission. There were hyperresonant sounds on palpation over both lungs, a low diaphragm and slight vesicular breath sound as well as occasional rales and sibilant rhonchi., Investigations: Laboratory tests showed increased blood cell sedimentation of 52 mm at 1 h, leukocytosis of 24.3/nl, and a raised cold-agglutinin titre. Haemophilus influenza and Pseudomonas aerogenes were cultured from the bronchial secretion. Lung function tests revealed severe obstruction with hyperinflation and partial respiratory failure with a much reduced 1-sec forced expiratory volume (FEV1) of 0.71, 19% of normal; residual volume (RV) 6.61, 550% of normal; arterial oxygen partial pressure 61 mm Hg; arterial CO2 partial pressure 36 mm Hg. Chest X-ray showed emphysematous changes with diffuse small nodular shadows. Bronchoalveolar lavage revealed 96% neutrophils, indicating marked granulocytic inflammation. A video-assisted thoracoscopic biopsy was performed which showed diffuse panbronchiolitis., Treatment and Course: The symptoms, radiological changes and lung functions clearly improved under long-term treatment with 500 mg erythromycin daily., Conclusion: Diffuse bronchiolitis is rarely seen outside of Asia, but it should be included in the differential diagnosis because, in contrast to other forms of chronic obstructive lung disease, it responds to long-term erythromycin medication.

In 7 patients with pulmonary alveolar proteinosis, differential cytology and lymphocyte subsets in BAL fluid were investigated. The study showed that pulmonary alveolar proteinosis is another disorder characterized by a lymphocytic alveolitis and activation of T-lymphocytes (expression of HLA-DR antigens and IL-2 receptors). Our data indicate that immunological mechanisms involving T-cell activation may contribute to be pathogenesis of pulmonary alveolar proteinosis.

In pleural effusions, histologically proven to be malignant in 13 patients with small cell lung cancer, the immunocytological profile of tumour cells, together with the distribution of the lymphocyte subpopulations, were established. In all cases, tumor cells were demonstrable with the monoclonal antibodies EMA, HEA-125, Sam 2, 6, 8 and 10. In contrast, conventional cytology was positive in only 6 of the 13 cases. As in other malignant or inflammatory effusions, phenotyping of the lymphocytes revealed an elevated T4/T8 ratio in comparison with the normal value for peripheral blood.

In the present paper, the results of cytological and immunocytological investigations of the broncho-alveolar lavage fluid obtained from 13 patients with pulmonary haemorrhage were studied for their differential-diagnostic usefulness. Five patients each were suffering from idiopathic pulmonary haemosiderosis (IPH) and the Goodpasture syndrome (GPS), one patient had a Wegener's granulomatosis, and two patients presented with vasculitis (VA) which was not amenable to further histological characterization. Parameters that proved particularly suitable for differentiation were the total cell count, the number of haemosiderin-positive macrophages, the extent of the expression of the transferrin receptor (OKT 9) on the macrophages, and the relationship of OKT 4 and OKT 8-positive lymphocytes.

Two patients with complicated acute pancreatitis are demonstrated. Sonographic factors are compared to laboratory and clinical findings. Ultrasound can add relevant information for classification and thereby supply an indication for surgery in patients with acute pancreatitis. This is especially true since laboratory classification of the grade of the disease is as yet not generally accepted.

Two cases of the Churg-Strauss syndrome are reported, seen within the past year. The first concerned a 31-year-old woman with a rapidly progressive illness characterized by bronchial asthma, leukocytosis and eosinophilia, abdominal pain, diarrhoea and purpura. In the second case, a 47-year-old man had granulomatous epididymitis and interstitial nephritis before the asthma and eosinophilia developed. In both the diagnosis was confirmed by lung biopsy. Immunosuppressive treatment achieved rapid regression of all symptoms and of the eosinophilia, without recurrence so far.

Fibrotic alveolitis appeared in a 54-year-old patient undergoing gold therapy. In differential diagnosis, this infrequent side effect of gold therapy is to be distinguished from rheumatoid lung fibrosis. Lymphocyte sub-populations were determined in the broncho-alveolar lavage fluid of the patient. The results obtained support the view that the side effect is due to an immunologically mediated process.