The distribution and number of Leu-7 (CD57) positive cells in lung tissue from patients with pulmonary fibrosis.

Leu-7 positive lymphocytes, including natural killer cells, play an important role in the immune system's surveillance function to prevent the development of cancer. The incidence of lung cancer is significantly high in patients with end-stage pulmonary fibrosis. We hypothesized that the number of Leu-7 positive cells may be decreased in areas of severe pulmonary fibrosis. To demonstrate this, Leu-7 positive cells were immunohistochemically stained in 41 lung specimens obtained from patients with idiopathic pulmonary fibrosis and pulmonary fibrosis associated with collagen vascular disorders. The number of Leu-7 positive cells was evaluated according to the pathological findings. In pathologically normal lung, Leu-7 positive cells were mostly found within the capillaries of the septa and rarely in the alveolar space or the stroma. The number of Leu-7 positive cells was 0.69 +/- 0.15 in areas of advanced fibrosis (n = 41), 2.39 +/- 0.60 in areas that had newly developeing fibrosis (n = 41), 1.14 +/- 0.57 in bronchiolitis obliterans organizing pneumonia (n = 9), and 1.35 +/- 0.87 in diffuse alveolar damage (DAD) (n = 11). The number of Leu-7 positive cells in areas of newly developing fibrosis (2.39 +/- 0.60) was significantly higher than that in areas of established fibrosis (0.69 +/- 0.15, P &#60; 0.05). Our present study demonstrates a significant decrease in the number of Leu-7 positive cells in areas of advanced fibrosis. This evidence may partly explain the high incidence of lung cancer associated with pulmonary fibrosis.</p

A Case of Ectopic Renin-secreting Orbital Hemangiopericy-toma Associated with Juvenile Hypertension and Hypokalemia

An unusual case of orbital tumor with high renin content and severe hypertension is described. The patient was a 15-year-old girl with juvenile hypertension (200-140 mmHg) associated with right exophthalmos and hypokalemia. The patient showed extremely high levels of plasma renin activity and plasma aldosterone concentration. No difference was present in plasma renin activity from either side of the renal veins. Preoperatively, hypertension responded to treatment with spironolactone. The tumor could not be completely removed because of intracranial metastasis and infiltration, and the hyperreninemia and secondary hyperaldosteronism persisted. The renin content in the orbital tissue was 1,403-2,225 ng/angiotensin I generated/h/g wet weight of tissue. The postmortem histopathologic diagnosis was orbital hemangiopericytoma. This is the first case of extrarenal (ectopic) renin-secreting (or -producing) hemangiopericytoma of the orbital origin. Furthermore this case is worthy of note in the point of view of the presence of the extrarenal renin-angiotensin system, particularly in the brain.</p

Myofibroblasts proliferation of idiopathic and collagen vascular disorders associated nonspecific interstitial pneumonia.

Nonspecific interstitial pneumonia (NSIP) has been recognized as a separate histological classification of interstitial lung disease. Similar features are found not only in idiopathic NSIP, but also in NSIP associated with collagen vascular disorder (CVD-NSIP). We examined the clinical symptoms, laboratory findings, and prognosis of 13 cases of idiopathic NSIP and 11 cases of CVD-NSIP. Immunohistochemical staining was performed using the streptavidin/biotin/peroxidase method with anti-alpha-smooth muscle actin antibody. No differences in the distribution of clinical features, laboratory findings, and prognosis were observed between idiopathic NSIP and CVD-NSIP. In immunohistochemical staining of the fibrosing areas, myofibroblasts were observed in 7 of 13 idiopathic NSIP cases, but in 10 of 11 CVD-NSIP cases. With regards to intra-alveolar organization, myofibroblasts were observed in all 10 CVD-NSIP cases, but they were observed in only 2 of 9 idiopathic NSIP cases. We found a significantly higher myofibroblast proliferation in the intra-alveolar organization of CVD-NSIP compared to idiopathic NSIP. Clinically, idiopathic NSIP and CVD-NSIP are similar, but are pathologically different.</p

A study on intraalveolar exudates in acute mycoplasma pneumoniae infection.

Pathologic features of Mycoplasma pneumoniae infection (M. pneumonia) are generally non-specific, and the literature regarding the pathologic features of M. pneumonia with intraalveolar exudates is limited. Clinical and histopathological studies were performed in 3 patients with M. pneumonia which did not respond to erythromycin and minocycline, but all rapidly recovered after corticosteroid therapy. In pathologic findings, we observed intraalveolar exudates and focal organization in M. pneumonia, and its intraalveolar lesions were compared between M. pneumonia and bronchiolitis obliterans organizing pneumonia containing fibrin (BOOP). Immunohistochemical studies were performed using the streptavidin biotin peroxidase complex method with anti-alpha-smooth muscle actin antibody and anti-pancytokeratin AE1/AE3 antibody. In pathologic findings, more fibrin deposits in intaalveolar lesions were observed in M. pneumonia than in BOOP. In intaalveolar lesions of M. pneumonia, a larger amount of nuclear debris, more neutrophils, and more erythrocytes were noted. Myofibroblasts were observed in the organization of BOOP, while in the intaalveolar lesions of M. pneumonia, myofibroblasts were not observed. These results suggest that M. pneumonia with intraalveolar exudates responds well to corticosteroid and its intraalveolar lesions apparently differed from those in BOOP.</p

Studies on alternative pathway in systemic lupus erythematosus -Using the chemotaxis assay- Part 2. Clinical studies

The relationship between the classical complement pathway and the alternative complement pathway was studied in 30 SLE patients. The activities of both pathways were measured by chemotaxis assay, and the following results were obtained. 1) The classical pathway-derived chemotactic index (CCI) was correlated with the level of CH50, C4 and C3, but not with those of C5. 2) The alternative pathway-derived chemotactic index (ACI) was correlated with the level of CH50, but not with those of C4, C3 and C5. 3) CCI was positively correlated with ACI. Both indices were low but CCI was more depressed than ACI. These findings suggest that the alternative complement pathway is activated mainly by the C3b positive feedback mechanism which is probably triggered via the classical complement pathway, and that the alternative complement pathway probably play a part in defence mechanism instead of reduced classical complement pathway in SLE

Studies on alternative pathway in systemic lupus erythematosus -Using the chemotaxis assay- Part 1. Fundamental studies of chemotaxis assay

To measure the activities of classical or alternative complement pathway using the modified Boyden's chemotactic chamber (each compartment 2ml and filter, pore size 3μ), suitable conditions were examined and the following results were obtained. 1) The upper compartment of the chamber contained 4×10(6) polymorphonuclear cells and 10% heat inactivated human serum in medium TC-199. 2) The lower compartment contained 10% fresh human serum as a complement source in medium TC-199. 3) To generate the classical complement pathway-derived chemotactic factor(s), 0.84mg of heat aggregated human Fr.Ⅱ was added to the lower compartment (4.2mg/ml fresh serum). 4) To generate the alternative complement pathway-derived chemotactic factor(s), 0.2mg of zymosan was added to it (1mg/fresh serum). 5) In this assay, the chemotactic activities derived from classical or alternative complement pathway were dose-dependent upon the serum (complement) concentrations in the lower compartment