Scleredema of Buschke associated with rheumatoid arthritis and Sjögren''s syndrome

A case of scleredema of Buschke associated with rheumatoid arthritis and Sjögren's syndrome is described. The onset of the skin changes and rheumatoid arthritis was almost simultaneous and the sicca syndrome developed 4 years later.     

SYSTEMIC LUPUS ERYTHEMATOSUS: A CASE-CONTROL EPIDEMIOLOGIC STUDY IN JAPAN

Background. Systemic lupus erythematosus (SLE) is designated by the Japanese government as one of the intractable diseases and all patients, who suffer from these diseases, are registered to get financial aid for treatment. Using newly registered SLE patients, a case-control study was conducted to evaluate potential risk factors. Methods. Two-hundred and eighty-two women SLE patients, newly registered to receive financial aid for treatment, and 292 randomly selected health examination participants at public health centers (controls) were surveyed from April 1988 through March 1990. By means of a self-administered questionnaire, data concerning demographic variables, smoking and drinking habits, past medical and reproductive history, and family history were collected. Results. Based on unconditional logistic regression analysis, the risk of SLE was significantly increased for current smokers (age-adjusted odds ratio (OR) = 2.31, 95% confidence interval (CI) (1.34–3.97). Alcohol and milk intake were inversely associated with risk. Family histories of asthma and collagen diseases, including SLE, were associated with significantly elevated risk of SLE (OR = 2.07, 95% ci 1.14–3.77; OR = 5.20, 95% CI 1.08–24.95, respectively). Regarding reproductive function, women with menarche at age 15 or later had significantly higher risk than those, who started menstruating before age 12 (OR = 3.82, 95% CI 1.66–8.81 for menarche at > 15 years and OR = 2.90, 95% a 1.14–7.39 for menarche at 16y). Conclusions. Our study suggests several risk factors, including smoking, family history, and reproductive history that may increase the risk of SLE.     

Lack of Constrictive Effects of Cocaine on the Fetal Ductus Arteriosus in the Rat

Cocaine (12.5 or 25 mg/kg) was administered intraperitoneally to pregnant rats 15 min to 12 hr prior to Caesarean section on day 20 of gestation. The fetuses were immediately frozen and serially sectioned, and the diameter of their ductus arteriosus (DA) was calibrated. The DA was patent in all the fetuses examined and the DA caliber was not significantly different between the control and the cocaine-treated groups at any time interval after the cocaine dosing. It was concluded that cocaine has no constrictive effects on the fetal DA in the rat.     

Primary pulmonary choriocarcinoma in a four month old boy complicated with precocious puberty

A four month old boy with pulmonary choriocarcinoma, showing iso-sexual precocious puberty is reported. His serum human chorionic gonadotropin (HCG) was highly elevated. A round isolated tumor in the right lung, found by chest X-ray, magnetic resonance imaging and gallium scintigraphy, was removed surgically a month and a half after clinical manifestations were noticed. It was determined to be choriocarcinoma on the basis of histological findings and positive HCG on histochemical examination. Skull irradiation and chemotherapy (cisplatinum, vinblastin and bleomycin) had only a transient effect on reducing tumor size and normalizing the serum level of HCG. This case suggests that a more effective treatment regimen needs to be established.     

Protective effect of granulocyte colony-stimulating factor (G-CSF) in a granulocytopenic mouse model of Pseudomonas aeruginosa lung infection through enhanced phagocytosis and killing by alveolar macrophages through priming tumour necrosis factor-alpha (TNF-α) production

We investigated the effects of G-CSF in a granulocytopenic mouse model of Pseudomonas aeruginosa lung infection. The model was prepared by intratracheal instillation of the bacteria, while granulocytopenia was induced by intraperitoneal injection of 4.0 mg of cyclophosphamide (CPA). There was no difference in the survival rate between G-CSF-treated animals and the normal group, and the number of neutrophils in the blood and lung recovered to normal in the former group. However, the phagocytic and killing activities of neutrophils were lower in G-CSF-treated mice than in controls. Interestingly, the mortality rate increased significantly when anti-TNF-α antibody was combined with G-CSF, although it was intermediate between CPA alone and CPA–G-CSF-treated mice. However, the improved mortality was not associated with a change in the number of neutrophils in the circulation and lung. Administration of anti-TNF-α antibody resulted in a significant suppression of TNF-α in bronchoalveolar lavage fluid and of enhanced alveolar macrophage function (phagocytic and bactericidal activity) against P. aeruginosa in G-CSF-treated granulocytopenic mice. We showed also increased TNF-α mRNA expression and TNF-α production in vitro using G-CSF-pretreated alveolar macrophages compared with control untreated macrophages. Our results are the first evidence to suggest that G-CSF provides a synergistic protective effect against lethal P. aeruginosa lung infection in the granulocytopenic host. This effect is probably due to enhancement of alveolar macrophage function through endogenous TNF-α production, in addition to increasing the number of circulating neutrophils.     

Successful treatment with topical mesalazine for a patient with intractable crohn’s colitis

A 32‐year‐old woman was admitted to our hospital for intractable Crohn’s disease. Crohn’s colitis was diagnosed at another hospital in August 1999. She had been successfully treated with oral mesalazine and an elemental diet. In March 2000, she had been admitted to the previous hospital for her third flare up of symptoms. She responded to oral mesalazine administration and total parenteral nutrition, but bowel symptoms were exacerbated and C‐reactive protein level was elevated after a polymeric diet was started. Prednisolone 30 mg/day was started in addition to total parenteral nutrition, but hematochezia and elevated serum C‐reactive protein level persisted, and she was referred to our hospital. She had about four bowel movements per day with fresh bleeding at the time of admission. Total parenteral nutrition and oral mesalazine were continued. The dose of oral prednisolone was increased from 15 mg/day to 40 mg/day. However, the frequency of bowel movements did not decrease and C‐reactive protein level continued to exceed 1.5 mg/day. Mesalazine enemas were begun on 23 January 2001, and were effective in improving hematochezia and serum C‐reactive protein. Both radiologic and endoscopic examinations revealed remarkable improvement.     

Flow Cytometric Analysis of Lymphocyte Subsets in the Bronchoalveolar Lavage Fluid and Peripheral Blood of Healthy Volunteers

The lymphocyte subsets in the bronchoalveolar lavage fluid (BALF) and the peripheral blood of 25 healthy volunteers were examined by analysis with a fluorescence-activated cell sorter. Comparison of the lymphocyte subsets in the BALF with those of the peripheral blood revealed much higher values for the ratios of each Leu 3a+ (CD4), Leu 3+8-, and Leu 2+15- cells, while the ratios of Leu 1+ (CD5), Leu 2a+ (CD8), Leu 7+, Leu 8+, Leu 10+, Leu 11a+ (CD16), Leu 12+, and Leu 2+15+ cells were low in the BALF. The above results indicate that the lymphocyte subsets in the BALF from healthy individuals are mainly composed of cells with surface phenotypes of helper T cells and cytotoxic T cells with virtual absence of cells carrying suppressor T and NK cell phenotypes, and with low B cell ratio. Therefore, it is assumed that the local immune mechanism of the lung is different from that of the peripheral blood.     

Phenotypic characterization of T cells in bronchoalveolar lavage fluid (BALF) and peripheral blood of patients with diffuse panbronchiolitis; the importance of cytotoxic T cells

We investigated the contribution of T cells in diffuse panbronchiolitis (DPB) by identifying T cell subsets in BALF of 36 patients with DPB, before and after long-term treatment with macrolide antibiotics, and 16 healthy control subjects. The percentages of lymphocytes and CD3⁺γδ⁺ cells in BALF of DPB patients and control subjects were similar, but the absolute number of these cells was higher in DPB patients. Treatment resulted in a significant reduction in the absolute number of these cells. A further two-colour analysis of T cell subsets in BALF showed a significantly higher ratio and number of CD8⁺HLA-DR⁺ cells in DPB patients. Treatment resulted in a significant reduction of activated T cells. Most BALF CD8⁺ cells were CD8⁺CD11b⁻ cytotoxic T cells. The number of these cells in BALF of DPB patients (26.69 ± 5.86 × 10³/ml) was higher than the control (2.02 ± 0.38 × 10³/ml; P< 0.001), and a significant reduction was observed after treatment (7.69 ± 2.59 × 10³/ml; P< 0.01). The number of CD4⁺ cells was also higher in DPB patients than in controls, and most were CD4⁺CD29⁺ memory T cells. However, treatment did not influence the number of these cells. The number of lymphocytes, CD3⁺γδ⁺, CD8⁺CD11b⁻, CD8⁺HLA-DR⁺, and CD4⁺CD29⁺ cells was higher in patients with bacterial infection than in those without bacterial infection, and interestingly, macrolide therapy reduced the number of lymphocytes, CD3⁺γδ⁺, CD8⁺CD11b⁻ and CD8⁺HLA-DR⁺ cells, irrespective of bacterial infection. In peripheral blood, the percentage of CD8⁺HLA-DR⁺ cells was also higher in DPB patients than in healthy subjects, and significantly decreased after treatment. The percentage of CD8⁺CD11b⁻ cells in peripheral blood was similar in DPB patients and normal subjects, and treatment significantly reduced the percentage of these cells. Finally, the expression of the adhesion molecules CD11a/CD18 (α/β-chains of LFA-1) on lung CD3⁺ cells and CD49d (α-chain of VLA) on lung CD4⁺ cells was enhanced compared with that on peripheral blood in DPB patients. Our results suggest that elevation of memory T cells and activation of CD8⁺ cells, mainly cytotoxic T cells, in the airway lumen of DPB patients may contribute to chronic bronchial inflammation, possibly through up-regulation of adhesion molecules. Our findings also indicate that macrolide antibiotics may have a direct or indirect suppressive effect on cytotoxic T cells, and as such, reduce inflammation and improve clinical condition.     

Female predominance of immune response to SSA/Ro antigens and risk of neonatal lupus erythematosus

Eight-hundred and twenty-five patients attending the hospital with clinical signs and symptoms of collagen diseases were tested for anti-SSA/Ro antibody. The frequency of positivity to SSA/Ro was significantly higher in females, 11.0%, than in males, 3.2% (P less than 0.01). Five babies born to anti-SSA/Ro positive mothers all had neonatal lupus erythematosus and in one there were various cardiac abnormalities.