[A case of systemic scleroderma complicating pulmonary hypertension].

The patient was a 7-year-old girl. At the age of 6, deposits of pigment had appeared on the skin of her face and limbs, the skin had become sclerosed, and she had developed dyspnea on exertion. Her previous physician had hospitalized her.

Meta-analysis of the results of intravenous gamma globulin treatment of coronary artery lesions in Kawasaki disease.

The objective of this study was to evaluate the efficacy of intravenous gamma globulin (IVGG) therapy on the prevention of coronary artery lesions (CALs) in patients with Kawasaki disease (KD), with reference to the literature on meta-analyses in randomized controlled studies. Studies from 1984 to the end of 2000 obtained from the National Library of Medicine or from the bibliographies of these articles were used in the analysis. The total number of patients with KD covered in 17 articles was 4020.

Efficacy of plasma exchange therapy for Kawasaki disease intractable to intravenous gamma-globulin.

Kawasaki disease (KD) causes coronary artery lesions (CALs) in 500 Japanese children each year. Intravenous gamma-globulin (IVGG) decreases the incidence of these lesions from 25% to 8% of the total KD cases. We examined whether plasma exchange is a safe and effective prophylaxis against CALs in children with KD intractable to IVGG therapy.

Recombinant adeno-associated virus preferentially transduces human, compared to mouse, synovium: implications for arthritis therapy.

Despite a number of published reports, including from our own laboratory, suggesting that adeno-associated virus (AAV) transduces mouse synovium, a careful analysis demonstrated transduction predominantly of the subsynovial muscle tissue, while the synovial lining is poorly transduced. To investigate the potential of AAV to transduce human synovium, three human rheumatoid arthritis (RA) and two murine collagen-induced arthritis (CIA) synovial cell lines were infected with recombinant AAV (rAAV) vectors encoding either mouse IL-10 or IL-4. Low-level transgene expression was observed.

Inflammatory cytokines and systemic-onset juvenile idiopathic arthritis.

Systemic-onset juvenile idiopathic arthritis (JIA) is a severe and steroid-dependent disease, which sometimes progresses to the fatal disease macrophage activation syndrome. An investigation of inflammatory cytokine levels revealed increases in IL-6 in serum of systemic-onset disease patients. Continuously elevated levels of IL-6 in serum may play a important role in manifesting the clinical symptoms and signs of systemic-onset JIA, including spiking fever, rash, arthritis, and serositis.

[A case report of childhood systemic lupus erythematosus complicated with lupus cystitis].

The patient was a 13-year-old girl. In August 2000, she presented with a fever, together with diarrhea, vomiting, arthralgia, nasal bleeding and malaise, and was examined by another physician. Because her platelet count was low, and there were positive reactions for anti-nuclear antibodies, anti-DNA antibodies and platelet-associated IgG, idiopathic thrombopenic purpura, and systemic lupus erythematosus (SLE) was suspected.

[Usefulness of interferon-gamma-based diagnosis of Mycobacterium tuberculosis infection in childhood tuberculosis].

We studied the usefulness of interferon-gamma measurement reagent QuantiFERON-TB 2 G (QFT-2G), used to diagnose tubercle bacilli infections, as an indicator both for diagnosing primary tuberculosis (PTB) and for assessing therapeutic amorg pediatric Tuberculosis Outpatent cases effectiveness. Five cases showing typical PTB findings, such as cavitation, swollen lymph nodes, and nodular shadows at the pulmonary hilum, and diagnosed as tubercle bacillus infections, all showed positive reactions to QFT-2G, and in 3 asymptomatic cases without abnormalities detected in diagnostic imaging but QFT-2G-positive, one developed tuberculosis (TB) later. Among 12 patients who gave negative reactions to QFT-2G at their first visit and during observation from 6 months to 1 year, no TB occurrences was seen.

[Three cases of childhood-onset male systemic lupus erythematosus (SLE) successfully treated with a combination of pulse methylprednisolone and pulse cyclophosphamide].

We reported three cases of childhood-onset male systemic lupus erythematosus (SLE), all of whom successfully treated with a combination of pulse methylprednisolone (mPSL) and pulse cyclophosphamide (IVCY). All of them had severe lupus nephritis and were complicated with other collagen diseases. Two cases were complicated with Sjögren syndrome (SS) and the other was complicated with both SS and anti-phospholipid syndrome (APS).

Clinical study of tocilizumab in children with systemic-onset juvenile idiopathic arthritis.

Systemic-onset juvenile idiopathic arthritis (sJIA) is a severe and steroid-dependent disease of unknown etiology that sometimes progresses to a fatal disease known as the macrophage activation syndrome. The investigation of inflammatory cytokines and receptor levels revealed an increase in interleukin (IL)-6 and soluble IL-6 receptor (sIL-6R) in serum of patients with active sJIA. The clinical symptoms and signs of the disease are presumably attributable to the continuous elevation of IL-6 and sIL-6R levels in serum.

[A case of autoimmune hepatitis needed to be differentiated from EBV hepatitis, in that the histology of liver biopsy specimen was useful for diagnosis].

A 10-year-old girl with autoimmune hepatitis (AIH) was reported. She was admitted to our hospital because of cholestasis and elevation of liver enzymes for 2 months. Laboratory examination revealed that EBV-DNA copy number in the PBMNC (peripheral mononuclear cells) was 1.

Proteasome inhibition enhances AAV-mediated transgene expression in human synoviocytes in vitro and in vivo.

To explore the potential applicability of recombinant adeno-associated virus (rAAV) vectors in the treatment of rheumatoid arthritis (RA), primary human fibroblast-like synoviocytes (FLS) derived from patients with RA were infected with rAAV encoding mouse IL-10 under the control of the CMV promoter. Addition of the proteasome inhibitor carbobenzoxy-l-leucyl-l-leucyl-l-leucinal (zLLL) to the cultures dramatically enhanced expression of the IL-10 transgene, in a dose-dependent manner. The increased expression was transient, peaking at 3 days and returning to near baseline by 7 days.

Therapeutic efficacy of humanized recombinant anti-interleukin-6 receptor antibody in children with systemic-onset juvenile idiopathic arthritis.

Objective: To investigate the safety and efficacy of a recombinant human anti-interleukin-6 (anti-IL-6) receptor monoclonal antibody (MRA) that indirectly inhibits the effects of IL-6 in children with systemic-onset juvenile idiopathic arthritis (JIA) refractory to high-dose, long-term corticosteroids.

Methods: An individual escalating-dose trial was conducted in 11 children with active systemic-onset JIA who met the inclusion criteria. All were first administered an intravenous dose of 2 mg/kg MRA.

[Cyclophosphamide pulse therapy for pediatric systemic sclerosis].

We encountered three patients with pediatric systemic sclerosis. Patient 1 had systemic scleroderma, pigmentation and interstitial pneumonia at the age of 10 years. Nine months after disease onset, she was treated with intravenous cyclophosphamide pulse therapy as induction therapy.

[Effects of therapies on childhood systemic lupus erythematosus].

We analyzed the effects of three therapies on 30 patients with childhood systemic lupus erythematosus, and classified the patients into three groups. The therapies were as follows; Group A (8 cases), methylpredni-solone (mPSL) pulses plus oral prednisolone (PSL) alone, Group B (10 cases), mPSL pulses plus oral PSL and mizoribine (MZB) or azathioprine (AZP), Group C (12 cases), mPSL pulses and intravenous cyclophosphamide (IVCY) pulse therapy plus oral PSL and MZB or AZP. Three years after treatment, we compared the laboratory data (C3, C4, CH50 and anti-DNA antibody), the SLEDAI scores and numbers of relapses in these three groups.

Long-term remission in three patients with childhood-onset systemic lupus erythematosus.

Abstract Three cases of childhood-onset systemic lupus erythematosus (childhood SLE) with long-term remission are reported. These cases were not complicated with collagen diseases and had no SLE disease activity index scores either 3 or 5 years after onset. We suggest that some patients with childhood SLE may be able to abandon the maintenance therapy, and that careful observation is needed for each individual case.

[A case of classical polyarteritis nodosa diagnosed by myocardial biopsy].

We experienced a girl with polyarteritis nodosa (PN) diagnosed by myocardial biopsy. The symptoms began with high fever and skin rash. These symptoms and laboratory findings temporarily improved by oral prednisolone, however, she flared up with chest pain about 40 days after onset of the disease.

Gene transfer of a fibronectin peptide inhibits leukocyte recruitment and suppresses inflammation in mouse collagen-induced arthritis.

Objective: Cell adhesion plays an essential role in arthritis by recruiting and retaining leukocytes in the joint. Fibronectin, a major extracellular matrix component in synovium, plays a central role in cell-cell and cell-matrix interactions through ligation of cell surface integrins. The present study was designed to determine the effects of gene transfer of a 15-amino acid peptide derived from the 33-kd carboxy-terminal cell and heparin-binding domain of fibronectin (FN-C/H-II) on established arthritis in mice.