A study of ten Japanese patients with seronegative spondylarthropathy: a tentative proposal

We reviewed ten patients with seronegative spondylarthropathy (SNSA), who all fulfilled the European Spondylarthropathy Study Group criteria for spondylarthropathy (SpA); seven patients also met the Amor criteria for SpA. Seronegative spondylarthropathy was not a uniform syndrome but rather a wide spectrum of complex disease with characteristics of sacroiliitis and enthesopathy. The most frequent symptom at diagnosis of SNSA was inflammatory low back pain, followed by asymmetric oligoarthralgia and Achilles tendonitis and/or plantar fasciitis. Systemic complications were revealed as eye and skin involvement. Imaging methods including pelvic radiography, scintigraphy, and computed tomography scanning were useful in detecting spondylarthropathic changes, which were characteristic of SNSA. Human leukocyte antigen (HLA) typing showed various patterns among patients, in which HLA-B27 was positive in three patients with ankylosing spondylitis. HLA-B51, which is a well-known genetic factor associated with Behçet's disease (BD), was positive in two patients who were apparently distinct from BD. Two patients with palmoplantar pustulosis showed symptoms and signs characteristic of SNSA. Although we have few SNSA patients in the present study, we would like to propose that HLA-B51 positive SpA would be considered as a subset of SNSA, and that pustulotic SpA also would be classified as a member of SNSA. This led us to suggest the possibility to change the concept of SNSA proposed by Moll et al. The optimal treatment remains to be defined, but sulfasalazine was effectively used with almost all patients in combination with nonsteroidal anti-inflammatory drugs.     

Analysis of Risk Factors for Hepatocellular Carcinoma in Patients With HBs Antigen- and Anti-HCV Antibody-Negative Alcoholic Cirrhosis: Clinical Significance of Prior Hepatitis B Virus Infection

Background: The hepatitis B virus (HBV) or hepatitis C virus (HCV) markers frequently are detected in alcoholic patients with hepatocellular carcinoma (HCC). However, risk factors for the development of HCC in patients with HBs antigen (Ag)- and anti-HCV antibody (anti-HCV)-negative alcoholic cirrhosis have not been clearly documented. The present study was conducted to elucidate the occurrence rates of HCC in HBs Ag- and anti-HCV-negative male alcoholic cirrhosis and to assess the risk factors for hepatocellular carcinogenesis.
Method: We prospectively studied 91 consecutive patients with HBs Ag- and anti-HCV-negative alcoholic cirrhosis for 0.5 to 12.5 years (median 5.9 years). Potential risk factors assessed for liver carcinogenesis included the following six variables: age, total alcohol intake, association of continuing alcohol intake after diagnosis, indocyanine green retention rate at 15 min, anti-HB core antibodies (anti-HBc), and association of diabetes mellitus.
Results: Cumulative occurrence rates of HCC were 6.4%, 18.9%, and 28.7% at the end of the 5th, 7th and 10th years, respectively. When classified by anti-HBc, the occurrence rates of HCC in 31 patients with anti-HBc and 60 patients without anti-HBc were 15.6% and 2.9% at the 5th year, 28.4% and 13.5% at the 7th year, and 40.4% and 22.1% at the 10th year, respectively. The occurrence rates of HCC were also significantly related to the cumulative alcohol intake. Cox proportional hazard model identified that cumulative alcohol intake ( p = 0.0047) and positive anti-HBc antibodies ( p = 0.0598) were independently associated with the occurrence rates of HCC.
Conclusion: These epidemiologic results suggest that heavy cumulative alcohol intake and prior exposure to HBV infection are risk factors for the development of HCC in patients with HBs Ag- and anti-HCV-negative alcoholic cirrhosis.     

The methodological aspects of off-line sampling of exhaled air for nitric oxide measurement in Japanese]

The measurement of exhaled nitric oxide (eNO) is a non-invasive biomarker of bronchial inflammation. Despite the usefulness of eNO measurement, NO analyzers are too expensive for widespread use by general practitioners. In comparison, the off-line (bag collection) method of eNO measurement may be more useful. In Japan, however, there have been few studies about eNO in asthmatics using the off-line method. This study shows methodological aspects of the off-line method. Briefly, with a SIEVERS bag collection kit, we recommend that the flow rate and pressure level of exhaled air should be 70 ml/sec and 10 cm H2O, respectively and that the sampled air should be measured within 12 hours.     

A case of small cell lung cancer with an initial symptom of breast metastasis]

We report a case of small cell lung cancer with an initial symptom of breast metastasis. A 55-year-old woman was admitted complaining of multiple breast masses. Chest computed tomography (CT) and magnetic resonance imaging (MRI) showed a left hilar mass. Specimens obtained from a breast mass and transbronchial biopsy revealed neoplastic cells suggesting small cell carcinoma Small cell lung cancer with breast metastasis was diagnosed. Systemic chemotherapy resulted in partial remission of the primary lesion and breast metastases.     

Gastroduodenal Lesions after Transcatheter Arterial Chemo-embolization in Patients with Hepatocellular Carcinoma

We investigated the incidence and endoscopic features of gastroduodenal lesions which appeared after transcatheter arterial chemo-embolization (TACE), performed 29 times in 25 patients with inoperative hepatocellular carcinoma. The new development or exacerbation of the gastroduodenal lesions after TACE was evident in 13 of the 29 (45%). The side of the lesions ranged from the gastric body to the second portion of the duodenum, and the lesions that developed were multiple ulcers in four, and multiple erosions with white coat in nine. The development of these gastroduodenal lesions may be due to mucosal ischemia caused by embolic materials, the toxic effect of antineoplastic drugs infused, or to stress. In light of these events, upper gastrointestinal endoscopy should be added to the usual examinations done for patients undergoing TACE.     

Clinical and laboratory features of scleroderma patients developing skeletal myopathy

Skeletal muscle involvement, or myopathy, has been a recognized feature of systemic sclerosis (SSc). We studied retrospectively 302 Japanese patients with SSc to elucidate the clinical and laboratory features in scleroderma patients developing skeletal myopathy during their clinical course. Forty-three patients (14%) developed skeletal myopathy during their course of the disease. The mean age of the patients who developed skeletal myopathy was significantly lower than that of those who did not. The ratio of male to female was significantly higher in the myopathic patients. The patients with diffuse cutaneous SSc were more likely to develop myopathy than those with limited cutaneous SSc. The prevalences of heart involvement, pulmonary fibrosis, diffuse pigmentation of the skin, and contracture of phalanges were significantly greater in those with skeletal myopathy than in those without. None of the patients with skeletal myopathy had anticentromere antibody. These findings suggested that the SSc patients with severe internal organ involvement, such as pulmonary fibrosis and heart disease, and some other complications were prone to develop skeletal myopathy during their clinical course of the disease.     

Cyclosporin treatment in steroid-resistant and acutely exacerbated interstitial pneumonia

OBJECTIVE: The aim of this study was to evaluate the efficacy of cyclosporin A (CsA) in patients with interstitial pneumonia (IP). DESIGN: Retrospective comparative study. PATIENTS: We reviewed 33 patients (23 males and 10 females with a mean age of 62.5 years) with histologically-proven progressive IP who were treated with CsA. All patients had corticosteroid-resistant IP or developed acute exacerbation of IP in their courses. RESULTS: The underlying systemic diseases were: idiopathic interstitial pneumonias (IIPs) in 19 patients, and collagen vascular diseases (CVDs) in 14. The histopathological patterns and underlying diseases of IP were classified as usual interstitial pneumonia (UIP)/idiopathic pulmonary fibrosis (IPF) in 10 patients, cellular-nonspecific interstitial pneumonia (NSIP)/IIPs in 3, fibrotic-NSIP/IIPs in 5, organizing pneumonia (OP)/IIP in 1, UIP/CVDs in 4, cellular-NSIP/CVDs in 7, fibrotic-NSIP/CVDs in 2, and diffuse alveolar damage (DAD)/CVD in 1, respectively. They received a low dosage of CsA combined with corticosteroids. The prognoses after treatment with CsA were well correlated with histopathological patterns. Cellular-NSIP and OP showed better prognoses than fibrotic-NSIP, UIP or DAD. In addition, CVDs had better prognoses than IIPs, when compared on the basis of the same histopathological patterns. Furthermore, the prognoses in the CsA-treated group were significantly better than in those without CsA treatment in regard to acute exacerbation of UIP/IPF. CONCLUSIONS: CsA combined with corticosteroids may be an efficacious treatment for corticosteroid-resistant IP and for acute exacerbation of IPF.     

Thymic carcinoma. A clinicopathological study of seven patients]

The treatment of seven cases of thymic carcinoma is reported. According to the Masaoka classification of the disease, there were five cases of stage III and two of stage IVb. Five were identified histopathologically as squamous cell carcinoma, one as undifferentiated carcinoma, and one as small cell neuroendocrine carcinoma. Surgery was performed during the course of chemotherapy and radiotherapy in 5 cases, and in 2, the organs infiltrated by neoplastic cells were partially excised together. Radiotherapy was performed as adjuvant therapy in one case of partial excision. In another case, after six years of chemotherapy and radiotherapy that yielded a partial response (PR), cancerous infiltration of the chest wall occurred, and partial removal of the chest wall became necessary. After the remaining case showed a PR to chemotherapy, complete macroscopic excision of the tumor was undertaken, but mediastinal lymph gland metastasis was present, and so radiotherapy was also initiated. In two cases, surgery was not performed. One of these was treated with chemotherapy alone, the other, only with radiotherapy. The mean survival period of the five patients who died as a result of their tumors was 44.8 months. One of the two who survived for over four years had squamous cell carcinoma, the other, undifferentiated carcinoma. The case of small cell neuroendocrine carcinoma was assessed as having a PR, and so complete extraction of the thymic neoplasm was carried out, and followed with radiotherapy. This patient is still alive.     

Steroid‐free living donor liver transplantation for HCV – a multicenter prospective cohort study in Japan

This prospective, non‐randomized, multicenter cohort study analyzed the safety and efficacy of a steroid‐free immunosuppressive (IS) protocol for hepatitis C virus (HCV)‐positive living donor liver transplant (LDLT) recipients in Japan. Of 68 patients enrolled from 13 transplant centers, 56 fulfilled the inclusion/exclusion criteria; 27 were assigned the steroid‐free IS protocol (Fr group) and 29 the traditional steroid‐containing IS protocol (St group). Serum HCV RNA levels increased over time and were higher in the St group until postoperative day 90 (POD 14, p = 0.013). Preemptive anti‐HCV therapy was started in a higher percentage of recipients (59.3%) in the Fr group than in the St group (31.0%, p = 0.031), mainly due to early HCV recurrence. The incidence of HCV recurrence at one yr was lower in the Fr group (22.2%) than in the St group (41.4%; p = 0.066). The incidence of acute cellular rejection was similar between groups. New onset diabetes after transplant, cytomegalovirus infection, and renal dysfunction were significantly less frequent in the Fr group than in the St group (p = 0.022, p < 0.0001, p = 0.012, respectively). The steroid‐free IS protocol safely reduced postoperative morbidity and effectively suppressed both the HCV viral load in the early post‐transplant period and HCV recurrence in HCV‐positive LDLT recipients.