Hepatic flares promote rapid decline of serum hepatitis B surface antigen (HBsAg) in patients with HBsAg seroclearance: A long-term follow-up study.

AIM: Serum hepatitis B surface antigen (HBsAg) seroclearance is one of the ultimate goals of management of chronic hepatitis B. We investigated the kinetics of serum HBsAg before HBsAg seroclearance in patients with chronic hepatitis B. METHODS: We retrospectively analyzed 392 Japanese chronic hepatitis B patients who had been followed for 5 years or more between 1980 and 2000. Serum HBsAg levels were measured annually using chemiluminescent enzyme immunoassay. RESULTS: During a median follow up of 14 years, 50 patients demonstrated HBsAg seroclearance (annual incidence rate, 0.91%). Multivariate analysis with baseline characteristics revealed that HBsAg of less than 3.3 log IU/mL (hazard ratio [HR], 2.22; P = 0.008) and treatment with nucleoside/nucleotide analog (HR, 0.12; P = 0.001) were independent predictive factors for seroclearance. The median HBsAg levels at 20, 10, 5, 3 and 1 year prior to seroclearance were 3.89, 2.84, 1.84, 0.78 and -1.10 log IU/mL, respectively. The rapid decline group, comprising patients who achieved HBsAg seroclearance within 5 years after confirmed HBsAg levels of 2 log IU/mL, demonstrated: (i) high alanine aminotransferase (ALT) levels; and (ii) a low frequency of liver cirrhosis progression. A significant reduction in annual HBsAg levels was found in years marked by at least one ALT flare (ALT ≥200 IU/L) (flare [+], n = 62) than in those without (flare [-], n = 323) (0.29 vs 0.17 log IU/mL/year, P = 0.003). CONCLUSION: Hepatic flares promoted rapid declines and greater annual reductions of HBsAg levels in patients with HBsAg seroclearance.

Leukocytapheresis (LCAP) for treating refractory adult-onset Still's disease (AOSD).

This is the first report on the efficacy of leukocytapheresis (LCAP) in a patient with refractory systemic-type adult-onset Still's disease (AOSD). A 17-year-old Japanese woman with AOSD who had been treated with prednisolone and cyclosporine A presented with relapse of typical systemic AOSD, including high fever, rash, and liver dysfunction. Steroid pulse therapy (methylprednisolone 500 mg/day) was performed, which failed to stabilize the disease. Therefore, LCAP (twice/week for a total of five courses) was introduced in combination with high-dose steroids plus cyclosporin A. Elevated levels of serum ferritin and transaminases and neutrophil CD64 expression normalized after the patient's disease was successfully controlled by the induction of LCAP. In this case, elevated levels of interleukin (IL)-1ß and IL-18 were normalized after LCAP induction, suggesting that LCAP treatment modulates the deregulated cytokine-mediated inflammatory responses observed in AOSD. Our clinical observations suggest that LCAP may be beneficial for flare-up of systemic manifestations of AOSD refractory to conventional treatment, including high-dose steroids and immunosuppressants.

Successful treatment of HTLV-1-related overlap syndrome using tacrolimus.

A 56-year-old HTLV-I-positive woman, initially diagnosed as having Sjögren's syndrome, presented with muscle weakness, myalgia, face erythema and leg edema. Based on the presence of various autoantibodies, the diagnosis of overlap syndrome (dermatomyositis/Sjögren's syndrome) was made. Treatment with high-dose corticosteroid plus cyclosporine improved her symptoms. However, three months after the start of these treatments, exacerbation of myositis occurred. A muscle biopsy revealed prominent perivascular accumulation of mononuclear cells with perifascicular atrophy, which were consistent with dermatomyositis. Tacrolimus, which was substituted for cyclosporine led to marked improvement of the myositis symptoms.

Macrophagic myofascitis associated with rheumatoid arthritis.

Macrophagic myofascitis (MMF) is an unusual inflammatory myopathy characterized by muscle infiltration by macrophages and lymphocytes. Here, we describe a case of MMF which is associated with rheumatoid arthritis. A 53-year-old Japanese rheumatoid arthritis (RA) patient presented with focal tenderness of lower extremities. Magnetic resonance imaging showed evidence of myofascitis involving fascias of anterior tibialis muscle. Muscle biopsy showed a unique pathological pattern of MMF. MMF is known to be associated with vaccination containing aluminum. However, our case was not related to aluminum containing vaccinations and etiologies are unknown. The possible link needs to be discussed.

Idiopathic portal hypertension associated with systemic sclerosis and Sjögren's syndrome.

We report a patient with idiopathic portal hypertension (IPH) associated with systemic sclerosis (SSc) and Sjögren's syndrome. A 72-year-old Japanese woman was admitted to our hospital because of Raynaud's phenomenon, sclerodactyly, and dyspnea. The patient had splenomegaly, esophageal varices in the absence of extrahepatic portal obstruction, and cirrhosis of the liver. Immunological studies revealed positive anti-nuclear antibodies and high titers of anti-Scl-70, anti-SS-A, anti-centromere, and anti-mitochondrial M2 antibodies. Histological examinations of the liver biopsy specimen revealed stenosis and loss of small portal veins without findings of primary biliary cirrhosis. The patient was diagnosed as having IPH associated with SSc and Sjögren's syndrome. These observations suggest an immunological role in the pathogenesis of IPH.

Catastrophic transverse myelitis in a patient with systemic lupus erythematosus.

A 24-year-old Japanese woman was admitted to our hospital suffering from high fever and progressive paralysis in both legs. Magnetic resonance imaging of the spinal cord showed high-intensity signals from C5 to Th4 and from Th7 to L1 on T2-weighted images. The patient was diagnosed as having acute transverse myelitis trade mark, which was a complication of systemic lupus erythematosus based on the serological findings. Despite aggressive immunosuppressive treatments including corticosteroid pulse therapy, plasmapheresis, and intravenous cyclophosphamide, the paralysis of her lower extremities did not improve. In the catastrophic type of lupus-associated TM, which develops extensively and longitudinally along the spinal cord, the prognosis still seems to be poor despite intensive treatments.

Interpretation of and preference for probability expressions among Japanese patients and physicians.

BACKGROUND: Patients and physicians often communicate using qualitative probability expressions that describe expected outcomes or risks of a medical intervention without knowing whether or not they share the same understanding. OBJECTIVE: The aim of the present study was to determine interpretations of qualitative probability expressions in clinical settings by Japanese patients and physicians as well as their assessments and preferences about physicians' use of such expressions. METHODS: One hundred and sixty-eight consecutive patients aged 16 years or older, who attended a university hospital during a 2-week period in 1999, and 156 physicians recruited through the Japanese General Medicine Research Network participated in this cross-sectional survey, using a self-administered questionnaire. Participants were asked to assign numerical interpretations as a percentage to 10 qualitative expressions of probability in two clinical situations related to prescribing a medicine for a cold and an anti-cancer drug. They were also asked which type of expression, qualitative or quantitative, they usually use when communicating probabilistic clinical information and which they prefer. RESULTS: The estimates of probability expressions showed wide variations, especially among patients. Patients tended to assign lower and higher values to highly positive and negative probability expressions, respectively, than physicians. Clinical context also influenced the estimation: both groups tended to assign higher estimates in the anti-cancer drug situation than in the cold treatment situation. Factor analysis revealed three psychologically meaningful factors in each situation. More patients than physicians (64.6% versus 50.3%) thought that physicians do not use numbers in routine practice. More than 20% of both patients and physicians considered that the actual use of qualitative terms by physicians is undesirable. Nevertheless, a sizable number of patients (41.4%) and physicians (15.2%) considered it preferable that physicians do not use numbers. CONCLUSION: Since interpretation of qualitative expressions of probability in Japanese is subject to large interpersonal variability and differences between patients and physicians, as well as context dependence, the use of qualitative expressions alone might cause misunderstanding among the parties involved. However, the majority of patients prefer words to numbers at present. Therefore, physicians, at least in Japan, ought to provide patients with both numbers and words when critical decisions need to be communicated.