In addition to population-growth in the elderly, development of new therapeutic agents for rheumatoid arthritis (RA) contributes to an increase in the number of elderly patients with RA. It is also reported that the age of RA onset is going higher. Elderly-onset RA (EORA) is defined as RA developing after the age of 60 years. In RA patients, significant damage can be detected radiographically within the first 2 years after the initial presentation of symptoms; therefore, appropriate treatment should be administered at the earliest. Meanwhile, caution should be exercised in prescribing disease-modifying antirheumatic drugs (DMARDs) to elderly patients because the associated risk of adverse effects and toxicity is elevated in the elderly. However, excessive caution may prevent elderly patients from being implemented the ideal therapy. Compared to young patients with RA, patients with EORA are less frequently treated with biological agents or multiple DMARDs treatment and more frequently treated with prednisone. Some patients with EORA do not receive optimal treatment during the early stage of RA, when the disease is highly active and joint destruction rapidly progresses. EORA should be treated with appropriate DMARDs instead of corticosteroids in order to maintain the risk of infection minimal and the patients' physical function maximal.

View full abstract

  Sjögren's syndrome (SS) is a chronic autoimmune disease characterized by a progressive lymphocytic infiltration of the exocrine glands with varying degrees of systemic involvement. Chronic inflammation compromised the glands' function and leads to dry symptoms in the mouth/eyes. SS has been thought to be rare in children. Recent epidemiological study revealed, however, that the incident of childhood SS (cSS) per 100,000 children was more than 0.5. This indicate cSS is common disease after SLE in children with rheumatic diseases. Sicca symptoms are rare in cSS even though oral and ocular involvement are present. This may be from the slowly progression feature of the disease course. Two follow-up studies indicate that there were almost no changes in clinical symptoms and autoantibody profiles during the 10 years of follow-up. However, auto-antibody profiles of cSS including alpha-fodrin-antibody, specifically found in adult SS, are the same as that of adult SS. These data indicate that no essential difference exist in SS between adult and childhood patients, and also indicate the specific features seen in cCSS just reflect early stage of SS. In order to establish the diagnostic criteria for cSS in the feature, early pathophysiologic features should be included in the criteria.

View full abstract

  Hyperimmunoglobulin-E syndrome (HIES) is one of the primary immunodeficiency with the manifestations of recurrent infections especially with Staphylococcus aureus, characteristic facies, hyperextensibility of joints, multiple bone fractures, scoliosis, and delayed shedding of the primary teeth. It is a multisystem disease of autosomal dominant or recessive inheritance. Recently, the genetic causes of HIES (STAT3, TYK2, and DOCK8) were clarified.

View full abstract

  A case of severe autoimmune hemolytic anemia after ABO matched living donor liver transplantation.
  We present here a case of severe autoimmune hemolytic anemia after ABO matched living donor liver transplantation. The patient is 56 y.o male. He received living donor liver transplantation from his ABO matched son in May 2007. In July, he was suffered from a progressive anemia, and diagnosed as autoimmune hemolytic anemia by the laboratory examinations. Intensive treatment including predonisolone, azathiopurine, rituximab, plasma exchange, was given, however, the disease was resistant to the treatment. By the administration of cyclophosphamide combined with rituximab, remission was finally achieved. To date, immune mediated hemolytic anemia after ABO matched living donor liver transplantation has not been reported, although several cases of ABO mismatched living donor liver transplantation have been reported. His severe but transient clinical course of anemia suggests that the transient emergence of donor lymphocytes may be responsible for onset of hemolytic anemia.

View full abstract

  A 50-year-old woman was emergently admitted because of rapidly progressive unconsciousness, renal failure, hepatic dysfunction, hemolytic anemia, thrombocytopenia, and high-grade fever in July, 2008. Based on clinical and laboratory findings, we made a tentative diagnosis of thrombotic thrombocytopenic purpura (TTP) and immediately initiated the plasma exchange (PE). Despite the PE, she developed panperitonitis due to multiple intestinal perforation and massive splenic infarction within a week after the admission. Thrombosis of arterioles at perforated portion in the resected small and large intestines was histologically confirmed. Therefore, we made a definite diagnosis of catastrophic antiphospholipid syndrome (CAPS) based on the presence of antiphosphatidylserine-prothrombin complex antibodies (aPS/PT) throughout the course and lupus anticoagulant that was revealed positive on one occasion, and multiple thrombotic lesions. The underlying disease of CAPS appeared to be lupus erythematosus because of her clinical history and laboratory findings such as persistent leukopenia. Although it has been reported that CAPS causes systemic thrombosis at microvessels mostly within a week from the onset and the mortality rate in this disorder is as high as 50%, we successfully treated her in combination with high-dose corticosteroid, anticoagulation, concentrated human IgG, surgical procedures, and hemodialysis in addition to PE. Early diagnosis of CAPS and immediate start of PE may have contributed to the successful treatment.

View full abstract

  The patient was a 57-year-old male, who developed bloody stool around May 2006. He was examined by another physician in the department of gastroenterology in our hospital. Gastrointestinal (GI) endoscopy showed a duodenal ulcer, and the biopsy specimen revealed angiitis of the duodenum. He was admitted to our hospital on June 2006. Serum level of creatinine (Cr) was rapidly increased with hematuria and proteinuria. The titer of MPO-ANCA was elevated, and he was diagnosed with microscopic polyangiitis. Steroid pulse therapy was initiated, followed by the administration of prednisolone (PSL : 1 mg/kg/day) and intravenous cyclophosphamide (IVCY). Serum Cr was gradually decreased, but bloody stool was observed from the 10th hospital day. GI endoscopy showed bleeding from the duodenal ulcer. Steroid pulse therapy was performed, and the dose of PSL was increased to 1.5 mg/kg. Endoscopic hemostatic therapy was repeatedly performed without clinical improvement. Pancreatoduodenectomy was performed on the 15th hospital day.
  However, bleeding from the small intestine was observed repeatedly and the computed tomography of the chest showed cavity-forming nodules, which were diagnosed with angiitis by the biopsy specimen. The additional treatments of steroid pulse therapy, intravenous immunoglobulin therapy, IVCY and Rituximab did not result in favorable response. We report a refractory case of ANCA-related angiitis presented with gastrointestinal ulcer, rapidly progressive nephritis and multiple lung nodules.

View full abstract

  Treatment with corticosteroid and etanercept was started for a 69-year-old woman with rheumatoid arthritis. Eight weeks later, she developed fever and anorexia, and was admitted to our hospital with a poor general condition. With the suspicion of bacterial infection, antibiotic treatment was started on the first hospital day, but was ineffective. From the fourth hospital day, multiple generalized, partially confluent, erythematous papules appeared. To exclude viral infection, we measured antibody titers against various viruses, and found that the levels of anti-measles IgG and IgM antibodies were elevated, which led to a diagnosis of modified measles. During the course of measles, she developed severe leukopenia, and bone marrow aspiration revealed bone marrow suppression. This was considered to be due to measles infection, leading to a diagnosis of severe modified measles. This is the first case report of modified measles which developed during the use of an anti-TNF-α preparation, and suggests that immunosuppression induced by the preparation may play a role in measles reinfection and its aggravation.

View full abstract