The mortality of antineutrophil cytoplasmic autoantibody (ANCA) -associated vasculitis is still high especially in cases of severe life-threatening disease such as diffuse alveolar hemorrhage (DAH) and rapidly progressive glomerulonephritis (RPGN). Although aggressive immunosuppressive therapy is reported to be effective, the use of cytotoxics is associated with a high degree of adverse effects, with infection being the most common cause of early death. We supposed that the use of plasmapheresis plus steroid therapy without using other immunosuppressive agents would attain better efficacy while limiting adverse effects. We diagnosed 22 patients with MPO-ANCA-associated RPGN in our institution between 1997 and 2006. DAH occurred in 7 of the patients (2 males), age 72±9yr, serum creatinine 5.7±3.3mg/dL, C-reactive protein 14.3±7.5mg/dL, ANCA titer 569±581U/mL with a mean follow-up time of 34±22 months. Hemoptysis associated with multilobular infiltrates, hypoxia, and progressive anemia was seen in all patients. A ventilator was required in 3 patients. All patients were treated with full plasma volume apheresis daily until DAH improved, and received methylprednisolone pulse and subsequent oral prednisolone (0.7~1.0mg/kg/day). DAH resolved with apheresis in 7 of 7 patients (100%) with 3.5±1.3 treatments. The average interval between hemoptysis and first apheresis treatment was 2.9±2.0 days. Average hospitalization was 3.6 months (2~8 months). No patient was oxygen or ventilator dependent at the time of discharge. All patients had impaired renal function on admission and dialysis was required in 5 patients. Three patients were dependent on dialysis but the others had improved renal function. No patient experienced severe infection within the first 6 months but one patient died as a result of aspiration pneumonia occurring 16 months after leaving the hospital. Conclusion : Plasmapheresis plus steroid therapy was effective in attaining remission while avoiding the use of cytotoxics. Such therapy can be lifesaving with respect to the pulmonary complications of this syndrome.

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Dendritic cells (DCs) in circulation are immature DCs derived from precursors in bone marrow. In humans, two distinct subsets of immature DCs, myeloid DCs (DCm) and plasmacytoid DCs (DCp), have been identified. These immature DCs may have originally evolved to serve as effector cells in antimicrobial innate immunity. DCm ingests and kills bacteria and fungi. DCp represents the key effector cells in the early innate immune response by producing large amounts of IFN-α against viral and bacterial infection. In addition, mature DCs after ingesting antigens are the most potent antigen-presentation cells that play a pivotal role in initiating the acquired immune response in lymph nodes. During primary immune responses, the mature DC subset is one of the crucial determinants for polarlizing naïve T cells into helper T cell subsets, such as Th1 and Th2. We examined the number of DCm and DCp in the peripheral blood of 48 chronic hemodialysis (HD) patients and 18 age-matched healthy subjects, using flow cytometry. We found a significant decrease in the number of DCp in HD patients, resulting in a marked increase in the DCm : DCp ratio in those patients. Serum levels of β₂ microglobulin (β₂MG) were significantly correlated with the number of DCp in HD subjects. The number of each DC subset was not significantly changed before and after HD treatment. The relation between DC subset ratio and helper T subset ratio was not significant. In conclusion, characteristics of peripheral DC subsets were rather different in HD patients. The reduction of blood DCp counts is common in HD patients and the inadequate number of DCp may be involved in their compromised immunity.

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We report a case of Clostridium difficile-associated diarrhea (CDAD) in a patient undergoing chronic hemodialysis caused by diabetic nephropathy. A 58-year-old female had received maintenance hemodialysis for 9 years and complained of persistent watery diarrhea. Despite the administration of probiotics and loperamide, diarrhea continued. She had never received antibiotics treatment. CD antigen was positive in the stool. Colon fiberscopy showed no pseudomembranous lesion in the total colon and the rectum. She was diagnosed as having CDAD, so vancomycin was given by oral administration for 2 weeks, which was very effective and diarrhea disappeared. Diarrhea did not recur after the administration of vancomycin. This study suggested that physicians should consider CDAD in persistent diarrhea in maintenance hemodialysis patients.

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A 75-year-old-female began to undergo hemodialysis due to chronic renal failure caused by diabetic nephropathy in 1992. She had been treated for moderate temperature burn ulcers on her lower limbs by ointment and other treatments on four different occasions. She felt right foot pain early in March 2007 and thereafter, right diabetic foot gangrene gradually appeared, ranging from the first and second toes to the dorsal pedis ; thereafter, she was admitted to our hospital on March 27, 2007. The patient's gangrene was associated with infection, and we therefore considered it was impossible to treat here by internal medicine therapy consisting of only antibiotics. In addition, continuous stenosis of the right anterior tibial artery, right posterior tibial artery, and right peroneal artery of the lower limbs was observed on magnetic resonance angiography (MRA). Skin perfusion pressure (SPP) was 28mmHg in the right dorsal pedis, 26mmHg in the right plantar pedis, and 107mmHg in the right proximal ankle joint. As a result, we initially thought it would therefore be necessary to amputate the right distal lower leg ; however, we first performed percutaneous transluminal angioplasty (PTA) for the right anterior tibial artery and, after completing this procedure, the SPP increased to over 40mmHg in both the right dorsal pedis and plantar pedis. As a result, we could therefore perform Chopart's amputation in order to successfully treat the patient and reduce the extent of the amputation.

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We report a hemodialysis patient associated with nonclostridial anaerobic cellulitis after tooth extraction for chronic marginal periodontitis. The patient was a 72-year old Japanese male treated with maintenance hemodialysis therapy for end-stage renal failure due to diabetic nephropathy. He developed dysphagia, fever, marked neck swelling and severe neck pain. He had undergone a tooth extraction six days previously. Neck computed tomography showed gas-density lesions in his peripharyngeal space. He was admitted and underwent surgical exploration of the cervical region, drainage, and debridement immediately. On bacteriological examination, Streptococcus anginosus/milleri and Prevotella intermedia were detected from his purulent matter and he was diagnosed with nonclostridial anaerobic cellulitis. He was treated with antibiotics (ABPC/SBT 3g/day, and CLDM 1,200mg/day), γ-globulin (5.0g/day×3 days), and daily rinsing, in addition to maintenance hemodialysis and insulin administration for strict blood glucose control. After the operation, the amount of purulent matter decreased by these conservative therapies and his symptoms immediately improved, therefore, drainage was discontinued on March 9, 2006, and he was discharged the next day. One month later, the facial and neck abscess was not detected on computed tomography. In the present case, immediate diagnosis and surgical drainage were effective, despite the life-threatening disease and compromised conditions. Moreover, this case suggested that odontogenic infection may lead to a serious infection, such as cellulitis.

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