[in Japanese]

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[in Japanese]

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[in Japanese]

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    The association of HLA-B51 with Behçet’s Disease (BD) is well-known. Recent reports have suggested that HLA-A26 might also be associated with BD. We therefore investigated the frequency of HLA-A26 in BD and its relevance with clinical features. HLA phenotyping data were obtained from 161 patients (76 males and 85 females), who had been diagnosed with BD at Teikyo University Hospital from 1989 until 2009 according to the 1987 criteria of BD Research Committee of Japan. The frequencies of B51 and A26 were 48.4% and 29.2%, respectively, which were significantly higher than those of the healthy Japanese population (p＜0.0001and p＝0.0135 ［Fisher’s exact probability test］). The incidence of mucocutaneous lesions, ocular manifestation and epididymitis were not significantly correlated with A26. The incidence of vascular involve-ment (VBD) and that of joint involvement were significantly lower in A26-positive patients compared with A26-negative patients (4.3% vs. 21.1% ［p＝0.0085］ and 23.4% vs. 41.2% ［p＝0.0463］, respectively). The incidence of intestinal involvement (IBD) appeared to be higher in A26-positive patients, although it did not reach statistical significance (p＝0.1412). The results confirm the significant association of HLA-A26 with BD in Japanese patients. Moreover, the data suggest that HLA-B51 and HLA-A26 might have significant influences on the expression of various organ involvements.

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    Clinical squeal of the treatment of rheumatoid arthritis (RA) patients with adalimumab (ADA) was evaluated. A total of 159 patients with active RA were enrolled at Konan Kakogawa Hospital and Kobe University Hospital. The patients’ profile at the start of study was age 57.8±12.8, women/men 129/30, Class 2.1±0.6 and X-ray stage 2.9±1.0. Of 159 patients, 115 (72.3%) were naïve biologics (naïve group) and 44 (27.7%) experienced more than one another biologics (switch group). Both naïve and switch groups showed significant improvements in disease activity including CRP, ESR and MMP-3 at 12-month periods, whereas it was relatively lesser in the switch group. At 6-month period, the drug adherence rate was approximately 80% both in naïve group and the switch group switched from infliximab. Instead, the drug adherence rate was 38% in the switch group switched from etanercept and 62% in the switch group switched from more than 2 biologics. In our facilities, 51 (32.1%) were treated with ADA, and 108 (67.9%) were treated with ADA and methotrexate (MTX). At 3 to 9-month periods, patients treated with ADA and MTX showed better improvement in CRP than those of treated with ADA solely. Of 46 patients, 14 (30.4%) patients treated with ADA for1year showed no progression in joint destruction as judged by X-ray study.

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Objective: Transverse arch flattening and plantar hyperkeratosis are major foot problems for RA patients. A foot orthosis is often used for bolstering the transverse arch. However, diagnostic imaging of transverse arch correction by orthosis had not been reported. We investigated the feasibility of ultrasound (US) diagnosis for the evaluation of the transverse arch.
Methods: A transverse US probe was placed on the dorsal side of metatarsophalangeal (MTP) joints. The transverse arch angle is formed by the line connecting the second MTP to the third MTP joints and the line connecting the third to the fourth MTP joints. Randomization was performed employing the computer-generated block method.31outpatients with RA and plantar hyperkeratosis were treated with a metatarsal pad consisting of a metatarsal pad and reorienting bandage for hallux valgus, or a placebo band made of the reorienting bandage for hallux valgus alone for four weeks. The Foot function index (FFI) at the final assessment was compared with that at baseline in both the metatarsal pad and placebo band groups.
Results: In participants wearing metatarsal pad (n＝15), the transverse arch angle was significantly increased (6.5±5.5°). These significant differences were not found in the placebo band group (n＝16), (P＝0.12). The metatarsal pad group demonstrated significantly improved FFI values in comparison with their baseline assessments (P＝0.002). These significant differences were not found in the placebo band group (P＝0.91).
Conclusion: The efficacy of a metatarsal pad was significant in US diagnosis and clinical assessment in patients with plantar hyperkeratosis of RA.

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    This study evaluated the clicking of the proximal ulnar stump after the Sauvé-Kapandji (SK) procedure in patients with rheumatoid arthritis from 1988 to 2008. 51 patients (9 male, 42 female) underwent 60 SK procedures and the clicking of the ulnar stump was examined with a minimum follow-up period of1year after the SK procedure. The age at the time of operation ranged from 21.8 to 75.5 (average;54.5) years old. The follow-up period was from 1 year 1 month to 20 years 6 months (average: 7 years 6 months) after the SK. Twenty-one cases with extensor tendonrupture received an additional tendon transfer or tendon graft. External fixation was used for 1week for the cases without tendon repair and 3 weeks for the cases done tendon repair. Existence of clicking about the ulnar stump with or without pain was diagnosed at the time of follow-up. The ulnar distance (UD), indicating the distance between the distal radial joint and the proximal ulnar stump was measured using X-ray film. Tapering of the proximal ulnar stump was defined when the diameter of the proximal ulnar stump was less than half of the diameter of the ulna. UD, tapering and the scallop sign of the radius were classified by clicking and pain and evaluated statistically. Forty percent of the cases complained of clicking and 8.3% of the cases complained of painful clicking. The average UD was 24.7±6.6 mm of cases with clicking, 24.3 mm±6.4 mm without clicking, 24.5±6.6 mm with painless clicking and 23.7±6.4 mm with painful clicking. Both tapering and the scallop sign appeared in 35% of the cases, 15% of the cases with painless clicking, and 1.7% of those with painful clicking. In addition, no significant differences were observed in the clicking, UD, tapering or the scallop sign. Pain of the proximal ulnar stump was no more frequent than the other cases.

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    To clarify the clinicopathological features of Churg-Strauss syndrome (CSS), we examined 12 cases that presented with clinical features described by Churg and Strauss and also fulfilled the cristeria of American college of rheumatology. Patients, 5 male and 7 female, were 51.8 years of mean age. Anti-neutrophil cytoplasmic antibody (ANCA) was positive in 7 cases (58%, MPOANCA in 6, PR3-ANCA in1). All patients had a medical history of bronchial asthma (BA). Various organ involvements such as mononeuritis multiplex (12 cases), skin (9 cases), sinusitis (8 cases), lung and heart (5 cases, respectively), sensory organ (4 cases), central nervous system (2 cases), and gastrointestinal tract and kidney (1case, respectively) were observed. ANCA positive patients had significantly higher prevalence of skin involvement than ANCA negative patients. We also observed a tendency of ANCA negative patients having a higher frequency of lung and heart lesion.
    Skin biopsy was performed in6cases.Extravascular infiltration of eosinophils was observed in all of them, but necrotizing vasculitis and extravascular granuloma, which is thought to be characteristic to CSS, was observed in only1case, respectively. Neural biopsy was done in 4 cases. Almost all the cases showed axonal degeneration and decrease of nerve fiber density, but no cases showed necrotizing vasculitis and extravascular granuloma.
    In conclusion, various types of organ involvement were observed in12cases with CSS, but typical pathological changes such as necrotizing vasculitis and extravascular granuloma were not common in CSS.

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    Henoch-Schönlein purpura (HSP) is characterized by systemic leukocytoclastic vasculitis of small vessels, while medium-sized vessels can rarely be involved. Clinical manifestations usually include palpable purpura, arthralgia, nephritis and abdominal pain. HSP occurs commonly in children between 3 and 15 years old, while rather rarely in adults. A 58-year old man was referred to our hospital because of complaining of epigastric pain and high grade fever. Since an abdominal CT scan showed a wedge-shaped area of contrast defect in the renal parenchyma on both sides, he was hospitalized on suspicion of pyelonephritis. However, during hospitalization, he developed severe epigastric pain, purpura of both lower extremities and,-polyarthritis. A skin biopsy was performed. The pathological result showed the presence of leukocytoclastic vasculitis. Therefore, he was diagnosed with HSP. A follow-up abdominal CT scan revealed a high density area consistent with the previous failure of bilateral renal parenchyma contrast, suggesting renal hemorrhagic infarction. After 40 mg of prednisolone was administered intravenously once daily, abdominal and joint pain rapidly improved. Purpura of both extremities and the abnormal findings of CT scan diminished. We here report a rare adult case of HSP complicated with involvement of medium-sized vessels, which resulted in hemorrhagic renal infraction. It seems that it will be necessary to accumulate further cases of HSP complicated with mediumsized vessel involvement.

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