Behçet disease associated with myelodysplastic syndrome.

There have been 22 reported cases of Behçet disease associated with myelodysplastic syndrome. The majority of cases belong to incomplete types of Behçet disease and the refractory anemia subtype of myelodysplastic syndrome. We describe a case of a 49-year-old woman with Behçet disease who developed myelodysplastic syndrome with abnormal karyotype-trisomy 8. This change was not due to immunosuppressive agents because her Behçet disease was not treated with these drugs before the onset of myelodysplastic syndrome. This is the first report of a case of Behçet disease with pathologic evidence associated with the chronic myelomonocytic leukemia subtype of myelodysplastic syndrome. After reviewing the past case studies, we suggest that patients with myelodysplastic syndrome and trisomy 8 might be prone to have Behçet disease. Furthermore, more intestinal ulcers but with less eye lesions and arthritis have been noted in patients of Behçet disease with myelodysplastic syndrome than in those without myelodysplastic syndrome.

Cytokine production from peripheral blood mononuclear cells in patients with ankylosing spondylitis and their first-degree relatives.

BACKGROUND: To understand the cytokine levels in different disease activities of patients with ankylosing spondylitis (AS), we measured proinflammatory and antiinflammatory cytokine production from peripheral blood mononuclear cells (PBMC) in patients with AS and their first-degree relatives (FDR). METHODS: PBMC were obtained from 26 patients with AS and 24 FDR and then stimulated with PHA for 72 h. In the supernatants, the following three cytokines, tumor necrosis factor-alpha (TNF-alpha), interleukin 1beta (IL-1beta), and IL-10, were measured by ELISA. Disease activity in AS patients was divided into high disease activity (Group 1) and low disease activity (Group 2), based on the Bath AS Disease Activity Index (BASDAI > or =4 or <4). Healthy FDR of AS patients (Group 3) and healthy subjects (Group 4) were used as a control group. RESULTS: TNF-alpha production from PBMC was significantly increased in Group 1 patients compared to Group 2 patients (1371 +/- 1008 pg/mL vs. 355 +/- 89 pg/mL, p <0.05) or FDR (1371 +/- 1008 pg/mL vs. 552 +/- 89 pg/mL, p <0.05) or healthy subjects (1371 +/- 1008 pg/mL vs. 436 +/- 114 pg/mL, p <0.01). IL-1beta also showed a similarly significant difference between the two groups (Group 1 vs. Group 2, Group 1 vs. Group 4) (p <0.05). In contrast, IL-10 was significantly decreased in Group 1 when compared to Group 2 (126 +/- 64 pg/mL vs. 272 +/- 150 pg/mL, p <0.05). CONCLUSIONS: Patients with high BASDAI had increased production of TNF-alpha and IL-1beta compared to those with low BASDAI or healthy FDR, suggesting that proinflammatory cytokines may play an important role during active inflammation.

An unusual case of Candida tropicalis and Candida krusei arthritis in a patient with acute myelogenous leukemia before chemotherapy.

A 79-year-old male with acute myelogenous leukemia developed acute right knee arthritis during admission, after the use of broad-spectrum antibiotics before chemotherapy. The initial synovial fluid sample appeared to be mildly inflammatory with a low white cell count. The fungal septic arthritis was not diagnosed until Candida tropicalis, a rare species of Candida, was isolated in the synovial fluid. Although fluconazole is effective in treating the microorganism, the untreated leukemia rendered the infection incurable and led to the growth of fluconazole-resistant Candida krusei. We reported the unusual case of fungal arthritis and reviewed the literature.

Undifferentiated spondyloarthropathy in Chinese patients.

BACKGROUND: Undifferentiated spondyloarthropathy (USpA) is a unique group in spondyloarthropathy (SpA). This study will investigate the clinical and laboratory characteristics of USpA in the Chinese population. METHODS: Forty two patients with USpA were enrolled from our rheumatology outpatient facility in this retrospective study. SpA was diagnosed according to the European Spondyloarthropathy Study Group (ESSG) criteria. Patients were considered having USpA when they had SpA but did not meet the criteria for the diagnosis of ankylosing spondylitis (AS), Reiter's syndrome or reactive arthritis (ReA), psoriatic arthritis (PsA), and inflammatory bowel diseases (IBD)-related arthritis, etc. Laboratory tests included erythrocyte sedimentation rate (ESR), serum C-reactive protein (CRP), immunoglobulin A (IgA), and human leukocyte antigen B27 (HLA-B27). RESULTS: Among the 42 USpA patients, the ratio of men to women was 1.47:1, and the age at onset was 32.33 +/- 10.83 years old. Approximately 61.9% of patients had peripheral arthritis, 30.95% had uveitis, and 64.29% had positive HLA-B27. Among these female USpA patients, compared to males, there is a trend of older age at disease onset, higher percentage of HLA-B27 positive, more peripheral arthritis and uveitis, longer disease duration, and higher level of ESR, and IgA and CRP in serum. The items reaching significant difference between males and females were longer disease duration (p < 0.001), higher level of ESR (p < 0.001), and serum IgA (p = 0.03). There was no significant difference in clinical and laboratory characteristics between HLA-B27-positive and -negative groups. CONCLUSIONS: Studies on USpA patients have not been reported in the Chinese population. In this study, we demonstrate the unique demographic characteristics, clinical and laboratory data of USpA in the Chinese population. These findings should be confirmed by analyzing larger number of patients and longer time for further follow-up. Such studies are crucial to understand the pathogenesis of USpA and evaluate its prognosis.

Serum anti-Yersinia antibody in Chinese patients with Kawasaki disease.

BACKGROUND: Many infectious agents have been implicated as an etiology to develop Kawasaki disease (KD). In Taiwan, studies on the relationship between Yersinia and KD have not been reported. METHODS: We measured sera for anti-Yersinia antibodies by using enzyme immunoassay (EIA) in 31 patients with KD and 60 healthy children (HC). Yersinia strains included Y. pseudotuberculosis I, II, III, IV, V, VI and Y. enterocolitica O3, O8 and O9. RESULTS: Data of 31 patients with KD showed that for the IgG antibody, serum anti-Y. pseudotuberculosis II, III, Y. O8 and O9 antibody were significantly higher when compared to the HC. Except for Y. pseudotuberculosis IV, all other Yersinia strains of either IgA or IgM antibodies increased significantly in patients with KD vs. the HC. If we compared the number of patients who had significant elevation of OD and those of HC, we found IgA anti-Yersinia antibodies (PST I, PST II, O3, O8, O9), IgM (PST VI, O8) and IgG (PST II, O8, O9) were significantly elevated in KD patients than in HC. A significant relationship was present between KD with myocarditis and increased anti-Yersinia antibody titer. CONCLUSIONS: The findings in this study suggest that preceding Yersinia infection may play a role in the pathogenesis of KD. Further study of the relationship between KD with myocarditis and increased anti-Yersinia antibody is needed.

Second-degree atrioventricular block as an early manifestation of adult systemic lupus erythematosus.

Second-degree atrioventricular (AV) block had not been reported as an early manifestation of adult systemic lupus erythematosus (SLE). An 18-year-old woman of SLE presented with asymptomatic second-degree AV block with 2:1 conduction block on electrocardiogram (ECG) during admission. Serologic tests were negative for anti-Sjögren's syndrome A (anti-SS-A/Ro) and anti-SS-B/La antibodies, but positive for anti-ribonuclearprotein antibodies. Her abnormal ECG completely resolved soon after high-dose intravenous methylprednisolone infusion, and she was maintained successfully with a low dose of oral steroid. The possible pathogenesis of this complication is discussed. Follow-up with periodical ECG is recommended for adult lupus patients to screen for possible conduction system involvement, and treatment should be started as soon as possible.