San-Yuan Kuo

The clinical features and prognosis of male lupus in Taiwan

Our objective was to describe the clinical manifestations, laboratory data, and prognosis in male Chinese lupus patients in Taiwan. Seventy-two male lupus patients, diagnosed and followed in Tri-Service General Hospital between 1983 and 1996, were studied and their clinical data analyzed, retrospectively. The mean age at diagnosis was 34–16 y (mean–s.d., range: 13 – 82 y). The peak incidence of age at diagnosis was between 13 and 40 years. The three most frequent clinical manifestations were renal disease, 75%; malar rash, 61%; and arthritis, 53%. The three most frequent abnormal laboratory findings were anti-nuclear antibodies (ANA), 97%; hypocomplementemia, 76%; and anti-ds DNA, 57%. The 1-, 5-, and 10-year survival rates were 85%, 76%, and 75%, respectively. In summary, a higher frequency of renal disease, malar rash and photosensitivity, but a lower frequency of arthritis and lymphadenopathy were demonstrated in our 72 ethnic Chinese male Taiwanese lupus patients compared to previous reports of Caucasians. The prognosis seems to be similar to that of both Chinese and Caucasian males but seems to be poor when compared to either Chinese or Caucasian female lupus patients.

Effects of anti-rheumatic herbal medicines on cellular adhesion molecules

OBJECTIVE To test the hypothesis whether herbal medicines ameliorate inflammatory diseases via the modulation of cellular adhesion molecules (CAMs). METHODS Human neutrophils, synovial fibroblasts, and endothelial cells were incubated with different concentrations of Tripterygium Wilfordii Hook-f (TWH-f) or Tetrandrine in the presence or absence of interleukin 1 (IL1). The amount of soluble E-selectin, intercellular adhesion molecule-1 (ICAM-1) and vascular cellular adhesion molecule-1 (VCAM-1) secreted by cells were determined by ELISA. The cell surface expression of these three CAMs was detected by flow cytometry. RESULTS TWH-f at high concentration (50 ng/ml) has a significant (p<0.05) inhibitory effect on both the secretion and the expression of the cellular adhesion molecules. However, Tetrandrine did not demonstrate the same effects. CONCLUSIONS The cellular adhesion molecules of the endothelium and leucocytes may constitute excellent targets for the development of new anti-inflammation medicines. These results indicate that TWH could be a potential therapeutic agent in the treatment of inflammatory diseases.

Cytokines and Cell Surface Markers in Prediction of Cardiac Allograft Rejection

Endomyocardial biopsy is generally used to quantify heart allograft rejection and guide immunotherapy. Biopsy, however, is invasive, costly, and risky. Since rejection requires lymphocyte activation, the purpose of this study was to assess alternative methods to evaluate rejection dynamics by investigating serum levels of cytokines and cell surface markers after heart transplantation. Interleukin-2-receptor bearing CD4+T (IL-2R/CD4) cell levels were higher in the peripheral blood of human transplant recipients with rejection grade 2 (p < 0.02). HLA-DR/CD3 levels were somewhat higher in rejection grade 2. There was no correlation between biopsy scores and serum levels of tumor necrosis factor (TNF-alpha), IL-2, or percentage of T cell, NK cell, B cell, CD4+T cell, CD8+T cell, HLA-DR/CD4, HLA-DR/CD8, IL-2R/CD3, IL-2R/CD8. Interleukin-1 (IL-1 beta) was not detectable in all of the samples. The current studies suggest that monitoring lymphocyte IL-2R/CD4 and HLA-DR/CD3 levels is useful in predicting cardiac transplant rejection.

Systemic lupus erythematosus with simultaneous onset of Kikuchi-Fujimoto’s disease complicated with antiphospholipid antibody syndrome: a case report and review of the literature

Histiocytic necrotizing lymphadenitis, called Kikuchi-Fujimoto’s disease (KFD), is an idiopathic, self-limited condition rarely associated with systemic lupus erythematosus (SLE). The cause of concomitant KFD and SLE is still unknown. We describe a 19-year-old man simultaneously diagnosed with both KFD and SLE complicated with deep vein thrombosis (DVT). To the best of our knowledge, this is the first case report of KFD associated with SLE complicated with antiphospholipid antibody syndrome (APS). Our patient was successfully treated with intravenous pulse methylprednisolone, anticoagulation with heparin, oral hydroxychloroquine, azathioprine, and low-dose aspirin.

Etanercept as a rescue agent in patient with adult onset Still’s disease complicated with congestive heart failure

Adult onset Still’s disease (AOSD) is an uncommon disorder of unknown cause. The clinical symptoms of AOSD are a spiking fever, a typical rash, arthralgia or arthritis, sore throat, lymphadenopathy, and splenomegaly. Pleuropulmonary and cardiac involvement are rare. We report a patient with a two-year history of AOSD with myocarditis refractory to cyclosporine and glucocorticoid. Significant congestive heart failure due to left ventricle dysfunction and hyperferritinemia developed during the hospital course. After therapy with etanercept, the patient’s clinical manifestations recovered and she regained normal left ventricular systolic function.

CELL SURFACE MARKERS AND CIRCULATING CYTOKINES IN GRAFT VERSUS HOST DISEASE

Graft versus host disease (GVHD) remains the major obstacle to the widespread application of allogeneic bone marrow transplantation (BMT) despite improvement in drug prophylaxis. T cells in the donor bone marrow recognize and react against host alloantigens and thereby initiate GVHD, but the precise mechanisms by which host tissues are damaged remain unclear. In the current study, we determined the cytokine secretion, cell population distribution, and cell surface markers expression by ELISA and flow cytometer, to understand further the pathophysiology of GVHD. Our results demonstrated that there was no significant change in the cell ratio of B-and T- lymphocytes, and helper/suppressor cells during GVHD development when compared to the condition before transplantation. Furthermore, the percentage of natural killer cells, the interleukin-2 receptor (IL-2R) or the HLA-DR antigen on both CD4 and CD8 positive cells presented no significant difference between pre-transplantation and during GVHD. The serum cytokine secretion of IL-1, TNF-alpha, IL-2, ICAM-1, endothelin, TGF-beta showed no difference before BMT and during GVHD. However, when patients in the developing of GVHD, there was significant difference in the serum levels of soluble IL-2R (slL-2R), epidermal growth factor (EGF), and platelet derived growth factor (PDGF). In addition, with patients who develop GVHD, the mixed lymphocyte reaction also presented a significant difference. This study indicated that some serum cytokines such as sIL-2R, growth factors, and the mixed lymphocyte reaction may be used as parameters for the early detection of the development of GVHD.

Ruptured renal microaneurysms complicated with a retroperitoneal abscess for a patient with systemic lupus erythematosus

Renal artery aneurysm is extremely rare among patients with systemic lupus erythematosus.(SLE). Herein, we report on a 22-year-old male lupus patient who presented with acute abdominal pain, anemia and subsequent hypertension. Abdominal computed tomography revealed a peri-renal hematoma over the right kidney. A renal angiography revealed bilateral renal microaneurysms. The patient subsequently developed a right-side retroperitoneal abscess 4 weeks after hematoma formation and received an emergent laparotomy with drainage. Subsequent culture of the abscess-derived fluid revealed the presence of Proteus mirabilis and Escherichia coli. Following appropriate antipyretic and immunosuppressive drugs therapy, the patient recovered successfully. To the best of our knowledge, this is the first report of SLE associated with a retro-peritoneal abscess probably secondary to a ruptured renal microaneurysm.

A Comparison of the Diagnostic Sensitivity and Specificity of Two Anti-cyclic Citrullinated Peptides (CCP1 and CCP2) Tests for Rheumatoid Arthritis

The detection of anti-cyclic citrullinated peptide (CCP) autoantibodies has been considered a useful tool to identify rheumatoid arthritis (RA) patients. Here, we examined the sensitivity and specificity of two detection methods, namely the ELISA-based (anti-CCP1) and the EliA-based (anti-CCP2) for anti-CCP in 40 RA patients. Methods. Anti-CCP1 and anti-CCP2 antibody tests were performed on 79 patients with arthropathy. The sensitivity, specificity, positive predictive value, and negative predictive value for-discriminating between rheumatoid arthritis (RA) and non-RA were calculated for both tests. Results. Both anti-CCP1 and anti-CCP2 shared very similar specificities (97.4%) toward diagnosing RA. However, there was no significant associations between the titer of anti-CCP1 and anti-CCP2. Conclusion. Anti-CCP test was more sensitive and specific than traditional IgM-RF. However, anti-CCP2 was not significantly better than anti-CCP1 in the diagnosis of RA.

Life Threatening Pancytopenia with Azathioprine in a Systemic Lupus Erythematosus Patient

Severe and rapidly evolving pancytopenia due to toxicity from low dosage (＜3 mg/kg/day) azathioprine (AZA) in patients with autoimmune diseases is uncommon. We describe a 38-year-old woman with systemic lupus erythematosus (SLE) who developed pancytopenia 5 week after low-dose AZA (0.75 mg/kg/day) was started as a corticosteroid – sparing agent. After cessation of AZA, the clinical and hematologic abnormalities resolved. We report here a case of early and severe myelosuppression associated with AZA and review similar cases reported in the literature.

Diagnostic Validity of Computed Tomography for HLA-B27 Negative Ankylosing Spondylitis: A Retrospective Study of 209 Patients

Objective: Radiographic sacroiliitis is considered the hallmark of ankylosing spondylitis (AS). However, large interobserver variations make plain radiographic diagnosis of sacroiliitis notoriously difficult. The heterogeneity of HLA-B27 negative AS makes its diagnosis even more challenging. Several reports have shown that sacroiliitis tends to be underestimated using radiography, and that computed tomography (CT) facilitates AS diagnosis in patients with suspected spondyloarthritis. However, no studies have stressed the clinical utility of CT, particularly in the diagnosis of HLA-B27(-) AS. Methods: We retrospectively evaluated 209 HLA-B27(-) patients with chronic lower back pain and suspected AS. Radiography and CT reports were examined and outcomes were compared. Results: Among 408 sacroiliac (SI) joints examined using both radiography and CT, there was agreement between the two methods in the sacroiliitis grading of 82 (20.1%) SI joints. However, sacroiliitis grade using CT was higher in 276 (67.6%) SI joints and lower in 50 (12.3%) SI joints. CT evaluation of SI joints showed that 78.9% of patients met the radiographic sacroiliitis criteria of the modified New York criteria, while only 26% of patients satisfied the criteria for plain radiography. Surprisingly, 117 patients (57.3%), who did not meet the modified New York AS diagnosis criteria for plain radiography met the criteria for CT. Conclusion: CT was sensitive and useful in providing evidence for the diagnosis of AS. We suggest that HLA-B27(-) patients with equivocal plain radiography results and chronic inflammatory lower back pain be examined using CT.