Real-world risk factors for herpes zoster in patients with rheumatoid arthritis undergoing tofacitinib treatment.

BACKGROUND: This study sought to assess the risk factors of herpes zoster (HZ) in rheumatoid arthritis (RA) patients treated with tofacitinib (TOFA). METHODS: This retrospective study reviewed RA patients receiving TOFA. We compared clinical characteristics, laboratory profiles, concomitant medication use, and HZ incidence in patients with and without recent biologic synthetic disease-modifying anti-rheumatic drugs (bDMARDs) treatment, which is defined as their administration ≤ 180 days prior to the initiation of TOFA treatment. We used univariate Cox proportional hazards models and Kaplan-Meier analysis to assess risk factors. RESULTS: Among 304 RA patients, 97 had recent bDMARDs use and 207 did not. Patients with recent bDMARDs use typically had lower weekly doses of methotrexate, less hydroxychloroquine use, and shorter follow-up times. In the recent bDMARDs group, 64 (66.0%) used tumor necrosis factor inhibitors (TNFi), 19 (19.6%) used tocilizumab, and 14 (14.4%) used abatacept. The overall incidence rate (IR) of HZ was 5.62 per 100 person-years. Patients with recent bDMARDs use exhibited a higher HZ risk compared to those without recent bDMARDs use (IR ratio: 2.34, 95% confidence interval [CI]: 1.04-5.19, p = 0.028). Recent bDMARDs use (hazard ratio: 2.4, 95% CI: 1.12-4.95, p = 0.024) was an independent risk factor for HZ among multivariable analysis. Kaplan-Meier analysis confirmed increased HZ risk in RA patients on TOFA with recent bDMARDs use (log-rank p = 0.015). CONCLUSION: HZ is common in RA patients treated with TOFA, and recent bDMARDs (TNFi, tocilizumab and abatacept) use is a risk factor of HZ. HZ vaccination, therefore, should be recommended for this group.

Interstitial fibrosis increases the risk of end-stage kidney disease in patients with lupus nephritis.

OBJECTIVE: To evaluate the risk of end-stage kidney disease (ESKD) in LN patients using tubulointerstitial lesion scores. METHODS: Clinical profiles and histopathological presentations of 151 biopsy-proven LN patients were retrospectively examined. Risk factors of ESKD based on characteristics and scoring of their tubulointerstitial lesions [e.g. interstitial inflammation (II), tubular atrophy (TA) and interstitial fibrosis (IF)] were analysed. RESULTS: The mean age of 151 LN patients was 36 years old, and 136 (90.1%) were female. The LN cases examined included: class I/II (n = 3, 2%), class III/IV (n = 119, 78.8%), class V (n = 23, 15.2%) and class VI (n = 6, 4.0%). The mean serum creatinine level was 1.4 mg/dl. Tubulointerstitial lesions were recorded in 120 (79.5%) patients. Prior to receiving renal biopsy, nine (6.0%) patients developed ESKD. During the follow-up period (mean, 58 months), an additional 47 patients (31.1%) progressed to ESKD. Multivariate analyses identified serum creatinine [hazard ratio (HR): 1.7, 95% CI: 1.42-2.03, P < 0.001] and IF (HR: 3.2, 95% CI: 1.58-6.49, P = 0.001) as independent risk factors of ESKD. Kaplan-Meier analysis further confirmed a heightened risk of ESKD associated with IF. CONCLUSION: Tubulointerstitial involvement is commonly observed in the histopathological presentation of LN. However, IF, rather than II or TA, was found to increase the risk of ESKD in our cohort. Therefore, to predict renal outcome in LN patients prior to adjusting immunosuppressive treatment, the degree of IF should be reviewed.

Risk Factors and Incidence of Serious Infections in Patients With Systemic Lupus Erythematosus Undergoing Rituximab Therapy.

OBJECTIVE: To evaluate the risk and protective factors of serious infection (SI) in patients with systemic lupus erythematosus (SLE) within 180 days of rituximab (RTX) treatment. METHODS: Patients with SLE treated with RTX were analyzed. SI was defined as any infectious disease requiring hospitalization. The clinical characteristics, laboratory profiles, medications, and incidence rate (IR) are presented. Multivariate Cox proportional hazards models and Kaplan-Meier analysis for risk factors of SI were performed. RESULTS: A total of 174 patients with SLE receiving RTX treatment were enrolled. The overall IR of SIs was 51.0/100 patient-years (PYs). Pneumonia (30.4/100 PYs), followed by soft tissue infections, intra-abdominal infections, and Pneumocystis jiroveci pneumonia (all 6.1/100 PYs) were the leading types of SIs. Twelve patients died during the 180-day follow-up (crude mortality rate: 14.6/100 PYs). Chronic kidney disease (CKD), defined as an estimated glomerular filtration rate < 60 mL/min/1.73 m2 (hazard ratio [HR] 2.88, 95% CI 1.30-6.38), and a background prednisolone (PSL) equivalent dosage ≥ 15 mg/day (HR 3.50, 95% CI 1.57-7.78) were risk factors for SIs among all patients with SLE. Kaplan-Meier analysis confirmed the risk of SI for patients with SLE with CKD and a background PSL equivalent dosage ≥ 15 mg/day (log-rank P = 0.001 and 0.02, respectively). Hydroxychloroquine (HCQ) reduced the risk of SIs in patients with SLE (HR 0.35, 95% CI 0.15-0.82; log-rank P = 0.003). CONCLUSION: SI was prevalent in patients with SLE after RTX treatment. Patients with SLE with CKD and high-dose glucocorticoid use required constant vigilance. HCQ may reduce the risk of SI among patients with SLE administered RTX.

Risk factors for mortality in systemic lupus erythematosus patients: Analysis of adult and pediatric cohorts in Taiwan.

BACKGROUND: Overall survival of systemic lupus erythematosus (SLE) patients significantly increased in recent decades, however, the relative risk of mortality is still high. Long-term survival outcome of pediatric SLE remains unclear. This study aims to explore the long-term survival rate and its predictors in patients with systemic lupus erythematosus (SLE). METHODS: A retrospective, hospital-based cohort study was performed between 2004 and 2018 in a tertiary referral medical center in Taiwan. Data on comorbidities, medications, and causes of admission were collected for risk factor analysis using time-dependent multivariate Cox proportional hazards models. RESULTS: A total of 2392 adults and 115 pediatric SLE patients were enrolled (female, n = 2157 and 95, respectively). The 10-year survival rates were 93.2%, 90.2%, 98.9%, and 100% in adult women, adult men, girls, and boys with SLE, respectively. The overall mortality rate was 2.09 case/100 patient-years (PY) for male SLE and 1.39 case/100 PY for female SLE patients. Male SLE patients did not have a statistically significantly higher mortality rate than female SLE patients in each age stratification. Infectious disease (n = 119), heart failure (n = 21), and cerebrovascular accident (n = 14) were the leading causes of death in adult SLE patients. Advanced age (hazard ratio [HR]: 1.04, 95% confidence interval [CI]: 1.03-1.05), treatment with mean dosage of systemic glucocorticoid equivalent to >10 mg/d of prednisolone (HR: 1.71, 95% CI: 1.14-2.57), comorbidities with malignancy (HR: 1.94, 95% CI: 1.22-3.09), chronic kidney disease (HR: 1.86, 95% CI: 1.25-2.77), hypertension (HR: 1.42, 95% CI: 1.01-1.98), and admission due to bacterial pneumonia (HR: 1.92, 95% CI: 1.12-3.31) and sepsis (HR: 2.78, 95% CI: 1.51-5.13) were independent risk factors for mortality in SLE patients. CONCLUSION: SLE patients with advanced age, malignancy, chronic kidney disease, hypertension, treated with a higher average dosage of glucocorticoids, and admission due to bacterial pneumonia and sepsis have an increased risk of mortality.

Cytomegaloviral or Pneumocystis Jiroveci Pneumonia Increases Mortality in Systemic Lupus Erythematosus Patients with Pulmonary Hemorrhage: Evidence from Bronchoalveolar Lavage Fluid.

OBJECTIVE: To evaluate the role of cytomegaloviral or Pneumocystis jiroveci pneumonia (CMV/PJP) in systemic lupus erythematosus (SLE) patients with pulmonary hemorrhage (PH). METHODS: We retrospectively examined hospital records for 27 SLE patients with PH who received bronchoalveolar lavage fluid (BALF) analyses. Clinical profile and mortality rates were compared between groups with and without CMV/PJP. Risk factors for PH-related mortality were analyzed. RESULTS: Among 27 SLE patients with PH, 15 had pathogens from BALF samples, and 8 had CMV/PJP. Although CMV/PJP was treated, the RR for 90- and 180-day mortality rates of SLE patients with CMV/PJP were higher than those without these infections (5.94, 95% CI 1.44-24.48; 7.13, 95% CI 1.81-28.06, respectively). Risk factors for 90- and 180-day mortality were presence of CMV/PJP (OR 14.2, 95% CI 1.83-109.9; OR 25.5, 95% CI 2.91-223.3, respectively) and use of pulse methylprednisolone for PH treatment (OR 12.0, 95% CI 1.48-97.2; OR 8.5, 95% CI 1.13-63.9, respectively). Factors increasing the 90-day mortality rate were duration of mechanical ventilation exceeding 14 days (OR 11.1, 95% CI 1.11-112.0) and use of aggressive immunosuppression close to PH onset (OR 7.56, 95% CI 1.09-52.4). Three of the 7 patients receiving aggressive immunosuppression died with the presence of CMV/PJP. CONCLUSION: Owing to the high prevalence of CMV/PJP and its association with mortality, routine BALF analysis is recommended for all suitable SLE patients with PH. Use of aggressive immunosuppression does not benefit SLE patients with opportunistic infections during PH attack.

[Association Between Sleep Patterns and Diurnal Blood Pressure Variation and Its Predictors in Women With Systemic Lupus Erythematosus].

BACKGROUND: Sleep disturbances and hypertension are common health issues in patients with systemic lupus erythematosus (SLE). Limited information is available regarding the objective sleep quality and the variation of diurnal blood pressure (BP) in patients with SLE. Moreover, the relationship between sleep patterns and diurnal BP variation in SLE patients is not clear. PURPOSE: To explore the subjective/objective sleep patterns and the diurnal BP variation in women with SLE, to identify the factors associated with diurnal BP variation, and to identify the predictors of this variation. METHODS: A cross-sectional, descriptive, correlational study was conducted and 42 women with SLE were recruited. Participants completed the Pittsburgh Sleep Quality Index (PSQI), Hospital Anxiety and Depression Scale, and Brief Pain Inventory. Rheumatologists rated current lupus disease activity. Additionally, the participants wore a wrist actigraph for 7 consecutive days and underwent 24h ambulatory BP monitoring for one day. RESULTS: The mean Global PSQI score was 7.74 ± 3.21; 69% of the participants reported poor subjective sleep quality; the actigraphy-measured sleep efficiency was 85.29 ± 5.95%; and 42.9% had poor objective sleep quality. Total sleep time at night was positively associated with diurnal change in diastolic BP (r = .315. p < .05) and pain severity was negatively associated with diurnal change in systolic BP (r = -.430, p < .01) and diastolic BP (r = -.371, p < .05). Multiple linear regression analysis was used to predict diurnal BP variation. Moreover, pain was a significant predictor of diurnal change in systolic BP (ß = -0.397, p < .01) and diurnal change in diastolic BP (ß = -0.325, p < .05). CONCLUSIONS / IMPLICATIONS FOR PRACTICE: The findings of the present study suggest that healthcare professionals should routinely evaluate sleep quality and pain in SLE patients. Improving both the poor sleep and pain management of these patients is clinically important. Further studies of the association between pain management and diurnal BP variation are needed.

Adherence to guidelines in monitoring amiodarone-induced thyroid dysfunction.

RATIONALE, AIMS AND OBJECTIVES: Baseline thyroid function testing and regular follow-up of thyroid function under amiodarone usage was recommended by guidelines. Little is known about the status of amiodarone monitoring in real-world clinical care in Taiwan. The objective was to determine the rate of thyroid monitoring and to assess the clinical and physicians' characteristics associated with adequate monitoring in a tertiary referral centre for arrhythmia. METHODS: We reviewed the medical records of patients receiving amiodarone during the period 2008-2009 at Taipei Veterans General Hospital. The rate of baseline and follow-up thyroid function monitoring during amiodarone therapy were calculated. Factors associated with guideline adherence to monitoring were analysed. RESULTS: Among the 1319 enrolled cases, 36.4% (n = 480) underwent baseline thyroid function testing and 1.1% (n = 15) received measurement of anti-thyroid peroxidase antibody before amiodarone initiation. Regular follow up of thyroid function under amiodarone usage occurred in only 8.6% (n = 114) of cases. Baseline thyroid function was more likely to be present in patients of younger age (P < 0.001), female sex (P = 0.01), and in those who received amiodarone therapy from cardiologists (P < 0.001) or electrophysiologists (P < 0.001) with fewer years of service (P < 0.001). Upon multivariate analysis, only physicians' expertise (cardiologist versus non-cardiologist, OR = 5.67, 95% CI: 2.44-13.16) and years of service (OR = 0.97, 95% CI: 0.95-0.998) were significantly associated with adequate thyroid monitoring. CONCLUSIONS: The rate of thyroid monitoring with amiodarone therapy had been suboptimal. Strategies to enhance guideline adherence are needed.

Hepatitis B Virus Reactivation in Rheumatoid Arthritis Patients Undergoing Biologics Treatment.

Background: Immunosuppressants can induce hepatitis B virus (HBV) reactivation; however, informative data about the risk of different immunosuppressive regimens, including biologics, on HBV reactivation (HBVr) among patients with rheumatoid arthritis (RA) are incomplete. Methods: Among 2334 RA patients who had available hepatitis B surface antigen (HBsAg) data, 123 patients positive for HBsAg who were not receiving anti-HBV prophylaxis were enrolled. These patients were undergoing varied mono or combination immunosuppressive therapy, including 36 who were receiving biological disease-modifying antirheumatic drugs (bDMARDs). Results: During 3459 person-months of follow-up, 30 (24.4%) patients developed HBVr. The multivariate Cox proportional hazard models showed that glucocorticoid significantly increased the risk of HBVr. Among all kinds of immunosuppressive treatments, glucocorticoid in combination with bDMARDs and synthetic disease-modifying antirheumatic drugs (sDMARDs) had the highest risk of HBVr (adjusted hazard ratio [HR] = 5.14; 95% confidence interval [CI] = 1.77-14.92; P = .003). Rituximab had the greatest risk for HBVr (adjusted HR = 16.51; 95% CI = 1.82-149.67; P = .01) among the patients who received bDMARDs. Conclusions: Glucocorticoid has a detrimental effect on HBVr in RA patients. Antiviral prophylactic strategies should be justified according to the risk of HBVr under different combinations of immunosuppressive therapy in rheumatic patients.

Amiodarone-induced thyroid dysfunction in Taiwan: a retrospective cohort study.

BACKGROUND: The incidence and risk factors of amiodarone-induced thyroid dysfunction are variable in the literature. OBJECTIVE: The aim of this study was to investigate the clinical and biochemical features and risk factors of amiodarone-induced thyroid dysfunction in Taiwan. SETTING: This study was conducted at a tertiary referral center for arrhythmia. METHOD: Retrospective analysis of patients treated with amiodarone during the years 2008-2009 was performed. MAIN OUTCOME MEASURE: Incidence and risk factors of amiodarone-induced thyrotoxicosis (AIT) and amiodarone-induced hypothyroidism (AIH) were assessed. RESULTS: Of the 527 patients, 437 (82.9 %) remained euthyroid, 21 (4.0 %) developed AIT, and 69 (13.1 %) were affected with AIH. In univariate analysis, AIT was associated with younger age, and the risk factors for AIH included older age, higher baseline thyroid stimulating hormone (TSH) titer, lower baseline free T4 level, lower cumulative amiodarone dosage, and shorter amiodarone treatment duration. Cox regression analysis was performed to determine the different risk categories in the elderly population of age 65-74 (young-old), 75-84 (old-old), and ≥85 years old (oldest-old). Additionally increased risk of AIH was found in the groups of old-old (HR 2.09, 95 % CI 1.11-3.96) and oldest-old (HR 2.57, 95 % CI 1.21-4.75). In the multivariate analysis of risk factors for AIH, baseline TSH level (HR 1.38, 95 % CI 1.12-1.70) and cumulative amiodarone dosage (HR 0.95, 95 % CI 0.93-0.97) remained statistically significant. CONCLUSION: AIH was much more common than AIT in Taiwan, an area with sufficient iodine intake. Higher baseline TSH level was the predominant independent risk factor for the development of AIH.

Lung involvement in primary Sjögren's syndrome: Correlation between high-resolution computed tomography score and mortality.

BACKGROUND: Lung involvement is one of the major systemic manifestations of primary Sjögren's syndrome (pSS). This study aims to demonstrate the correlation between high-resolution computed tomography (HRCT), pulmonary function test (PFT) results, and outcome in these patients. METHODS: Forty-four pSS patients were enrolled and their PFT results and HRCT findings/scores were retrospectively investigated. RESULTS: All patients had reduced carbon monoxide-diffusing capacity (DLCO; <75% of the predicted value); <60% of the predicted value of peak expiratory flow (PEF), of forced vital capacity (FVC), and of forced expiratory volume in the 1st second (FEV1) were noted in 15 (34.1%) patients, 13 (29.5%) patients, and 12 (27.3%) patients, respectively. HRCT scores had a negative correlation with DLCO (r = -0.376, p = 0.012), but not with other PFT results. Twelve patients (27.3%) expired during a mean follow-up of 3.7 years; 11 (91.7%) patients died of respiratory failure in the lung-involved patients, of which three were present with pneumonia. The expired patients had lower predicted values of FEV1 (63.1 ± 19.4% vs. 79.0 ± 22.7%, p = 0.017), FVC (58.7 ± 20.4% vs. 77.1 ± 17.5%, p = 0.005), and PEF (54.3 ± 20.5% vs. 72.0 ± 24.8%, p = 0.035), and higher HRCT scores (9.2 ± 5.7 vs. 5.2 ± 3.5, p = 0.033) than those patients who survived. Patients with FEV1, FVC, PEF < 60% of the predicted value, or high HRCT score (13-18) presented shorter median overall survival (p = 0.005, p < 0.001, p = 0.021, p < 0.001, respectively). Multivariate analysis adjusted for PFT results showed that HRCT ≥13 was an independent risk factor for mortality (p = 0.007). CONCLUSION: The clinical outcome of pSS patients with lung involvement in Taiwan is not very favorable. Although HRCT score was poorly correlated with PFT, high HRCT score was significantly associated with higher mortality.

Clinical features and outcomes of posterior reversible encephalopathy syndrome in patients with systemic lupus erythematosus.

OBJECTIVE: To analyze the clinical features and outcomes of patients with posterior reversible encephalopathy syndrome(PRES), the risk factors of PRES-related intracranial hemorrhage (ICH), and all-cause mortality in patients with systemic lupus erythematosus (SLE). METHODS: Twenty-six episodes of PRES were identified in 23 SLE patients, using an electronic medical records database of 3,746 SLE patients. RESULTS: The prevalence of PRES was 0.69% among SLE patients. The scores of the SLE Disease Activity Index without neurologic descriptors (SLEDAI-N) were significantly elevated from baseline for a mean of 3.3 during PRES (P = 0.009). Rapidly deteriorating renal function, pulmonary hemorrhage, thrombotic microangiopathy, macrophage activation syndrome, or multiple organ dysfunction syndrome appeared during 65.4% of episodes. In 16 episodes, patients completely recovered from PRES-related symptoms within a median of 7 days. Visual impairment was reversed within 2 days in 8 of 15 patients, but impairment in other patients was protracted for up to 4 months, especially when ICH was present. Hypoalbuminemia (<20 gm/liter; odds ratio [OR] 30, 95% confidence interval [95% CI] 2.04­441.84) and thrombocytopenia (<30,000/mm(3); OR 21, 95% CI 1.27­346.93) were risk factors for PRES-related ICH. Patients with SLEDAI-N scores >18 during a PRES attack had significantly higher mortality rates than did patients with SLEDAI-N scores ≤18 (P = 0.009 by log rank test). CONCLUSION: PRES frequently occurs during active SLE with multiple complications. Hypoalbuminemia and thrombocytopenia may contribute to PRES-related ICH. The extra neurologic disease activity of lupus during PRES may influence the mortality rate of SLE patients.

Thrombotic microangiopathy in systemic lupus erythematosus: a cohort study in North Taiwan.

OBJECTIVES: Thrombotic microangiopathy (TMA) co-existing with SLE is rarely reported. This study aimed to investigate the triggering factors, clinical features and outcomes of SLE patients with TMA in Northern Taiwan. METHODS: Twenty-five TMA cases out of 2461 SLE patients admitted to Taipei Veterans General Hospital, between 2000 and 2010, were enrolled. RESULTS: When TMA occurred, 16 (64.0%) patients had infection; 22 (88.0%) were in an active disease state with a SLEDAI score >10. Among the infection group, 13 (81.3%) had an increase in the SLEDAI score of ≥ 4. We found that older age (≥ 50 years), low platelets (≤ 20,000/nm(3)), presence of infection, acute renal failure (ARF) or four or more TMA features were independent risk factors for persistent haematological abnormalities (P < 0.05); older age (≥ 50 years) and a high reticulocyte index (>2%) were the risk factors for persistent renal function impairment (P < 0.05). The overall mortality rate was 52.0% (13 out of 25); older age (≥ 40 years), low complement value, presence of infection (P < 0.001), two or more infection sources, ARF and four or more TMA features were the statistically significant factors contributing to a higher mortality rate. Patients receiving plasma exchange seven times or more had a significantly higher rate of improvement in renal function and haematological abnormalities. CONCLUSIONS: Our study showed that infection was one of the major triggers for the flare-up of SLE disease activity and occurrence of TMA in SLE. Infection is also a strong risk factor for outcome in SLE patients with TMA. Plasma exchange can be considered as an adjuvant treatment modality.

Systemic amyloidosis manifesting as a rare cause of hepatic failure.

In 1854, the term "amyloid" was first used in the description of a liver specimen at autopsy by Virchow. The kidneys and heart are the most commonly involved organs in amyloidosis; liver and gastrointestinal tract involvement is less common, and the symptoms are usually mild. Here, we report the case of a 57-year-old male patient who presented with oral hemorrhagic bullae, thrombocytopenia and jaundice. Disseminated intravascular coagulation profile was positive. Abdominal sonography showed ascites, and abdominal computed tomography disclosed heterogeneous enhancement of the liver, with focal low attenuation regions and splenomegaly with poor contrast enhancement. Liver decompensation was highly suspected. Diagnostic laparoscopy with liver biopsy and colonoscopic biopsy from the rectum were subsequently performed. Typical apple-green birefringence was demonstrated on polarized light microscopy by Congo red staining. Systemic amyloidosis was diagnosed and colchicine prescribed. However, liver function deteriorated and intermittent gastrointestinal bleeding was found during the patient's hospitalization. The patient died due to uncorrectable coagulopathy and massive gastrointestinal bleeding. The final diagnosis was idiopathic amyloidosis with hepatic failure. Although amyloidosis rarely presents with hepatic failure, it should be considered in patients with signs of liver decompensation. Clinicians should be aware of this rare but potentially lethal presentation and arrange appropriate treatment promptly.

Mycobacterium-associated lobular panniculitis, mimicking a rheumatoid nodule in a patient with rheumatoid arthritis.

Mycobacterium-associated lobular panniculitis can mimic a rheumatoid nodule and has been seldom reported in rheumatoid arthritis (RA). We describe a 69-year-old woman with RA who presented initially with fever and an indurated skin lesion on the right thigh. Lobular panniculitis was diagnosed after biopsy and was then treated with prednisolone. After this therapy, pulmonary infiltration developed and was later shown by transbronchial biopsy to be caused by Mycobacterium tuberculosis. The panniculitis skin lesion became smaller after prednisolone therapy and was further improved after antituberculosis drugs were added. Reexamination of the previously biopsied skin tissue disclosed acid-fast bacilli. Reactivation or new infection of M. tuberculosis is a current important issue in RA patients, especially after treatment with disease-modifying anti rheumatic drugs or antitumor necrosis factor agents. Mycobacterium-associated lobular panniculitis should be included in the differential diagnosis of indurated skin disorder in RA patients, and acid-fast staining or polymerase chain reaction examination of tuberculosis should be performed routinely on biopsied skin tissue.

Hemolytic uremic syndrome with ischemic glomerulonephropathy and obliterative vasculopathy in a systemic sclerosis patient treated with cyclosporine-A.

Hemolytic uremic syndrome (HUS) is not a commonly reported complication in post-transplantation patients treated with cyclosporine-A (CSA), and is extremely rare in systemic sclerosis (SSc) patients treated with this drug. CSA may contribute to the development of chronic ischemic glomerulonephropathy and vasculopathy, features not easily distinguished from SSc-related nephropathy. Here, we describe a 41-year-old Chinese man with diffuse-type SSc treated with CSA who developed thrombocytopenia, acute renal failure and hemolytic anemia and was diagnosed with HUS. Renal function and thrombocytopenia improved gradually after intensive treatment of plasma exchange (PE) and high-dose steroid therapy. After PE, renal biopsy showed ischemic glomerulonephropathy and obliterative vasculopathy. This case illustrates that PE can improve the hematological disorders and characteristic renal changes of HUS in SSc patients treated with CSA. However, this therapy may not be effective in normalizing serum creatinine level in SSc patients once CSA has triggered the normal kidney to develop glomerulonephropathy and vasculopathy with ischemic and sclerotic changes.

Behcet's disease with uveitis in Taiwan.

BACKGROUND: Behcet's disease is a multisystemic inflammatory disease which may lead to blindness. The purpose of this study was to describe and compare the clinical features and visual outcome of Behcet's uveitis in Taiwan. METHODS: The medical charts of 227 consecutive patients (414 eyes) with Behcet's uveitis who were seen in the Uveitis Clinic of Taipei Veterans General Hospital were reviewed: 96 patients (group A) between 1984 and 1993 and 131 (group B) between 1994 and 2003. RESULTS: The male-to-female ratio was 1.6. The mean age at the time of uveitis onset was 30.8 years. Males were most affected in the 3rd and 4th decades and females in the 2nd to 5th decades. There were more skin and gastrointestinal lesions in males, and less skin and genital lesions in group B. HLA-B51 antigen was found in 35.7% of patients. Treatment with cyclosporin, azathioprine and methotrexate was more frequent in group B. Uveitis occurred bilaterally in 83.9% of patients. At the first visit, potential visual acuity (VA) was 0.1 or less in 24.7% of eyes in males and 11.4% in females. The mean time from presentation to loss of useful vision (> 0.1) was 51 months in 19.1% of eyes. Kaplan-Meier survival analysis estimated the risks of losing useful vision at 5, 10 and 15 years as being 22.6%, 43.0% and 58.5%, respectively. VA at the first and last visits were better and VA deterioration slower in group B. CONCLUSION: Visual outcome is poorer in males than in females and has improved in the recent decade, but still a significant proportion of patients lose their vision quickly. The number of new patients has not decreased.

[Variation of diversity and activity of ammonia-oxidizing bacteria community in the integrated vertical-flow constructed wetlands].

The activity of ammonia-oxidizing bacteria (AOB) in different layers of Integrated vertical-flow constructed wetlands (IVCW) treating eutrophic scenic water was measured, and the diversity and spatial distribution of AOB community structure in IVCW was investigated using PCR-DGGE. The results indicated that because of the integrated influence of competition of plant rhizodeposition, heterotrophic bacteria, DO and ammonia concentration, there were significant spatial differences in the activity and diversity of AOB along the flow direction of wetland. The activity of AOB was 0.79 mg x (kg x h)(-1) (in NO3- -N, the same below) near the surface of down-flow system in IVCW. From the surface of down-flow system to up-flow system, the activity of AOB decreased gradually, and slightly increased to 0.17 mg x (kg x h)(-1) near the surface of up-flow system. The spatial variation of diversity of AOB showed the similar change trend with the activity in IVCW and the diversity index in down-flow system (1.92) were higher than those in up-flow system (1.65). Most of AOB belong to oligotrophic bacterium in IVCW, and the population of ammonia-oxidizing bacteria showed a higher percentage of Nitrosomonas-like sequences from the wetland samples. Uncultured beta proteobacterium, Comamonas sp., Nitrosomonas oligotropha were also detected. The variation of the AOB community demonstrated spatial pattern in IVCW, which might be related to different wetland environment.