Hsiao-Yi Lin

Differences Between Juvenile-onset Ankylosing Spondylitis and Adult-onset Ankylosing Spondylitis

Background: Ankylosing spondylitis (AS) is a chronic inflammatory disease, which involves the spine, peripheral joints and entheses. Juvenile‐onset ankylosing spondylitis (JAS) affects children under the age of 16 years. JAS has been noted to present as clinical courses different from those of adult‐onset ankylosing spondylitis (AAS). Therefore, the purpose of the present study was to compare the possible risk factors, clinical manifestations, laboratory markers, radiological changes, and functional outcome between these 2 patient groups. Methods: AS patients were enrolled from the rheumatologic clinic of a tertiary medical center from January 1 to June 30 in 2006. The demographic data, clinical symptoms/signs, Bath AS indices, HLA‐B27, inflammatory markers, radiological findings, and treatment history were acquired with questionnaires, clinical evaluation, and chart review. The differences between JAS and AAS patients were evaluated and analyzed. Results: A total of 169 patients (142 males, 27 females) were included, comprising 47 JAS and 122 AAS patients. The ages of onset were 12.8 ± 2.7 years and 25.0 ± 7.4 years for JAS and AAS, respectively. They had similar gender distribution, years of delay to diagnosis and disease duration. A substantial proportion of our patients (40.4% of JAS and 34.4% of AAS) had physical trauma in the 1 month before disease onset. Also, 22.7% of JAS patients had intense physical training, while 25.2% of AAS patients did heavy work during the period. The first manifestation of JAS was mainly peripheral enthesopathy or arthritis, but axial symptoms in most AAS. More JAS patients had peripheral enthesopathies and arthritis on any occasion. Although there was a trend of higher score in Bath AS Disease Activity Index (BASDAI), Bath AS Metrology Index (BASMI) and Physicians Global Assessment (PGA) score, JAS patients had a comparable Bath AS Functional Index (BASFI) and Bath AS Patients Global Assessment (BAS‐G) as AAS patients. As to the laboratory and radiological tests, JAS patients had higher levels of C‐reactive protein and erythrocyte sedimentation rate, and more radiographic changes of hip joints. Conclusion: JAS and AAS patients had distinct presentations. JAS presented more peripheral enthesopathies and arthritis at disease onset and at any time of the course. If treated effectively, JAS will not lead to a worse functional outcome than AAS. Therefore, it is mandatory to diagnose and treat JAS as early as possible.

Renal Function in Gout Patients

Patients with gouty arthritis were examined at Veterans General Hospital to evaluate whether their renal function is impaired and to define the factor(s), if any, of renal function deterioration. A total of 152 cases were included in the study, and the patients were divided into two groups. One group (n = 80) exhibited pure gout without any associated medical problems or preexisting renal disorders. The second group (n = 72) included patients with gout and hypertension. The group with pure gout was further stratified into patients with tophi (n = 21) and those without (n = 59). Seventy-two sex- and age-matched normal adults served as the control group. We found (1) that the renal function was impaired in the pure-gout group when compared with sex- and age-matched normal individuals (serum creatinine 1.56 +/- 0.64 vs. 0.90 +/- 0.16 mg/dl, p = 0.0001; creatinine clearance 59.91 +/- 30.90 vs. 97.10 +/- 27.19 ml/min, p = 0.0001); (2) that the renal function was significantly more aggravated in patients with clinically visible tophi than in those without (gout with tophi vs. gout without tophi: serum creatinine 1.89 +/- 0.90 vs. 1.44 +/- 0.48 mg/dl, p = 0.040; creatinine clearance 47.27 +/- 31.90 vs. 64.40 +/- 29.53 ml/min, p = 0.030), and (3) that a further significant decline of the renal function was noted in gouty patients with an associated medical illness, i.e., hypertension (gout with hypertension vs. pure gout: serum creatinine 2.10 +/- 0.97 vs. 1.56 +/- 0.64 mg/dl, p = 0.0001; creatinine clearance 45.06 +/- 24.69 vs. 59.91 +/- 30.90 ml/min, p = 0.0029).(ABSTRACT TRUNCATED AT 250 WORDS)

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.

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.

Onset of Systemic Lupus Erythematosus During Pregnancy

When systemic lupus erythematosus (SLE) is first suspected during pregnancy, though rare, the diagnostic criteria are not different from those for nonpregnant women. The pregnancy outcome is good if treatment with adequate immunosuppressive agents starts as soon as the diagnosis is made. There are 4 cases in this report who had SLE onset during pregnancy. Although 2 of them suffered from preeclampsia, all 4 pregnancies resulted in favorable outcomes after the lupus was controlled by medical treatment.

Ataxic sensory neuronopathy in a patient with systemic lupus erythematosus

The occurrence of ataxic sensory neuronopathy (ASN) is rare in patients with connective tissue diseases (CTDs). ASN has been described in case reports and case series in patients with CTDs, mostly Sjögren’s syndrome, and most often occurring during middle or old age. ASN in association with systemic lupus erythematosus (SLE) is extremely rare; there has been only one reported case in the literature. In addition, to our knowledge, adolescent onset of symptoms in CTD-associated ASN has not been reported previously. We report the case of a young woman who presented with ASN, characterized by sensory ataxia, with elevated antinuclear antibodies, leukopenia and anemia; she fulfilled the diagnostic criteria for SLE about 7 years after the onset of sensory ataxia. Our case points out that ASN may be the initial presenting feature of SLE. SLE should be included in the differential diagnosis of ASN, especially in patients of young age.

Acute respiratory distress syndrome in a pregnant woman with systemic lupus erythematosus: a case report

When the disease activity of systemic lupus erythematosus (SLE) is controlled appropriately, a pregnant woman who has lupus is able to carry safely to term and deliver a healthy infant. While the physiology of a healthy pregnancy itself influences ventilatory function, acute pulmonary distress may decrease oxygenation and influence both mother and fetus. Though respiratory failure in pregnancy is relatively rare, it remains one of the leading conditions requiring intensive care unit admission in pregnancy and carries a high risk of maternal and fetal morbidity and mortality, not to mention the complexity caused by lupus flare. We report a case of SLE complicated with lupus pneumonitis and followed by acute respiratory distress during pregnancy. Though there is a high risk of maternal and fetal morbidity and mortality, maternal respiratory function improved after cesarean section and treatment of the underlying causes. The newborn had an extremely low birth weight but was well at discharge.

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 Sjögrens 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.

Relationship between The Level Of Anti-Double Strand DNA Antibody And Lupus Renal Diseases In Patients With Systemic Lupus Erythematosus

Objective: The goal of this study was to investigate the development of acute lupus nephritis or end stage renal disease in patients who had persistently high or variant levels of anti-dsDNA. Methods: Patients who fulfilled the diagnostic criteria for SLE were placed into one of three groups: persistent high levels, variant levels, and persistent normal levels of anti-dsDNA. Clinical renal diseases were based on the present of acute lupus nephritis, and the development of end stage renal disease (ESRD). Results: A total of 257 patients were enrolled in the study. Among them, 86 patients never have an elevation of anti-dsDNA during the follow-up period, 46 patients had variant levels of anti-dsDNA, and 125 patients had persistently high levels of anti-dsDNA. We compared the lupus nephritis flares and ESRD between normal anti-dsDNA group with high anti-dsDNA group or variant anti-dsDNA group, and the results showed significantly statistics different in acute lupus nephritis between the comparisons (p＜ 0.001, p＜0.001 respectively). However, there were no statistics different in ESRD (p=0.539, p=0.061 respectively). Conclusion: Patients who consistently showed a high anti-dsDNA level would have higher rate of acute lupus nephritis. Besides, if their serum elevated level of anti-dsDNA could not back and consistently within normal range, these patients also have less favorable renal outcome. Therefore, more intensive follow up and aggressive medical treatments are needed for these patients.

Arthropathy and Tendinopathy in Patients with Tophaceous and Non-tophaceous Gout: A Case-controlled Ultrasonographic Study

Objective: To determine the characteristics of tendon and joint involvement in patients with tophaceous and non-tophaceous gout on ultrasonography (US). Method: We performed a prospective, observational, case-controlled study using US to evaluate the knee, ankle, 1st and 2nd metatarsal-phalangeal (MTP) joints, and the tendons and entheses of the lower limbs. Differences were analyzed by Fisher’s exact or Mann-Whitney U-tests. Results: Twenty-four patients with tophaceous gout and 36 subjects with nontophaceous gout were recruited. Double contour signs (DCS) of the femoral condyles were significantly more prevalent in the tophaceous gout group compared to the non-tophaceous group (50% vs. 15.3%; p=0.001). The 1st and 2nd MTP joints of patients with tophaceous gout had significantly more DCS, periarticular tophi, and bone erosions than patients with non-tophaceous gout (p≤0.002). Intratendinous tophi in the Achilles tendons (43.8% vs. 6.9%), proximal/distal patella tendons (29.2%/41.7% vs. 1.4%/8.3%), tibialis anterior/posterior tendons (14.6%/39.6% vs. 0/1.4%), and peroneus longus and brevis tendons (29.2% vs. 0) were significantly more frequent in patients with tophaceous gout than patients with non-tophaceous gout (p≤0.002). Enthesopathies were commonly observed; specifically 7.5% of proximal patella tendons, 20% of distal patella tendons, and 13.3% of Achilles tendons presented with enthesopathies. No statistical differences existed between groups. Conclusion: Patients with tophaceous gout had significantly more prevalent intra- and extra-articular pathology on US, suggesting a larger quantity of monosodium urate depositions, than patients with non-tophaceous gout. We thus recommend a more aggressive and longer duration of urate-lowering therapy in patients with tophaceous gout.