Clinical correlation of brachial artery flow-mediated dilation in patients with systemic sclerosis

Abstract

Objective To investigate the clinical significance of flow-mediated dilation (FMD) in systemic sclerosis (SSc).

Methods Thirty-three SSc patients and 12 healthy controls were studied. Ultrasound assessment of the brachial artery FMD was performed on all subjects. The results were expressed as the percentage of increase in brachial artery diameter following hyperemia.

Results Limited cutaneous SSc (lcSSc) patients had significantly lower FMD values than healthy controls (5.3 ± 2.7 versus 7.7 ± 2.0 %, p < 0.05), while the values in diffuse cutaneous SSc (dcSSc) patients (6.7 ± 4.0 %) were comparable to those in lcSSc patients and healthy controls. Although FMD values did not correlate with any clinical features in dcSSc patients, there was an inverse correlation between FMD values and disease duration in lcSSc patients (r = ?0.64, p < 0.05). Furthermore, lcSSc patients with decreased FMD values showed significantly higher prevalence of digital ulcers and elevated right ventricular systolic pressure than those with normal values (for each; 75 versus 10 %, p < 0.05).

Conclusion The FMD values represent the severity of vascular damages, which progress along with disease duration and lead to digital ulcers and pulmonary arterial hypertension, in lcSSc patients.     

Serum L-ficolin levels in patients with systemic lupus erythematosus

Abstract

Objective L-ficolin plays an important role in innate immunity and is involved in apoptosis. The objective of this study was to investigate the relationship between serum L-ficolin levels and clinical manifestations in patients with systemic lupus erythematosus (SLE).

Methods Serum L-ficolin levels were determined by enzyme-linked immunosorbent assay in 66 SLE patients and 50 healthy controls.

Results Median serum L-ficolin levels were 5.0 and 8.7 μg/ml in SLE patients and controls, respectively (p = 0.0001). There were no significant differences in serum L-ficolin levels between the active disease group [SLE Disease Activity Index (SLEDAI) > 6] and the inactive disease group (SLEDAI < 5). Decreased serum L-ficolin levels were associated with thrombocytopenia (median of with vs. without thrombocytopenia 3.4 vs. 5.3 μg/ml, p = 0.008). There were no correlations between serum L-ficolin levels and SLEDAI, serum C3, or serum C4 levels.

Conclusion The association between L-ficolin and thrombocytopenia suggests a pathogenic role for L-ficolin in thrombocytopenia in SLE.     

Sexual dysfunction and physical performance in female systemic lupus erythematosus patients

Aim of the workTo investigate the sexual dysfunction in systemic lupus erythematosus (SLE) patients and its relation to physical fitness and other disease parameters.Patients and methodsThe study included 47 SLE females and 47 matched healthy controls. SLE disease activity index (SLEDAI) and systemic lupus international collaborative clinics damage index (SLICC DI) were assessed. Physical performance of the patients was assessed by the grip ability test (GAT), gait velocity test, Jebsen hand function test, fatigue severity scale (FSS) and the modified health assessment questionnaire (mHAQ). Sexual dysfunction of the patients was assessed by the sexual function index (FSFI). Depressive disorders of the patients were assessed by Beck depression inventory.ResultsThe mean age of the patients was 28.7 ± 7.2 years and disease duration 4.4 ± 2.9 years. GAT was significantly lower (43.65 vs 70.00; p = 0.001), and FSS higher (6.5 vs 1; p = 0.001) in patients compared to control. The total FSFI was significantly lower (23.41 vs 29.60; p = 0.001) and all domains scores were significantly lower except the pain domain (p = 0.001). Beck depression inventory score was higher in patients (13.29 vs 12.74; p = 0.365). SLE females with prescribed corticosteroids and azathioprine had significantly lower FSFI as compared to those without while FSFI score was higher in those receiving hydroxychloroquine. The FSFI significantly correlated with C4 level (r = 0.328, p = 0.024 and inversely with, SLEDAI (r = ?0.07, p = 0.001), FSS (r = ?0.54, p = 0.001), mHAQ (?0.37, p = 0.01) and with Beck depression inventory (r = ?0.57, p = 0.001).ConclusionMarried female patients with SLE revealed a higher degree of sexual dysfunction of all domains except pain.     

Clinical manifestations and clinical syndromes of Filipino patients with systemic lupus erythematosus

The aim of this study was to describe the presenting clinical manifestations and syndromes of Filipino patients on diagnosis of systemic lupus erythematosus (SLE). We performed a retrospective review of medical records of Filipino SLE patients included in the lupus database of the University of Santo Tomas (UST) in Manila, Philippines. All patients fulfilled the American College of Rheumatology criteria for SLE. The following data were recorded: (1) demographic profile, (2) clinical manifestations on SLE diagnosis, and (3) clinical syndromes prior to and during fulfillment of diagnostic criteria for SLE and disease interval from diagnosis of a clinical syndrome to SLE diagnosis. Clinical data of 1,070 patients entered into the UST lupus database as of October 2005 were analyzed. The average age at SLE diagnosis was 28.5 ± 11.5 (range 5–71) years, with 1,025 female and 45 male subjects. The most common presenting manifestation was arthritis (68%), followed by malar rash (49%), renal involvement (47%), photosensitivity (33%), and oral ulcers (33%). The following clinical syndromes were recorded prior to or during SLE diagnosis: nephrotic syndrome (30%), undifferentiated connective tissue disease (UCTD) (22%), autoimmune hemolytic anemia (AIHA) (6%), and idiopathic thrombocytopenic purpura (ITP) (6%). Among these, AIHA preceded the diagnosis of SLE at the longest interval (20.3 ± 30.6, range 1–194 months). In this large database of Filipino patients with SLE, the most common presenting manifestation was arthritis, with renal involvement occurring in almost 50%. Among the clinical syndromes, nephrotic syndrome was the most common, whereas AIHA recorded the longest interval preceding SLE diagnosis, at an average of 20.3 months. Our findings are similar to data from other countries and emphasize the broad range of manifestations of SLE. The findings also reinforce the need to establish and maintain SLE databases to enhance awareness, early diagnosis, and more efficient management of the disease.     

Survival in patients with systemic lupus erythematosus in India

Summary Survival in patients with systemic lupus erythematosus (SLE) in developed countries has improved considerably over the last 2 decades. In India, however, survival in patients with SLE reported 5 years ago from this tertiary referral centre was very poor. The present study was conducted to examine trends, if any, towards improvement in the survival of patients with SLE attending the same centre during the period 1981–1990. No statistically significant change in survival was noted. It appears that improvement in the survival of SLE patients would require an overall improvement in the standard of medical care in India.     

Clinical manifestations and clinical syndromes of Filipino patients with systemic lupus erythematosus

Abstract

The aim of this study was to describe the presenting clinical manifestations and syndromes of Filipino patients on diagnosis of systemic lupus erythematosus (SLE). We performed a retrospective review of medical records of Filipino SLE patients included in the lupus database of the University of Santo Tomas (UST) in Manila, Philippines. All patients fulfilled the American College of Rheumatology criteria for SLE. The following data were recorded: (1) demographic profile, (2) clinical manifestations on SLE diagnosis, and (3) clinical syndromes prior to and during fulfillment of diagnostic criteria for SLE and disease interval from diagnosis of a clinical syndrome to SLE diagnosis. Clinical data of 1,070 patients entered into the UST lupus database as of October 2005 were analyzed. The average age at SLE diagnosis was 28.5 ± 11.5 (range 5–71) years, with 1,025 female and 45 male subjects. The most common presenting manifestation was arthritis (68%), followed by malar rash (49%), renal involvement (47%), photosensitivity (33%), and oral ulcers (33%). The following clinical syndromes were recorded prior to or during SLE diagnosis: nephrotic syndrome (30%), undifferentiated connective tissue disease (UCTD) (22%), autoimmune hemolytic anemia (AIHA) (6%), and idiopathic thrombocytopenic purpura (ITP) (6%). Among these, AIHA preceded the diagnosis of SLE at the longest interval (20.3 ± 30.6, range 1–194 months). In this large database of Filipino patients with SLE, the most common presenting manifestation was arthritis, with renal involvement occurring in almost 50%. Among the clinical syndromes, nephrotic syndrome was the most common, whereas AIHA recorded the longest interval preceding SLE diagnosis, at an average of 20.3 months. Our findings are similar to data from other countries and emphasize the broad range of manifestations of SLE. The findings also reinforce the need to establish and maintain SLE databases to enhance awareness, early diagnosis, and more efficient management of the disease.     

核素心室造影评价系统性红斑狼疮病人的心室功能

应用核素心室造影评价35例系统性红斑狼疮（SLE）病人亚临床心脏受累期心功能的变化。结果显示，SLE病人左室收缩功能参数如左室射血分数、峰射血率、前1／3射血分数及峰射血时间均无显著性改变；活动期和非活动期病人左室舒张功能参数如峰充盈率、前1／3充盈分数明显降低，活动期更明显；62．5％的活动期和36．4％的非活动期病人前1／3充盈分数降低（＜20％）；活动期病人左室相角程标准差增大，收缩同步性减低；活动期和非活动期病人右室功能均降低。提示SLE病人即使左室收缩功能正常也存在左室舒张功能和右室功能障碍。     

Prevalence and classification of headache in patients with systemic lupus erythematosus

Studies on the prevalence of headache in systemic lupus erythematosus (SLE) have shown that it varies from 32 to 78%. The purpose of our study was to determine the prevalence and characteristics of headache in SLE compared with patients with different types of diffuse connective tissue diseases (DCTD) and its relationship with clinical and laboratory manifestations of SLE. We studied patients with SLE (SLE group) and patients with DCTD (control group). All patients were made to answer questionnaire to assess the presence of headache, characterized by at least five episodes of headache during the last year, which was classified according to the International Headache Society criteria. A total of 207 patients were studied, 115 in SLE group and 92 in the control group. The 1-year prevalence of headache was 75.7% in SLE group and 66% in the control group. When the groups were analyzed, 66.1% met the diagnostic criteria for migraine in the SLE group compared with 52.2% in the control group (p=0.04) and 13.9% for tension-type headache in SLE group compared with 16.3% in the control group. The former was the only variable that reached statistical significance comparing the two groups. Both headache and migraine were associated with Raynaud’s phenomenon in SLE patients (odds ratio of 2.80, 95% confidence interval: 1.11–7.05, p=0.02 and odds ratio of 2.34, 95% confidence interval: 1.04–5.23, p=0.03, respectively). These results suggest that headache is a common manifestation in SLE and in other DCTD and we cannot exclude the possibility that it may be related to the emotional stress induced by such clinical situations.     

Anorexia nervosa in a patient with systemic lupus erythematosus

Summary Anorexia nervosa has not been described in patients with systemic lupus erythematosus (SLE). The case of a patient with SLE who developed the classical features of anorexia nervosa is described. Although an impressive interplay between psychosocial and cultural factors was present, the possibility that this syndrome was produced by active CNS vasculitis remained.     

Clusters of clinical and immunologic features in systemic lupus erythematosus: analysis of 600 patients from a single center

OBJECTIVE: To analyze the prevalence and characteristics of the main clinical, hematologic, and immunologic manifestations of systemic lupus erythematosus (SLE) in a cohort of 600 consecutive patients from a single center, and to determine the specific characteristics of organ involvement in a homogeneous SLE population. METHODS: Patients were consecutively seen in our department either as inpatients or outpatients between 1980 and 2001. All had documented medical histories and underwent a medical interview as well as a routine general physical examination. Clinical and serologic characteristics of all patients were consecutively collected in a protocol form. RESULTS: The final cohort (survival cohort) consisted of 533 (89%) women and 67 (11%) men (female to male ratio, 8:1), with an average of 29 new patients per year. Mean age at onset of symptoms attributable to the disease was 31 years (range, 5 to 84 years) and mean age at the time of diagnosis of SLE was 33 years (range, 6 to 85 years). The most frequent SLE involvement was articular involvement, found in 498 patients (83%), followed by hematologic involvement in 451 patients (75%), specific SLE cutaneous involvement in 354 patients (59%), constitutional features in 252 patients (42%), and nephropathy in 203 patients (34%). Patients enrolled in the protocol before 1991 had a higher frequency of central nervous system (CNS) involvement (27% vs 10%, P <.001), thrombotic events (17% vs 9%, P =.003), and abnormal hematologic parameters (85% vs 66%, P <.01), but a lower frequency of articular involvement (79% vs 86%, P =.038) than those enrolled after 1991. The following were observed associations: specific SLE cutaneous involvement was associated with anti-Sm antibodies; renal involvement with hemolytic anemia and anti-double-stranded DNA antibodies; CNS involvement with thrombocytopenia and immunoglobulin G-anticardiolipin; thrombotic events with low total hemolytic complement, immunoglobulin G-anticardiolipin, and lupus anticoagulant; and myositis with anemia and anti-ribonucleoprotein antibodies. CONCLUSION: This large study, performed in a single center, has shown cluster associations between certain clinical, hematologic, and immunologic features of SLE, reflecting specific patterns of disease expression. The accurate evaluation of clinical features and laboratory markers at disease diagnosis and during evolution may improve the clinical treatment of SLE patients.     

Antinuclear antibodies measured by enzyme immunoassay in patients with systemic lupus erythematosus: relation to disease activity

To evaluate the correlation between measurements of antinuclear antibodies serum levels by enzyme immunoassay (ANA-EIA), and the degree of systemic lupus erythematosus disease activity. To retest the performance of the test compared to measurement of antinuclear antibodies by immunofluorescence (ANA-IIF). Eighty-five sera from 71 patients with SLE were tested. Demographic, clinical, laboratory, and SLEDAI status were collected. The sera were tested for ANA-EIA and by ANA-IIF at 1:40 and 1:160 dilutions. Serum levels of ANA-EIA were compared to the overall SLEDAI score and to each of its components. A SLEDAI score of ≥6 was considered clinically significant. The sera of fifty-one healthy volunteers served as controls. Serum levels of ANA-EIA were significantly higher in patients with a SLEDAI score of ≥6 compared to the group of patients with a SLEDAI score of <6 (P = 0.004). High serum levels of ANA-EIA correlated significantly with elevated anti DS-DNA antibodies (P < 0.001), low C₃ or C₄ levels (P < 0.001), pyuria (P < 0.011), arthritis (P = 0.019), and new rash (P = 0.019). Levels of ANA-EIA were significantly higher in patients tested positive by IIF compared to those who tested negative. Higher serum levels of ANA-EIA correlated with clinically significant disease activity in patients with SLE. Higher serum levels of ANA-EIA also correlated with some single items of the SLEDAI. The results also reiterated the validity of ANA-EIA testing in patients with SLE. Further longitudinal studies are needed in order to test the hypothesis that serum ANA-EIA levels might reflect fluctuations in disease activity.     

Audiologic evaluation in systemic lupus erythematosus patients and impact of hydroxychloroquine therapy

Aim of the work: To evaluate the hearing function in systemic lupus erythematosus (SLE) patients and assess the impact of chronic use of hydroxychloroquine (HCQ). Patients and methods: This study was conducted on 48 SLE patients and 12 matched controls. The SLE disease activity index (SLEDAI) was assessed. Otological manifestations and extended high frequency audiometry were assessed. SLE patients were divided into those receiving HCQ (n = 36) and those not (n = 12). Results: The mean age of the patients was 39.5 ± 9.7 years, 40 females and 8 males, median disease duration was 7 (1.5–20) years. The median (interquartile range) SLEDAI was 4 (2.75). The median daily dose of those receiving HCQ was 400 mg. There was a significant difference regarding patients reported otological manifestations such as tinnitus (p = 0.02), vertigo (p = 0.002) and hearing impairment (p = 0.04) compared to controls but not for deafness or ear buzzing in one or both ears. There was a significant decrease in the hearing threshold frequencies between patients and controls (p < 0.001). Those receiving HCQ showed more hearing impairment at frequency 9000 and 20,000 Hz than those not (p = 0.004, p < 0.001 respectively). The otological manifestations were comparable between those receiving HCQ and those not. There was no difference between those receiving 400 mg/day (n = 31) HCQ and those receiving 200 mg/day (n = 5) Conclusion: Otological symptoms and sensorineural hearing loss are frequent among SLE patients. Chronic administration of HCQ may have an ototoxic effect.     

Interstitial cystitis and systemic lupus erythematosus in a 20-year-old woman

A 20-year-old woman with systemic lupus erythematosus presented with dysuria, urinary frequency and suprapubic pain and was found to have a chronic interstitial cystitis, a chronic inflammation of the bladder wall, mostly affecting middle-aged women. Chronic interstitial cystitis is an uncommon manifestation of systemic lupus erythematosus.     

Coeliac disease in systemic lupus erythematosus: a case report

The case of a rare coexistence of systemic lupus erythematosus (SLE) with coeliac disease (CD) is described. Systemic lupus erythematosus was diagnosed prior to CD and initially SLE treatment was administered. After several years, when CD symptoms developed, the diagnosis was corrected and additional treatment with a gluten-free diet was applied with beneficial effects.     

Hemorheological parameters are related to subclinical atherosclerosis in systemic lupus erythematosus and rheumatoid arthritis patients

Objectives

Rheological characteristics of blood are strongly linked to atherothrombosis in the general population, but its contribution to atherosclerosis in systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) is currently unclear. This work examines the relationship between blood rheology, traditional cardiovascular (CV) risk factors, inflammation and subclinical atherosclerosis in SLE and RA.

Methods

Whole blood viscosity (WBV), plasma viscosity (PV), erythrocyte deformability (ED), aggregation (EA) and erythrocyte NO production were measured in 197 patients (96 SLE and 101 RA) and compared to 97 controls, all females without previous CV events. Clinical information was obtained and fasting lipids and acute phase reactants were measured. The relationship between hemorheological parameters, CV risk factors and inflammation was assessed in patients and the impact of these variables on carotid intima-media thickness (cIMT) was evaluated in univariate followed by multivariate regression analyses.

Results

WBV and ED are significantly lower in patients, while EA is elevated as compared with controls. Hemorheological disturbances correlate with CV risk factors and markers of inflammation and are more profound in patients with metabolic syndrome. Multivariable analysis showed that menopause (OR 34.72, 95%CI 4.44–271.77), obesity (OR 4.09, 95%CI 1.00–16.68) and WBV (OR 3.98; 95%CI 1.23–12.83) are positively associated whereas current corticosteroid dose (OR 0.87; 95%CI 0.78–0.98), and erythrocyte NO production (OR 0.16; 95%CI 0.05–0.52) are negatively associated with cIMT.

Conclusion

Disturbed hemorheological parameters in SLE and RA women are related to the presence of CV risk factors and inflammation. WBV and erythrocyte NO are independently associated with the early stages of atherosclerosis.     

Treatment adherence in patients with rheumatoid arthritis and systemic lupus erythematosus

This study assessed self-reported adherence in patients with rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) from underserved healthcare settings. We conducted a cross-sectional survey of 102 ethnically diverse patients--70 with RA and 32 with SLE--attending rheumatology clinics at publicly funded hospitals in Houston, Texas; 43% were Hispanic, 32% African-American, and 25% White. Treatment adherence was evaluated using the compliance questionnaire rheumatology (CQR; 0, low adherence and 100, high adherence) and the questionnaire of the Adult AIDS Clinical Trials Group (AACTG). The patients were also asked how often they forgot to take their prescribed medications or discontinued them on their own. Mean patient age was 48.5 years; 75% were female, 32% were African-American, 43% Hispanic, and 25% White. Only one third reported never forgetting to take their medications; 40% reported having stopped their medications on their own because of side effects, and 20% because of lack of efficacy. Mean CQR score was 69.1 +/- 10.5, suggesting moderate adherence overall. Differences were also observed across ethnic groups: 23% of ethnic minority patients had problems taking their medications at specified times compared to 11% of Whites (p = 0.03). Lower education and side effects were associated with lower adherence. No differences were observed between RA and SLE patients. Many patients with RA and SLE report problems with treatment adherence. These appear to be more prevalent in African Americans and Hispanics than Whites; the impact of decreased adherence on outcomes could be significant and should be considered when treating patients with RA and SLE.     

Pilot clinical study of Adacolumn cytapheresis in patients with systemic lupus erythematosus

The aim of this study is to investigate the clinical effects of cytapheresis using the Adacolumn system (selective removal of circulating monocytes and granulocytes by means of an extracorporeal type column) in patients with active systemic lupus erythematosus (SLE). An open uncontrolled multicenter pilot study was conducted in 18 SLE patients who were showing a SLEDAI score of 8 or more under conventional medication. Patients with lupus nephritis (>class 1, WHO classification) were excluded. Extracorporeal cytapheresis with the Adacolumn system was administered once a week for five consecutive weeks. The efficacy of the treatment was evaluated using the SLEDAI for 10 weeks after the first cytapheresis session. The median SLEDAI decreased from 16 at baseline to six at week 11 (10 weeks after the first apheresis) (p<0.001). Significant improvements in musculoskeletal and dermal systems were observed. Arthritis and alopecia were present in 14 and nine patients at baseline and this number decreased to five and one patients, respectively by week 11.Three mild and one moderate adverse events out of the 42 reported events were judged ‘probably related’ to the treatment; no serious adverse events were reported. Selective removal of monocytes and granulocytes from the blood in an extracorporeal circulation system was associated with clinical improvement in this small series of patients with SLE. Since this approach seems not to have the disadvantages of pharmacological immunosuppression, further controlled studies of Adacolumn cytapheresis are warranted in SLE.     

Nailfold videocapillaroscopy in patients with systemic lupus erythematosus

Nailfold videocapillaroscopy was performed in 21 controls (C) and 21 patients (P) with systemic lupus erythematosus (SLE) classified according to the American College of Rheumatology, with, at least, 1 year of diagnosed disease and having low activity (MEX-SLEDAI) and sequel (SLICC) indexes at the time of the examination, paired by sex and age. Red blood cell velocity (RBCV, mm/s) at rest and after the release of 60s arterial occlusion (RBCV_max, mm/s), time to reach it (TRBCV_max, s), functional capillary density (FCD, number of capillaries /mm²), afferent, apical and efferent capillary diameters (μm) (DAF, DAP and DEF, respectively) were obtained from videotapes analyzed by the CapImage software. The results did not show any significant difference between the groups that were analyzed, suggesting that morphological (capillary diameters) and functional (RBCV, RBCV_max, TRBCV_max and FCD) parameters are not affected by SLE when low activity and sequel indexes of the disease are present.     

Brachial artery flow-mediated dilation in patients with Tako-tsubo cardiomyopathy

Background

Tako-Tsubo cardiomyopathy (TTC) is a heart syndrome associated with transient myocardial contractile dysfunction. The pathogenesis of TTC remains unclear. The purpose of this study was to investigate brachial artery flow-mediated dilation (FMD) in patients with TTC.

Methods

The results of FMD tests of 4 women with TTC were compared with the results from 18 women with ST-elevation acute myocardial infarction (STEMI) and from 26 healthy female volunteers. FMD tests in all patients were performed within 24 hours of admission and again at 1-3 weeks as a follow-up.

Results

The FMD levels were significantly lower at the acute phase in patients with TTC than in patients with STEMI and in healthy volunteers (P <.01). After 1-3 weeks, the FMD test results of patients with TTC had greatly increased, and no significant differences were found between these results and the results from patients with STEMI (P >.05). Also, there were no significant differences in the FMD test results between the group of patients with TTC and the group of healthy volunteers (P >.05).

Conclusions

There is a pronounced and reversible endothelial dysfunction in patients with TTC, which can impair myocardial perfusion.