In-Woon Baek

Thrombotic risk in patients with immune thrombocytopenia and its association with antiphospholipid antibodies

Patients with immune thrombocytopenia (ITP) paradoxically have an increased risk of thrombosis. The presence of antiphospholipid antibodies (aPL) has been observed in a substantial proportion of ITP patients, but its clinical significance remains to be established. This study retrospectively investigated the prevalence and clinical significance of aPL in ITP patients and assessed the risk factors for thrombosis. One hundred and sixty‐five subjects with ITP were included in the study and followed for a mean period of 63·4 months. Sixty‐nine (41·6%) patients were positive for aPL at diagnosis, and their clinical characteristics and course of ITP were not different from those of aPL‐negative patients. Twenty‐one (12·7%) patients developed a thrombotic event during follow‐up and the cumulative incidence rate ratio of aPL‐positive to aPL‐negative patients for thromboembolism was 3·15 [95% confidence interval (CI) 1·21–8·17] after adjusting for confounding factors. Lupus anticoagulant and hypertension were identified by Cox regression analysis as independent risk factors for thrombosis [hazard ratio (HR) 4·1, 95% CI 1·4–11·9, P = 0·009 and HR 5·6, 95% CI 1·9–15·8, P = 0·001, respectively]. Our results showed that a substantial proportion of ITP patients were aPL‐positive, and that lupus anticoagulant and hypertension were independent risk factors for thrombosis. Detection of aPL can provide useful information for identifying patients at high‐risk for developing thrombosis.

High levels of uric acid in systemic lupus erythematosus is associated with pulmonary hypertension

To estimate the point prevalence of pulmonary hypertension (PH) and determine the associated factors for PH in patients with systemic lupus erythematosus (SLE).

Elevated Serum Levels of Syndecan-1 Are Associated with Renal Involvement in Patients with Systemic Lupus Erythematosus

Objective. Syndecan-1 (SDC-1) is a major constituent of the endothelial glycocalyx, which plays a role in maintaining vascular homeostasis and functions as a glomerular filtration barrier. SDC-1 is readily shed into the blood under various conditions, but the clinical implication of circulating SDC-1 in patients with systemic lupus erythematosus (SLE) remains unclear. We aimed to investigate the association of serum SDC-1 level with certain clinical manifestations of SLE. Methods. We measured serum SDC-1 levels by ELISA in 111 patients with SLE, 18 with rheumatoid arthritis (RA), and 20 healthy subjects, and investigated its association with clinical manifestations and laboratory variables. Results. Serum SDC-1 levels were higher in patients with SLE than in those with RA and healthy controls (both p < 0.001) and were positively correlated with SLE Disease Activity Index (SLEDAI; r = 0.367, p < 0.001) and anti-dsDNA antibody level (r = 0.259, p = 0.007), but inversely correlated with serum C3 and CH50 levels (r = −0.305, p = 0.001 and r = −0.244, p = 0.012). Patients with active nephritis had higher serum SDC-1 levels than patients with inactive nephritis and those without nephritis (both p < 0.001). In addition, serum SDC-1 levels were correlated with renal SLEDAI score (r = 0.540, p < 0.001) and excretion of proteinuria as measured by spot urine protein/creatinine ratio (r = 0.538, p < 0.001). In 14 patients with lupus nephritis (LN) whose serum samples were obtained at the time of renal biopsy, there was a positive correlation between serum SDC-1 levels and activity index (r = 0.632, p = 0.015). Conclusion. Serum SDC-1 levels are increased in SLE patients with nephritis, indicating that SDC-1 might be a useful serum biomarker for active LN.

A case of multicentric reticulohistiocytosis

Multicentric reticulohistiocytosis (MRH) is a rare non-Langerhans histiocytosis of unknown etiology with a predilection for joint and skin. The characteristic clinical features are papulonodular skin eruptions and inflammatory polyarthritis, sometimes progressive to arthritis mutilans, a severe destructive arthropathy. Although these manifestations can present at the same time, it is more common that one feature precedes the others. Notably, these features are similar to those found in some rheumatic diseases, such as rheumatoid arthritis or dermatomyositis, and this can lead to a misdiagnosis, especially during periods where only one feature is present. Herein, we report a female patient with polyarthralgia and subsequent skin eruptions, who was eventually diagnosed with MRH. Her symptoms seemed to resemble those of some rheumatic diseases, but several features such as affected joints and the characteristic shape of the skin lesions did not correspond to that. The histological result of infiltration of histiocytes and multinucleated giant cells in the skin ultimately facilitated the correct diagnosis. In this paper, we review MRH briefly and highlight several differential points which enable us to increase the likelihood of correctly diagnosing MRH.

Association of Anemic Hypoxia and Increased Pulmonary Artery Systolic Pressure in Patients With Systemic Lupus Erythematosus.

Pulmonary arterial hypertension (PAH) is a rare but serious complication of systemic lupus erythematosus (SLE). Chronic hypoxia is known to cause PAH resulting from pulmonary vascular remodeling. We investigated the association between anemic hypoxia and PAH in SLE patients.

Anemic hypoxia is associated with increased pulmonary artery systolic pressure in patients with systemic lupus erythematosus

Pulmonary arterial hypertension (PAH) is a rare but serious complication of systemic lupus erythematosus (SLE). Chronic hypoxia is known to cause PAH resulting from pulmonary vascular remodeling. We investigated the association between anemic hypoxia and PAH in SLE patients.

Mycobacterial tenosynovitis of the hand in a patient with systemic lupus erythematosus

Dear Editor, Mycobacterial tenosynovitis is the most common presentation of mycobacterial hand infection and may mimic lupus-related arthritis or tenosynovitis in a patient with systemic lupus erythematosus (SLE), often causing clinical confusion and delaying in diagnosis and appropriate treatment. Here, we report a female patient with SLE who developed mycobacterial tenosynovitis of the hand caused by co-infection of both Mycobacterium tuberculosis (TB) and M. intracellulare. A 51-year-old Korean woman with SLE presented with painful swelling from right thumb to wrist, which gradually worsened over the previous 10 days. The patient reported no recent history of insect bites, penetrating injuries or repetitive use of the hands. A diagnosis of SLE had been made 17 years earlier and her disease had been controlled readily by outpatient clinic visits since a hospitalization for herpes zoster infection 8 years previous. At the time of presentation, she received prednisolone 2.5 mg, hydroxychloroquine 200 mg, aspirin 100 mg, and azathioprine 50 mg daily. The hand, from the right thumb to wrist, was slightly warm, tender and swollen, and she was unable to flex the thumb completely to the palm (Fig. 1a). The white cell count was 3640/mm (62.4% neutrophils and 31.0% lymphocytes). The erythrocyte sedimentation rate (ESR) was 32 mm/h (reference, 0–20 mm/h) and C-reactive protein (CRP) was 0.44 mg/L (reference, 0.1–5.0 mg/L). Serologic tests revealed a C3 complement of 67.5 mg/dL (reference, 90–180 mg/dL), a C4 complement of 13.5 mg/dL (reference, 10–40 mg/dl), total hemolytic complement activity of 42.1 U/mL (reference, 23–46 U/ mL), and an anti-dsDNA titer of 162.5 IU/mL (reference, < 20 IU/mL). Chest X-ray revealed no lymphadenopathy or pulmonary infiltrates. Simple X-rays of the hands revealed soft tissue swelling along the first metacarpal and phalangeal bones of the right hand (Fig. 1b). Ultrasonography demonstrated hypertrophic echogenic synovium and fluid distention of the flexor hallucis longus tendon sheath and increased flow in the inflamed tendon sheath on color Doppler. We clinically suspected lupus tenosynovitis and performed empirically ultrasound (US)-guided injection of triamcinolone 10 mg. Her symptoms gradually improved. One month later, painful swelling recurred at the same site, and the patient received two more triamcinolone (20 mg) injections. Afterward, a nonsteroidal anti-inflammatory drug was added to her regimen, and the symptoms waxed and waned moderately. Seven months after the initial onset of symptoms, the lesion worsened again. The ESR was 29 mm/h and CRP was 0.96 mg/L. Magnetic resonance imaging (MRI) of the lesion was performed and revealed extensive fluid and soft tissue attenuation along the entire flexor tendon and tendon sheath in the wrist and marked enhancement of the paratendinous soft tissue lesion, suggesting exuberant tenosynovitis of all flexor tendons (Fig. 2). Subsequently, the patient underwent surgical tenosynovectomy. Severe teno-synovial hypertrophy was observed around all the flexor tendons of the wrist and the flexor pollicis longus of the thumb and fragmentary necrosis and/or liquefaction of the tendon sheath were observed, followed by radical debridement and draining. Surgical

FRI0365 Factors associated with left ventricular diastolic dysfunction in patients with systemic lupus erythematosus

Objectives Myocardial damage is common and often silent in patients with systemic lupus erythematosus (SLE). In this study, we investigated the clinical parameters associated with left ventricular diastolic dysfunction in SLE patients using algorithms of 2016 American Society of Echocardiography/European Association of Cardiovascular Imaging (ASE/EACVI) recommendations. Methods Sixty consecutive SLE patients and 38 controls matched for age and sex who were free of clinical cardiovascular disease were enrolled. Left ventricular diastolic dysfunction was assessed by echocardiography using 2016 ASE/EACVI guidelines. The demographic, clinical and laboratory data were obtained from medical records. Results Diastolic dysfunction was more common in SLE patients compared with controls (38.3% versus 13.2%, p=0.011), while LV ejection fraction was not different between groups. When patients were divided into 2 groups according to the presence of diastolic dysfunction, patients with diastolic dysfunction had higher prevalence of hypertension (p<0.001), dyslipidemia (p=0.031) and chronic kidney disease (p=0.045), but there was no difference between groups with regard to other organ involvement or autoantibody profile. Importantly, patients with diastolic dysfunction showed significantly higher SLICC/ACR damage index (p=0.001) and C-reactive protein levels (p=0.005). In multivariate regression analysis, hypertension (OR=16.6. 95% CI=3.466–79.479, p<0.001), higher SLICC/ACR damage index (OR=1.68, 95% CI=1.039–2.720, p=0.034), and CRP level (OR=1.12, 95% CI=1.004–1.254, p=0.042) was independently associated with diastolic dysfunction in SLE patients Conclusions Diastolic dysfunction is more common in SLE patients, and overall inflammatory burden reflected by SLICC/ACR damage index as well as conventional cardiovascular risk factors are associated with development of diastolic dysfunction in SLE patients. Disclosure of Interest None declared

SAT0466 Serum vitamin d deficiency is associated with active renal disease in systemic lupus erythematosus

Objectives 25-hydroxyvitamin D (25(OH)D) deficiency is common in systemic lupus erythematosus (SLE) as well as chronic kidney disease. In this study, we investigated the association of 25(OH)D deficiency and renal involvement in SLE patients. Methods Two hundred seventy-two SLE patients and 138 control subjects were enrolled; 102 patients with active nephritis, 42 patients with inactive nephritis, and 128 patients with non-renal disease. Serum 25(OH)D levels were measured, and clinical and laboratory data were obtained from medical records. Results Mean serum 25(OH)D levels were significantly lower in SLE patient than control subjects (19.6 ng/ml versus 21.7 ng/ml, p=0.006). Out of 272 patients, 61.8% were vitamin D deficient (defined as <20 ng/ml). Patients with active nephritis had lower serum 25(OH)D levels (16.9 ng/mL) than patients with inactive nephritis (20.2 ng/mL) and without nephritis (21.5 ng/mL) (p=0.030, p<0.001), but there was no difference between the inactive nephritis and non-renal disease. Moreover, serum 25(OH)D levels were positively correlated with complement C3 (γ=0.135, p=0.026) and C4 (γ=0.159, p=0.009), but inversely with anti-dsDNA antibody level (γ=−0.156, p=0.010). Analysis of receiver operating characteristic curve for differentiating active nephritis and non-renal disease revealed an area under the curve (AUC) of 0.676, which is better than those of anti-dsDNA antibody (AUC=0.585, p=0.038) and complement C3 (AUC=0.509, p=0.001), C4 (AUC=0.538, p=0.008). Conclusions Vitamin D is deficiency is more common in SLE patients with active nephritis, and its level could be a potential marker for active renal disease in SLE. A prospective cohort study is needed to further elucidate the causal relationships over time. Disclosure of Interest None declared

THU0052 Association between Antiphospholipid Antibodies and Arterial Thrombotic Events in Patients with Rheumatoid Arthritis

Background Antiphospholipid antibodies (aPL) were reported to be found by variable range from 5 to 75% in patients with rheumatoid arthritis (RA) but their clinical significance remains undetermined. Objectives The aim of this study is to investigate the association of the presence of aPL with thrombotic events in patients with RA. Methods aPL profile, including lupus anticoagulant (LAC), anti-cardiolipin antibodies (aCL), and anti-β2 glycoprotein I antibodies (aβ2GPI), was evaluated in 360 patients with RA according to the standard guideline. Clinical and radiographic data were collected retrospectively. Results aPL were identified in 36 patients (10.0%). LAC was the most common type (n=23, 6.4%) and aCL and aβ2GPI were detected in 5 and 12 patients (1.4 and 3.4%), respectively. Compared to aPL-negative group, aPL-positive group included more male patients (16 (44.4%) vs. 51 (15.7%), P<0.001) and had higher rate of ever-smoker (14 (38.9%) vs. 51 (15.7%), P=0.005) while there was no difference between two groups in the age, disease duration, and body mass index as well as in the rate of diabetes, hypertension, and dyslipidemia. There was also no significant difference in the positive rate of rheumatoid factor and anti-cyclic citrullinated peptide antibody, values of inflammatory indices and radiographic scores of hand x-ray between two groups. Of note, arterial thrombotic events more commonly developed in aPL-positive group than aPL-negative (5 (13.9%) vs. 7 (2.2%), P=0.004) while venous thrombosis did not differ between two groups (0 (0.0%) vs. 3 (0.9%), P=1.000). In multivariate regression analysis, the presence of aPL, old age, hypertension, and high value of baseline C-reactive protein were independently associated with arterial thrombotic events (P<0.05). Conclusions A proportion of RA patients was aPL-positive, and the presence of aPL was independently associated with arterial thrombosis. This result suggests that aPL might be contributable to increased risk for arterial thrombosis in patients with RA. References Olech E, Merrill JT. The prevalence and clinical significance of antiphospholipid antibodies in rheumatoid arthritis. Curr Rheumatol Rep 2006;8:100–8. Gladd DA, Olech E. Antiphospholipid antibodies in rheumatoid arthritis: identifying the dominoes. Curr Rheumatol Rep 2009;11:43–51. Disclosure of Interest None declared