Kensuke Oryoji

Successful treatment of rapidly progressive interstitial pneumonia with autologous peripheral blood stem cell transplantation in a patient with dermatomyositis

Aggressive autoimmune diseases are often treated by intensive immunosuppressive treatment such as high-dose methylprednisolone and intravenous cyclophosphamide. Autologous hematopoietic stem cell transplantation can facilitate high-dose immunosuppressive therapy (HDIT), which is myeloablative. We describe a 54-year-old female patient with rapidly progressive and refractory interstitial pneumonia due to dermatomyositis, which was successfully treated with high-dose cyclophosphamide and autologous blood stem cell transplantation. Following transplantation, dyspnea disappeared, and arterial blood gas analysis and respiratory function test showed marked improvement. This improvement was confirmed by diminished interstitial shadows on chest X-ray and computed tomography scans. Eighteen months after transplantation, the patient is doing well without symptoms and signs of interstitial pneumonia.

Reduced carotid intima–media thickness in systemic lupus erythematosus patients treated with cyclosporine A

Abstract Background Patients with systemic lupus erythematosus (SLE) are at risk of atherosclerosis. An increased carotid intima–media thickness (IMT) is considered to be a marker of early atherosclerosis. Objective To determine influential factors for increased carotid IMT in SLE patients. Methods We evaluated the impact of conventional risk factors for atherosclerosis on carotid IMT in 427 healthy controls and of clinical factors on carotid IMT in 94 SLE patients. Carotid IMT was measured by using a newly developed computer-automated system. Unconditional logistic regression was used to assess the adjusted odds ratios (ORs) and 95 % confidence intervals (95 % CI). Results Multivariate-adjusted mean carotid IMT (mm) was significantly reduced in SLE patients (0.51, 95 % CI = 0.36–0.66) compared to healthy controls (0.55, 95 % CI = 0.40–0.70) (P = 0.003). The SLE Disease Activity Index (SLEDAI) was associated with carotid IMT in a dose-dependent manner (Ptrend = 0.041). The current use of cyclosporine A (adjusted OR = 0.02, 95 % CI = 0.01–0.40, P = 0.011) and a history of steroid pulse therapy (adjusted OR = 0.01, 95 % CI = 0.01–0.25, P = 0.006) were significantly associated with a decreased risk of increased carotid IMT. Conclusions Our findings suggest that the current use of cyclosporine A can protect against increased carotid IMT, leading to a decreased risk of arteriosclerosis. Future studies with a larger sample size need to confirm that this association holds longitudinally.

Wegener’s granulomatosis detected initially by integrated 18F-fluorodeoxyglucose positron emission tomography/computed tomography

Early diagnosis is crucial for effective treatment of Wegener’s granulomatosis, although this disease shows only atypical symptoms in the primary stage. This report describes a patient suspected of having a malignancy based on integrated 18F-fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT), which showed increased uptake in pulmonary nodules and nasopharyngeal mucosa. Integrated PET/CT is therefore considered to be useful to confirm the distribution and determine the optimal site for biopsy.

A Case of Immunoglobulin G4-Related Disease with Extensive Multiorgan Involvements

We report a case of IgG4-related disease (IgG4-RD) with multiple ten-organ involvement. This case showed many clinical findings, such as bilateral swelling of salivary and lacrimal glands, autoimmune pancreatitis, interstitial nephritis, retroperitoneal fibrosis, periaortitis, systemic swelling of lymph nodes, pulmonary lesions, splenomegaly, and jejunal lesions. He was suspected as having SLE or malignant lymphoma but diagnosed as having IgG4-RD by the elevated serum IgG4 level and histological findings from kidney and lymph node. We report a case of IgG4-RD with multiple ten-organ involvement that was successfully treated with prednisolone therapy.

Quality of life in Japanese female patients with systemic lupus erythematosus: Evaluation using the Short Form 36 Health Survey

Objective. Aspects of health-related quality of life (HRQoL) are important for assessing perceived health status and treatment burden. We evaluated HRQoL using Short Form 36 Health Survey (SF-36) and factors associated with HRQoL. Methods. We collected basic and lifestyle-related, clinical, and treatment characteristics among 119 female Japanese patients with systemic lupus erythematosus (SLE). Odds ratios (ORs) and their 95% confidence intervals were assessed for associations between HRQoL and selected factors. Results. Irregularity of sleep was significantly associated with risk of lower role physical (RP) (OR = 8.27), vitality (VT) (OR = 8.45), and role emotional (OR = 10.7) domains. Compared with clerical work, non-clerical work was significantly associated with risk of lower RP (OR = 7.39), and unemployment was significantly associated with risk of lower VT (OR = 41.0). Daily soybean intake was associated with improved General Health or GH (OR = 0.17). Compared with Systemic Lupus Collaborative Clinics Damage Index (SDI) = 0, SDI > 2 was associated with risk of lower PF (OR = 7.88), RP (OR = 4.29), and bodily pain (OR = 3.06) domains. Conclusion. Reduced HRQoL was observed in our SLE patients. Interventions addressing sleep and work disturbances, as well as daily soybean consumption, could alter the HRQoL of SLE patients.

Successful Treatment of Lupus Cerebrovascular Disease with Mycophenolate Mofetil.

We report a case of neuropsychiatric systemic lupus erythematosus successfully treated with mycophenolate mofetil (MMF). The patient was a 40-year-old female who maintained with 7 mg of prednisolone plus 100 mg of azathioprine (AZ) per day. According to transient ischemic attack that occurred repeatedly and an elevated level of interleukin-6 (IL-6) in spinal fluid, she was diagnosed as having neuropsychiatric systemic lupus erythematosus (NPSLE). Initial increase in doses of prednisolone and AZ to 20 mg and 150 mg per day, respectively, was ineffective. After switching from AZ to MMF, her symptoms of NPSLE completely resolved with marked improvement of the IL-6 level in her spinal fluid, suggesting that MMF was effective.

Infected Thoracic Aortic Aneurysm Caused by Helicobacter cinaedi

The causative organism is not identified in some cases of infected aneurysms, a life-threatening condition. A 68-year-old man presented with chest/back pain and a 1-year history of intermittent fever and fatigue. Computed tomography revealed a thoracic aortic aneurysm. After several negative blood cultures, he was eventually diagnosed with an infected aneurysm caused by Helicobacter cinaedi via gene analysis of an aortic tissue specimen. As H. cinaedi is a low-virulence bacterium, infection with this pathogen should be suspected in cases of aortic aneurysms with unidentified causative organism and a long history of subjective symptoms. Detailed examinations, including polymerase chain reaction, should be conducted in such cases.

THU0486 Persistence with anti-Osteoporotic Therapy in Patients with Rheumatic Disease

Background Osteoporotic fragility fractures decrease physical performance and disability-free life expectancy. Patients with rheumatic disease develop osteoporosis due to local cytokine production, low levels of physical activity, and glucocorticoid therapy. Rheumatologists need to proactively manage osteoporosis in patients with rheumatic disease to extend their healthy and disability-free life years. While studies have reported that approximately 50% of postmenopausal patients with osteoporosis discontinue anti-osteoporotic therapy within 1 year, there is a paucity of data on treatment continuation and discontinuation in patients with rheumatic disease. We conducted a retrospective analysis of the status of oral anti-osteoporotic therapy in patients with rheumatic disease using the prescription database at a single rheumatology specialty center from June 2007 to May 2010, a period before teriparatide, a recombinant form of parathyroid hormone, and denosumab, a monoclonal antibody to receptor activator of nuclear factor kappa B ligand, were approved for clinical use. Objectives To estimate persistence with oral anti-osteoporotic therapy in patients with rheumatic disease and identify factors influencing the persistence and reasons for discontinuation. Methods Based on prescription database, we identified patients who started treatment with alendronate, risedronate, or selective estrogen receptor modulators during the study period. Patients who continued treatment after June 1, 2010, were “censored”. Patients who did not receive a prescription for 190 or more days during the study period were classified as “discontinued”. Persistence were estimated using Kaplan-Meier survival analysis. Multivariate Cox proportional hazard analysis was carried out to identify factors influencing the persistence. Reasons for discontinuation were abstracted from medical charts. Results Analysis of prescription database identified 557 patients (477 females and 80 males). Of these, 378 had rheumatoid arthritis, and 112 had systemic lupus erythematosus, dermatomyositis, systemic sclerosis, vasculitis syndrome, or other connective tissue diseases. Mean age at the start of anti-osteoporotic therapy was 62.1 years, and 481 patients (86.4%) received a mean prednisolone dose of 8.4 mg/day. Persistence with anti-osteoporotic therapy was 71.9%, 61.1%, 50.1% after 1, 2, and 3 years, respectively. Factors associated with persistence were the diagnosis of connective tissue disease and use of glucocorticoids. Among patients who discontinued therapy, the most frequent reason for discontinuation of anti-osteoporotic therapy was onset of adverse events (32.4%). Conclusions Patients with rheumatic disease receiving anti-osteoporotic therapy had a higher persistence than postmenopausal patients with osteoporosis. These results suggest that rheumatologists should take a proactive stance on managing osteoporosis in patients with rheumatic disease. Disclosure of Interest None declared

AB0314 Association of Rheumatoid Factor and Anti-Citrullinated Protein Antibody Status with Orthopedic Surgeries for Rheumatoid Arthritis Patients

Background Both rheumatoid factor (RF) and anti–citrullinated protein antibody (ACP) are associated with bone damage in rheumatoid arthritis (RA). However, there has been little investigation into whether these autoantibodies could interact and/or contribute to orthopedic surgeries for RA patients. Objectives To investigate the role of RF and ACP positivity status as a contributor to orthopedic surgeries for RA patients. Methods The study examined orthopedic surgery database with linkage to RF/ACP data set. Patients were categorized into the following four groups: double-positive (RF+ or high/ACP+ or high), RF+ or high/ACP- or low, RF- or low/ACP+ or high and double-negative (RF- or low /ACP- or low). Results Among 2,052 RA patients, 332 (16.2%) patients underwent orthopedic surgeries. Using receiver operating characteristics analysis, RF and ACP level to associate orthopedic surgeries were 34 IU/ml and 4.1 U/ml, respectively. 19.9% (244/1255), 19.7% (27/137), 14.3% (40/279) and 5.1% (21/411) of patients underwent orthopedic surgeries in RF high (RF>34 IU/ml)/ACP high (ACP>4.1 U/ml), RF high/ACP low, RF low/ACP high and RF low/ACP low patients, respectively (p<0.001). Next, we analyzed a total of 155 orthopedic surgical procedures of small joints (hand and forefoot). RF/ACP double positive was 87.7%, RF+/ACP- was 8.4%, RF-/ACP+ was 3.3% and double negative was 0.6%. Conclusions Concomitant presence of RF and ACP is associated with orthopedic surgeries. Our data suggest that RF may contribute to RA joint destruction regardless of the presence of ACP. Disclosure of Interest None declared

AB0256 Risk factors associated with generalized bone loss in patients with rheumatoid arthritis: retrospective longitudinal study.

Background Vertebral fractures are major cause of disability and impaired quality of life in elderly people. Low bone mineral density has been reported a major risk factor for vertebral fracture. In patients with rheumatoid arthritis, it have been suggested that an appropriate management of the disease and the quality of bone prevent vertebral fractures. However, there are limited data concerning risk factors for bone mineral density loss in patients with rheumatoid arthritis. Objectives To evaluate the risk factors for generalized bone mineral density loss in patients with rheumatoid arthritis. Methods In a longitudinal study, lumbar spine bone mineral density (BMD) was measured at baseline and after average 1.2 years. Data of RA disease activity, treatment agents towards RA and osteoporosis, dietary and physical status were collected. With univariate logistic regression analyses we tested baseline variables for association with BMD loss. Variables showing an association (p<0.10) with BMD loss were entered as possible predictors in a multivariate logistic analysis. Results: 218 patients were examined at baseline and follow-up time. Patients included in this study were on average 61 years old and the average disease duration were 12 years at baseline. In the univariate regression analysis, age, smoking status, Steinbrokcer’s stage, usage of drugs for osteoporosis and pre-existing of vertebral fracture at baseline were associated (p<0.10) with lumbar BMD loss. Subsequently in multivariate logistic regression analysis, only usage of drugs for osteoporosis was associated with decreased risk for lumbar spine bone mineral density loss (OR 2.57, 95% CI: 1.18-5.66, p= 0.017). In addition, smoking and shortage of calcium intake were associated with BMD loss among current users of drugs for osteoporosis, while among patients without drugs for osteoporosis, low body mass index was associated with BMD loss. Conclusions Results of this longitudinal study suggest that management of osteoporosis, including drugs and patient education towards life style, is important in patients with rheumatoid arthritis. Disclosure of Interest None Declared