A Case of Tjalma Syndrome Coincidentally Accompanied by an Ovarian Teratoma Successfully Treated with Intravenous Immunoglobulin-G Adjunctive Therapy

Tjalma or pseudo-pseudo Meigs' syndrome is a clinical condition that is characterized with ascites, pleural effusion, and increased serum CA-125 levels in patients with systemic lupus erythematosus (SLE) without the presence of ovarian tumor. On the other hand, Meigs' and pseudo-Meigs' syndromes represent the same manifestations with ovarian tumor. In this case report, we present a 43-year-old SLE patient suffering from Tjalma syndrome with the coexistence of incidental ovarian teratoma, who was successfully treated with intravenous immunoglobulin-G adjunctive therapy after inadequate response to surgical excision of the ovarian tumor, steroid, and cyclophosphamide pulse therapy.

A Case of Castleman's Disease Accompanied with Systemic Lupus Erythematosus

Castleman's disease is a rare atypical lymphoproliferative disorder. Although HHV-8 has been reported to be a cause of Castleman's disease, the etiology and pathogenesis of the disease remains mostly unknown. We experienced a 51-year-old female patient who was concurrently diagnosed with Castleman's disease and systemic lupus erythematosus. Castleman's disease has been rarely reported in patients with systemic lupus erythematosus. Thus, we report the case and briefly discuss relevant articles.

A Case of Etanercept Treatment in a Patient with Ankylosing Spondylitis on Peritoneal Dialysis

Treatments for patient with ankylosing spondylitis (AS) include non-steroidal anti-inflammatory drugs (NSAIDs), disease-modifying antirheumatic drugs (DMARDs) and anti-tumor necrosis factor-alpha (TNFalpha) agents. However, owing to the well-known nephrotoxicity of NSAIDs and some DMARDs, the use of these drugs is limited in AS patients with renal insufficiency. As the pharmacokinetics and metabolism of anti-TNFalpha agents in patients of end stage renal disease, especially those receiving peritoneal dialysis (PD), have not been investigated well, little is known about treating them with anti-TNFalpha agents. We described the safety and efficacy of etanercept, a soluble fusion protein comprising the TNF receptor 2 in linkage with the Fc portion of immunoglobulin G, in a 40-year-old male AS patient receiving PD.

Is the frequency of metabolic syndrome higher in South Korean women with rheumatoid arthritis than in healthy subjects?

BACKGROUND/AIMS: To compare the frequency of metabolic syndrome (MetS) and magnitude of insulin resistance, measured by the homeostatic model assessment of insulin resistance (HOMA-IR), between South Korean women with rheumatoid arthritis (RA) and healthy subjects, and to evaluate risk factors for MetS and increased HOMA-IR in patients with RA. METHODS: In a cross-sectional setting, 84 female patients with RA and 109 age-matched healthy female subjects were consecutively recruited at a university-affiliated rheumatology center in South Korea. MetS was defined according to the Third Report of the National Cholesterol Education Program's Adult Treatment Panel (NCEP-ATP III) 2004 criteria. RESULTS: The frequency of MetS did not differ significantly between patients with RA (19%) and healthy subjects (15.6%, p = 0.566), although patients with RA had a higher HOMA-IR compared with healthy subjects (p < 0.001). Patients with RA met the NCEP-ATP III 2004 criteria for high blood pressure more often than healthy subjects (44% vs. 19.3%, p < 0.001), and low high density lipoprotein cholesterol was more prevalent in healthy subjects (33%) than in patients with RA (14.3%, p = 0.004). Although no obvious risk factors for the presence of MetS were identified in patients with RA, higher serum C-reactive protein and disease activity score assessed using the 28-joint count for swelling and tenderness-erythrocyte sedimentation rate significantly contributed to a higher HOMA-IR. CONCLUSIONS: Despite their increased insulin resistance, South Korean women with RA did not have a significantly higher frequency of MetS compared with that in healthy subjects.

A Case of Pediatric-onset Mixed Connective Tissue Disease Presenting Raynaud's Phenomenon Affecting Tongue, Hands, and Feet

Mixed connective tissue disease (MCTD) was first described by Sharp and coworkers in 1972, characterized by symptoms of Raynaud's phenomenon or swollen hands, overlapping clinical features of systemic lupus erythematosus, systemic sclerosis, or polymyositis/dermatomyositis, and the presence of anti-U1 RNP antibody. MCTD is rare in children and constitutes 0.3~0.6% of all rheumatologic patients in pediatric rheumatology database of the United States. Here, we report the first Korean case of a 10-year-old female patient with MCTD, presenting Raynaud's phenomenon in the hands, feet, and tongue.

Frequency of Metabolic Syndrome in Female Patients with Systemic Sclerosis: A Preliminary Report

OBJECTIVE: A higher prevalence of metabolic syndrome (MetS) has been described in various rheumatic diseases such as rheumatoid arthritis and systemic lupus erythematosus, but no direct studies have investigated the prevalence of MetS in systemic sclerosis (SSc) patients compared with the general population. We compared the frequency of MetS and insulin resistance, as measured by Homeostatic assessment model-insulin resistance (HOMA-IR) between female SSc patients and healthy subjects. METHODS: In a cross-sectional setting, 35 female SSc patients and 84 age and sex-matched healthy subjects were consecutively recruited at a single center of a tertiary hospital between 2009 and 2010. MetS was defined according to the National Cholesterol Education Program's Adult Treatment Panel (NCEP-ATP III) 2004 criteria, using the Asian criteria for central obesity. RESULTS: The frequency of MetS was not significantly higher in SSc patients (20.0%) than in healthy subjects (14.3%, p=0.425), but SSc patients had an increased insulin resistance measured by HOMA-IR (p=0.001). Significantly more patients with SSc had increased triglyceride concentrations (p=0.004) and fewer SSc patients tended to meet the waist circumference criterion (p=0.051) according to the individual MetS features. Additionally, there were no significant differences in age, high-sensitivity C-reactive protein, body mass index, HOMA-IR, disease duration, cumulative glucocorticoids dose, modified Rodnan skin score or the proportion of limited SSc cases according to the absence or presence of MetS in SSc patients. CONCLUSION: Unlike other rheumatic diseases, our preliminary report did not show a significant difference in the prevalence of MetS between female SSc patients and healthy subjects.

Efficacy of Imatinib Mesylate Treatment for a Patient with Rheumatoid Arthritis and Who Developed Chronic Myelogenous Leukemia

Rheumatoid arthritis is associated with an increased risk of hematological malignancy as a result of the RA itself or its treatment. We report here on an unusual case of a 55-year-old female with long-standing rheumatoid arthritis and who was treated with low dose methotrexate and hydrochloroquine. She was diagnosed with chronic myelogenous leukemia that manifested with severe thrombocytosis and basophilia, and this was treated with imatinib mesylate. After 6 months, she achieved a complete cytogenetic response of the CML and a complete resolution of all the RA symptoms without DMARDs.

A case of accessory splenic tuberculosis mimicking a distal pancreatic tumor / 대한내과학회지

Abdominal tuberculosis usually affects the gastrointestinal tract, peritoneum, lymph nodes, liver or spleen. Tuberculosis of the spleen is uncommon, except when associated with miliary dissemination. We report a case of a 33-year-old man with tuberculosis of the accessory spleen, which was originally suspected to be a distal pancreatic tumor. He was admitted with a history of left upper quadrant abdominal pain for 3 months. Computed tomography imaging of the abdomen revealed a 4.5 cm sized poorly defined hypodense mass in the distal pancreas and showed multiple focal hypodense lesions in the enlarged spleen. We performed distal pancreatectomy and splenectomy under the preoperative expectation of a distal pancreatic tumor. Microscopic examination of the specimens revealed accessory splenic tuberculosis associated with splenic tuberculosis. Following this, he was treated with appropriate antituberculosis drugs and was discharged without any complications.

IL-15 Induced an Increased SDF-1 Expression in the Synovial Fibroblasts of Patients with Rheumatoid Arthritis / 대한류마티스학회지

OBJECTIVE: Interleukin-15 (IL-15) recruits and activates synovial T cells, and IL-15 plays an important role in amplifying and perpetuating inflammation in the pathogenesis of rheumatoid arthritis (RA). Stromal cell-derived factor-1 (SDF-1) is a potent chemoattractant for memory T cells in the inflamed RA synovium. This study investigated the effect of IL-15 on SDF-1 production in RA fibroblast-like synoviocytes (FLS). METHODS: The expressions of IL-15 and SDF-1 were determined from the synovium of patients with RA and osteoarthritis (OA) by performing immunohistochemistry. The expressions of SDF-1 was measured from the RA FLS that were cultured with IL-15 and IL-17 by real-time RT-PCR and ELISA. The SDF-1 expression was also measured, via ELISA, from the RA FLS stimulated by IL-15 together with the inhibitors of such intracellular signal molecules as phosphatidylinositol 3-kinase (PI 3-kinase, LY294002), STAT3 (AG490), MAP Kinase (PD98059), NF-kappaB (parthenolide) and activator protein 1 (AP-1, curcumin). RESULTS: IL-15 and SDF-1 were mainly expressed in the RA synovium compared to that of the OA synovium. IL-15 increased the SDF-1 expressions and it, and had an additive effect with IL-17 on the SDF-1 expressions in the cultured RA FLS. The IL-15 induced increase of the SDF-1 expression in the cultured RA FLS was blocked by the inhibitors of PI 3-kinase, NF-kappaB and AP-1. CONCLUSION: The SDF-1 expression was increased in the RA synovium and it was up-regulated by IL-15 in the RA FLS through the PI 3-kinase, NF-kappaB, and AP-1 pathways. These results imply that the IL-15 induced increase of the SDF-1 expressions may be involved in the immunopathogenesis of RA.

IL-15 Induced an Increased SDF-1 Expression in the Synovial Fibroblasts of Patients with Rheumatoid Arthritis / 대한류마티스학회지

OBJECTIVE: Interleukin-15 (IL-15) recruits and activates synovial T cells, and IL-15 plays an important role in amplifying and perpetuating inflammation in the pathogenesis of rheumatoid arthritis (RA). Stromal cell-derived factor-1 (SDF-1) is a potent chemoattractant for memory T cells in the inflamed RA synovium. This study investigated the effect of IL-15 on SDF-1 production in RA fibroblast-like synoviocytes (FLS). METHODS: The expressions of IL-15 and SDF-1 were determined from the synovium of patients with RA and osteoarthritis (OA) by performing immunohistochemistry. The expressions of SDF-1 was measured from the RA FLS that were cultured with IL-15 and IL-17 by real-time RT-PCR and ELISA. The SDF-1 expression was also measured, via ELISA, from the RA FLS stimulated by IL-15 together with the inhibitors of such intracellular signal molecules as phosphatidylinositol 3-kinase (PI 3-kinase, LY294002), STAT3 (AG490), MAP Kinase (PD98059), NF-kappaB (parthenolide) and activator protein 1 (AP-1, curcumin). RESULTS: IL-15 and SDF-1 were mainly expressed in the RA synovium compared to that of the OA synovium. IL-15 increased the SDF-1 expressions and it, and had an additive effect with IL-17 on the SDF-1 expressions in the cultured RA FLS. The IL-15 induced increase of the SDF-1 expression in the cultured RA FLS was blocked by the inhibitors of PI 3-kinase, NF-kappaB and AP-1. CONCLUSION: The SDF-1 expression was increased in the RA synovium and it was up-regulated by IL-15 in the RA FLS through the PI 3-kinase, NF-kappaB, and AP-1 pathways. These results imply that the IL-15 induced increase of the SDF-1 expressions may be involved in the immunopathogenesis of RA.