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 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 Successful Treatment of Refractory Synovitis Acne Pustulosis Hyperostosis Osteitis (SAPHO) Syndrome with Adalimumab / 영남의대학술지

Synovitis acne pustulosis hyperostosis osteitis (SAPHO) syndrome is a rare disease that involves the skin, bones and joints. It is thought to be caused by infection with low-toxicity bacteria and to be the result of reactive infectious osteitis. However, this hypothesis has not yet been clearly established. New SAPHO syndrome treatment methods are needed because the disease does not respond to treatment in many cases. In this paper, a case is reported of SAPHO syndrome with pain in the acromioclavicular joint and with squamous and pustular macules on the palms and soles. First, the patient was treated with aceclofenac, prednisolon and sulfasalazine for two weeks. However, the symptoms were not relieved, so methotrexate and pamidronate were added to the treatment. Since no improvement was seen after four weeks of treatment, adalimumab was prescribed. The skin lesions were relieved two weeks later, and the bone pain and arthralgia, four weeks later. No recurrence or adverse effects were observed at the 22-week follow-up.

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.

A Case of Anti-Cyclic Citrullinated Peptide Antibody-Positive Rheumatoid Arthritis Following Peginterferon Alpha-2A and Ribavirin Therapy for Chronic Hepatitis C / 대한내과학회지

Combination therapy with pegylated interferon (IFN) and ribavirin is the mainstay of treatment for chronic hepatitis C. An important side effect of IFN is the induction of an autoimmune disease such as autoimmune thyroid disease or, rarely, rheumatoid arthritis. However, the introduction of pegylated formulations of IFN can minimize the risk of autoimmune induction by reducing immunogenicity. Detection of anti-cyclic citrullinated peptide (anti-CCP) antibodies can help distinguish rheumatoid arthritis from hepatitis C-related arthritis. Here, we report the first Korean case of a 48-year-old female who developed anti-CCP antibody-positive rheumatoid arthritis following peginterferon alpha-2a therapy for hepatitis C.

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.

Coronary Artery Calcification Measured by Multi-Detector Computed Tomography in Systemic Sclerosis / 대한류마티스학회지

OBJECTIVE: There is some controversy regarding the early onset or high incidence of coronary atherosclerosis in patients with systemic sclerosis (SSc). Measurements of the coronary calcification score (CCS) by multi-detector computed tomography (MDCT) is an accurate and non-invasive method for detecting coronary atherosclerosis, and a high level of CCS (> or =160) can predict coronary events. This study examined the CCS using MDCT and evaluated the risk of coronary events in patients with SSc. METHODS: The clinical and laboratory characteristics of 35 patients with SSc were examined. The CCS was measured by MDCT, and the risk of coronary events were evaluated by CCS and the Framingham risk score (FRS). RESULTS: In 35 patients (2 males and 33 females, 20 with limited and 15 with diffuse type), the mean age was 52+/-12 years and the disease duration was 8+/-7 years. The mean CCS was 10.1+/-30.8, the CCS of 28 patients (80%) was 0, and all patients had a CCS<160. The CCS had no significant correlation with the clinical and laboratory characteristics. The FRS was evaluated in 29 patients. Twenty eight patients were categorized into the low-risk group (FRS<10%) and only one was classified into the moderate-risk group (FRS=13%). CONCLUSION: These results suggest that the risk of coronary events due to coronary atherosclerosis might not be high in patients with SSc.

Determinants of Insulin Resistance in Patients with Rheumatoid Arthritis / 대한류마티스학회지

OBJECTIVE: Rheumatoid arthritis (RA) is associated with an increased cardiovascular events. These may be related to insulin resistance (IR). We evaluated status of IR and analyzed the relationship between IR and clinical and laboratory characteristics in patients with RA. METHODS: We examined 52 RA patients (43 females) and 52 age and sex matched healthy controls. We measured Homeostasis model assessment (HOMA) IR, calculated according to fasting serum glucose and insulin. RESULTS: In patients, age was 50.8+/-10.2 years old, disease duration was 42.1+/-30.9 months. In controls, HOMA IR was 0.62+/-0.30 and in patients, it was 1.28+/-0.50. This difference was highly significant (p<0.001). Patients with early RA (disease duration is less than 36 months) were 28, and established RA (more than 36 months) were 24. HOMA IR was significantly higher in patients with established RA (1.42+/-0.45) than those with early RA (1.16+/-0.45) (p=0.03), and significantly correlated with disease duration (r=0.36, p=0.01), BMI (r=0.36, p<0.001), total cumulative prednisolon dose (r=0.34, p=0.01). Disease duration and BMI were independent predictors for HOMA IR (p<0.01, p=0.03). CONCLUSION: In patients with RA, IR measured by HOMA IR was more significantly increased than that of healthy control and significantly correlated with disease duration, BMI, and total cumulative prednisolon dose; however, the determinants of HOMA IR in RA patients were disease duration and BMI.

Increased Interleukin-17 Expression in Patients with Idiopathic Inflammatory Myopathies / 대한류마티스학회지

OBJECTIVE: Idiopathic inflammatory myopathies (IIMs) are systemic autoimmune diseases characterized by infiltration of T lymphocytes, monocytes, and macrophages in muscle tissues. Interleukin-17 (IL-17), a Th17 cytokine, has potent pro-inflammatory actions and plays a role in autoimmune diseases. We investigated the expression of IL-17 in muscle tissues of patients with IIMs. METHODS: We measured the IL-17 mRNA level of muscle tissues from 14 patients with IIMs (9 patients with dermatomyositis and 5 patients with polymyositis) by real-time RT-PCR and compared with controls. We also performed an immunohistochemical stain to detect IL-17 expression. RESULTS: The expressions of IL-17 were significantly enhanced in IIMs than controls. In immunohistochemistry, IL-17 was expressed in perimysial, endomysial and perivascular infiltrating inflammatory cells. CONCLUSION: These results suggest that IL-17 plays a role in the immunopathogenesis of IIMs.

The Expression of Toll-like Receptors in Idiopathic Inflammatory Myopathies / 대한류마티스학회지

Objective: To investigate the expression of toll-like receptor (TLR)-2, 4 and 9 in idiopathic inflammatory myopathies (IIMs). Methods: The expression of TLR-2, 4 and 9 was measured by real-time RT-PCR and immunohistochemical stain (IHS) from muscle tissues in patients with IIMs and controls. Results: The expression levels of TLR-2, 4 and 9 in IIMs were significantly higher than controls. TLR-2, 4 and 9 were mainly expressed on sarcolemma of muscle fibers, perimysial vascular endothelium and infiltrating inflammatory cells in dermatomyositis, whereas, they were mainly expressed on sarcolemma of muscle fibers, destructed muscle fibers, and enodmysial infiltrating inflammatory cells in polymyositis. Conclusion: TLR-2, 4 and 9 were highly expressed in muscle tissue of IIMs. These results suggest that TLR-2, 4 and 9 play a role in pathogenesis of IIMs.

Recurrent Respiratory Papillomatosis improved by Treatment with Interferon-alpha / 결핵

Recurrent respiratory papillomatosis(RRP) is a chronic disease that is caused by the human papillomavirus(HPV) type 6 and 11. The most common site of the lesions is the larynx, but papillomas can occur throughout the respiratory and upper gastrointestinal tracts. Frequent recurrence of disease, can result in airway compromise and even death when papillomas either obstruct the airway or spread to the lung parenchyma. We encountered a case of a recurrent respiratory papillomatosis in a 23-year-old patient, who improved after treatment with interferon-alpha. The patiented with hoarseness, exertional dyspnea, and a productive cough. We report this case with a brief review of the relevant literature.

A Case of Cerebral Aspergillosis in a Patients with Rheumatoid Arthritis / 대한류마티스학회지

We report a case of aspergillosis of the central nervous system in patient with rheumatoid arthritis (RA). A 46-year-old woman with 20-year history of RA and on treatment with corticosteroid, hydroxychloroquine and methotrexate, was admitted because of drowsiness, dizziness and dysarthria. On admission, physical examination and laboratory data showed, among other findings, disappearance of pupil reflex, positive Babinski and Chaddock reflex. Magnetic resonance imaging (MRI) of brain showed multiple high signal intensity lesion on medulla, pons, midbrain, basal ganglia, internal capsule, thalamus and hypothalmus. Stereotactic brain biopsy was performed and biopsy specimen revealed an invasive Aspergillus.

A Case of Neurofibromatosis Type 1 Associated with Minimal Change Disease / 대한신장학회잡지

Neurofibromatosis type 1 is the most common neurocutaneous disorders and affects between 1/2,000 and 1/4,500 people. This occurs at any age and is hereditary disease with autosomal dominant fashion. Renovascular hypertension is major form of renal manifestation of the disease. There are few reported cases in Japan and Hungary of Recklinghausen's neurofibromatosis with several glomerular lesions but their relationship is not apparent. A 21-year-old man was admitted to the hospital because of general edema. On admission, the blood pressure was 130/ 80 mmHg and general edema was noted. He had a plexiform neuroma on right flank and multiple cafe- au-lait spots on chest and extremites. Laboratory findings were as follows : Hemoglobin 14.2 g/dL, AST 28 IU/L, ALT 12 IU/L, albumin 1.2 gm/dL, total cholesterol 533 mg/dL, urinary protein 4.0 gm/ day, C3 86.6 mg/dL, C4 19.9 mg/dL, HBs Ag/Ab (+/-), HBe Ag/Ab (+/-), HCV Ab (-), HBV DNA probe 6,000 pg/mL. Renal biopsy was performed and the histological findings were compatible with minimal change disease. The immunohistochemical method revealed that HBsAg was negative. We experienced a case of minimal change disease concurrent with Neurofibromatosis type 1, but their relationship is not clear. We report this case with a brief review.

Aplastic Anemia in a Petrochemical Plant Worker / 대한산업의학회지

Aplastic anemia is characterized by pancytopenia with hypocellular bone marrow. Fifty percent of the cases are idiopathic and the rest are caused by various agents including drugs, chemicals, radiation and viruses. It is difficult to link specific etiologic agents, especially chemicals to the development of aplastic anemia because multiple or unknown exposures may be involved in. Benzene, a common industrial chemical and a component of gasoline, may lead progressively to pancytopenia, aplastic anemia and leukemia when exposed. A petrochemical worker with aplastic anemia was referred to our hospital to evaluate a relationship between the job history and the disease. He worked in the petrochemical plant for .21 years and was exposed to low-level benzene. There was not any other etiologic agent except benzene and this is the case report of aplastic anemia which possibly due to benzene exposure.