No abstract available.
Arthritis, Juvenile* ; Fractures, Compression* ; Humans

PURPOSE OF REVIEW: Rheumatoid arthritis (RA) is characterized by a chronic T-cell response that has escaped normal control mechanisms. This review summarizes recent insights in pathways that are functional in RA and that favor continuous and pathogenic T-cell activation. RECENT FINDINGS: T-cell activation is ultimately determined by positive signals from costimulatory molecules and negative signals from regulatory T cells. Blockade of the classic costimulatory pathway, CD28-CD80 or CD86, is beneficial in RA. Additional pathways that predominantly control the activation of memory and effector T cells are functionally important in synovial inflammation. Some of these costimulatory molecules(such as stimulatory killer cell immunoglobulin-like receptors and NKG2D) appear to be relatively specific for RA and not to play a role in normal immune responses. In addition to this predominance of positive signals, age-disproportionate decline in thymic activity in RA may lead to a diminution of regulatory T cells and loss of their negative signals. SUMMARY: The successful treatment trial of RA with CTLA-4Ig clearly documents the importance of T-cell costimulation in RA disease activity. Novel costimulatory pathways may be of even greater significance than CD28 in RA and may represent promising new therapeutic targets. The finding of reduced thymic activity in RA is exciting and will stimulate further studies of T-cell homeostasis and the function of regulatory cells.
Arthritis, Rheumatoid* ; Autoimmunity ; Homeostasis ; Inflammation ; Lymphocytes* ; Memory ; Receptors, KIR ; T-Lymphocytes ; T-Lymphocytes, Regulatory ; United Nations

The appearance of a tumor in the chest wall is rare compared to that in any other part of the body. It can be classified into benign and malignant types and can be located in the rib, clavicle, sternum, cartilage and soft tissues. Tumors that are metastatic are commonly located in the lung, breast, bone and pleura. But, the soft tissue mass of anterior chest wall is rarely metastasized from a distant organ that is not confined to the thoracic cavity. This and thus has rarely been described. A 68-year-old man was admitted to our hospital with a chief complaint of resting dyspnea. A huge non-tender mass of about 10*15 cm in size was visible on his left lower anterior chest wall. We pathologically confirmed that the mass was a metastatic renal cell carcinoma of clear cell type by incision biopsy. Through an incision biopsy, the mass was pathologically confirmed as a metastatic renal cell carcinoma of the clear cell type.
Aged ; Biopsy ; Breast ; Carcinoma, Renal Cell* ; Cartilage ; Clavicle ; Dyspnea ; Humans ; Lung ; Neoplasm Metastasis* ; Pleura ; Ribs ; Sternum ; Thoracic Cavity ; Thoracic Wall* ; Thorax*

BACKGROUND: The Rheumatoid Factor (RF) is the only serological marker in the diagnosis of rheumatoid arthritis (RA), but its sensitivity and specificity are not satisfactory for the diagnosis of RA. Therefore, we investigated the diagnostic performance of a new anti-cyclic citrullinated peptide antibodies test (anti-CCP) by the enzyme-linked immunosorbent assay (ELISA) in RA. METHODS: A cyclic peptide variant that contains citrulline was used as an antigenic substrate in ELISA. We performed the RF and anti-CCP in 324 RA patients, 251 non-RA patients (rheumatic diseases other than RA), and 286 normal individuals. Diagnostic performances such as sensitivity and specificity were evaluated by the receiver-operator characteristics (ROC) curve at optimal cut-off values. The optimal cut-off values were determined at the maximal point of the area under the curve. RESULTS: The sensitivity and specificity of anti-CCP were 72.8% and 92% at 3.8 U/mL. The sensitivity and specificity of RF were 80.6% and 78.5% at 9 U/mL. The sensitivity and specificity of anti-CCP and RF were 67%, 95.2% and 63.3%, 90% at 8.4 U/mL, 20 U/mL, respectively. A combination of anti-CCP with RF increased the sensitivity and specificity to 79.3%, 96.4%, respectively. Anti-CCP was positive in 23.8% among 63 sero-negative RA patients. CONCLUSIONS: We considered that the anti-CCP might be useful as another new serological marker for the diagnosis of a RA combination with RF, or not, because the anti-CCP has a higher diagnostic specificity than the RF and was an easy, convenient ELISA method in performance.
Antibodies* ; Arthritis, Rheumatoid* ; Citrulline ; Diagnosis ; Enzyme-Linked Immunosorbent Assay ; Humans ; Rheumatoid Factor ; Sensitivity and Specificity

This article has been retracted.

Systemic lupus erythematosus (SLE) is an autoimmune disorder affected by multiple genetic, hormonal and environmental factors, which makes it impossible to identify the exact cause of this ailment by only investigating SLE patients, who are genetically heterogeneous, and live in various environments. Therefore, the study of mouse models of lupus has provided valuable clues to help identify, and to validate, novel molecular pathways and targets implicated in the pathogenesis of the disease. While there is no perfect model to reflect all the disease phenotypes observed in human patients, disease subsets are represented in various animal models, which allows modulation of a particular pathophysiological pathway, resulting in the possibility of dissecting its specific contribution to disease development. Spontaneous mouse models of lupus have led to identification of numerous susceptibility loci, from which several candidate genes have been found, while induced models of lupus have provided insight into the role of environmental factors, as well as a better understanding of the cellular mechanisms by which SLE develops. Animal models also allow us to screen and evaluate potential preventive and therapeutic agents. Correlation of specific pathways in animal models to subsets of human disease offers the unique possibility of more accurate preclinical predictions of efficacy for single or combinatorial therapeutic approaches in the clinic. Here, we introduce various animal models of SLE, and review current data focused on genetic factors that are associated with susceptibility or phenotypes of lupus, leading into the present understanding of the genetic basis in lupus pathogenesis.
Animals ; Humans ; Lupus Erythematosus, Systemic ; Mice ; Models, Animal ; Phenotype

Leflunomide is a disease-modifying antirheumatic drug that has been available in Korea since 2003. Leflunomide induced interstitial pneumonitis has been reported as an adverse effect in other countries but not in Korea. A 57-year-old woman was treated with leflunomide since she had been resistant to methotrexate, hydroxychloroquine and sulfasalazine. She developed high fever, dyspnea, and non-productive cough 3 months after the administration of leflunomide. She was diagnosed leflunomide-induced interstitial pneumonitis based on history, physical, laboratory, radiologic and pathologic findings. The patient was treated by prednisolone 1 mg/kg/day with cholestyramine 24 g/day, resulting in dramatic improvement. Here we report a case of leflunomide induced pneumonitis treated successfully with high dose steroid.
Arthritis, Rheumatoid ; Cholestyramine Resin ; Cough ; Dyspnea ; Female ; Fever ; Humans ; Hydroxychloroquine ; Korea ; Lung Diseases, Interstitial* ; Methotrexate ; Middle Aged ; Pneumonia ; Prednisolone ; Sulfasalazine

OBJECTIVE: A number of soluble factors,which play important role in the pathophysiology of rheumatoid synovitis are also known to be involved in osteoclast differentiation and activation through RANKL (Receptor activator of NF-kB ligand). To investigate the importance of RANKL in the pathogenesis of bone erosion in rheumatoid arthritis (RA) patients, we analyzed the expression of RANKL and Osteoprotegerin (OPG) and examined the formation of osteoclasts in rheumatoid synovial fibroblasts under the influence of various osteotropic factors. METHODS: Primary culture synoviocytes or fibroblast-like synoviocytes isolated from synovial tissues of 8 RA patients were cultured and treated with IL-1beta (2 ng/ml), TNF-alpha (2 ng/ml), INF-gamma(1000 micro/ml), IL-15 (10 ng/ml), IL-12 (10 ng/ml), dexamethasone (10(-9) M), PMA (10 ng/ml) or 1,25 (OH)2D3 (10(-9) M) for 18 hours. Expression RANKL or OPG mRNA was measured by semiquantitative RT-PCR within linear amplification condition. TRAP (+) MNC (tartrate resistant acid phosphatase-positive multinucleated cell) formation was induced from primary culture synoviocytes or in coculture system of synovial fibroblasts with PBMCs in the presence of M-CSF and 1,25 (OH)2D3. RESULTS: 1. The intensity of base-line expression was different from patient to patient. Primary culture synoviocytes and synovial fibroblasts express RANKL and OPG mRNA with decreasing intensity when they are passaged. 2. Expresssion of RANKL mRNA was significantly increased by 1,25 (OH)2D3 and IL-1beta (158.8+/-21% and 197.2+/-17% of controls, p<0.05 and p<0.005, respectively), while decreased significantly by dexamethasone (25.6+/-4.6% of controls, p<0.005). Expression of RANKL mRNA was significantly increased by IL-1beta and decreased by dexamethasone, in a dose- and time-dependant manner. 3. TRAP (+) MNCs are formed from primary culture synoviocytes or in coculture system of synovial fibroblasts and PBMC in the presence of M-CSF and 1,25 (OH)2D3. Dexamethasone clearly inhibited TRAP (+) MNCs formation from synovial cells. CONCLUSION: The regulatory mechanism for the expression of RANKL or OPG in rheumatoid synoviocytes might be different from that in bone marrow cells. Modulating the expression of these molecules could have potential therapeutic implication targeting bone destruction in RA.
Arthritis, Rheumatoid ; Bone Marrow Cells ; Coculture Techniques ; Dexamethasone ; Fibroblasts* ; Humans ; Interleukin-12 ; Interleukin-15 ; Macrophage Colony-Stimulating Factor ; NF-kappa B* ; Osteoclasts* ; Osteoprotegerin ; RNA, Messenger ; Synovitis ; Tumor Necrosis Factor-alpha

BACKGROUNDS: Rheumatoid factor (RF) is common serological marker for the diagnosis of rheumatoid arthritis (RA), but its sensitivity and specificity are not satisfactory for the diagnosis of RA. Therefore, we investigated the diagnostic performance of a new antifilaggrin antibody test by enzyme linked immunosorbent assay (ELISA) in RA. METHODS: Recombinant human filaggrin was deiminated in vitro by peptidylarginine deiminase and used as the coating antigen for ELISA. We performed the RF and the antifilaggrin antibody for 324 RA patients, 251 non-RA patients (rheumatic diseases other than RA), and 286 normal individuals and evaluated the sensitivities and specificities of RF and antifilaggrin antibody. Optimal cut off values were calculated as mean+2SD in 95% confidence interval except 3SD for 286 normal individuals. Optimal cut off values of antifilaggrin antibody and RF were 9.6 U/ml and 12 U/ml, respectively. RESULTS: The sensitivities and specificities of antifilaggrin antibody were 44.8% and 89.2% at optimal cut off values. The sensitivity and specificity of RF were 75.0% and 83.3%. Combination of "antifilaggrin antibody and RF" showed significantly high specificity of 95.2% and that of "antifilaggrin antibody or RF" showed slightly high sensitivity of 79.3% at optimal cut off values. Antifilaggrin antibody was positive in 17.3% among 81 sero-negative RA patients. CONCLUSION: We considered that antifilaggrin antibody could be used a supplementary test of RF for the diagnosis of RA, because "antifilaggrin antibody and RF" had higher diagnostic specificity than RF alone and antifilaggrin antibody test was easy, convenient ELISA method in performance.
Arthritis, Rheumatoid* ; Diagnosis ; Enzyme-Linked Immunosorbent Assay ; Humans ; Rheumatoid Factor ; Sensitivity and Specificity

Behcet's disease (BD) is a multi-systemic inflammatory disease of unknown origin that affects nearly all organs including the nervous system. Although the neurological involvement is less frequent than other major presentations, it is important because it can produce severe disabilities. Peripheral nervous system manifestations are relatively rare in BD. Although few cases of peripheral neuropathy or myopathy have been reported in BD, they are cases of multiple neuropathies, sensorimotor peripheral neuropathy, or neuropathy autonomic dysfunction. Guillain-Barre syndrome (GBS), also known as an acute inflammatory demyelinating polyneuropathy, is an acute demyelinating polyradiculopathy of uncertain etiology. No case of GBS associated with BD in Korea has been reported. Herein we report on a patient of BD who suffered from weakness of extremities and was diagnosed as GBS.
Extremities ; Guillain-Barre Syndrome* ; Humans ; Korea ; Muscular Diseases ; Nervous System ; Peripheral Nervous System ; Peripheral Nervous System Diseases ; Polyradiculopathy