A case of Sweet syndrome involving the central nervous system / 대한내과학회지

Sweet syndrome is an unusual disease characterized by the sudden onset of fever, leukocytosis, and painful erythematous plaques, and the dermal infiltration of neutrophils at the site of skin lesions. Although Sweet syndrome can also present with extra-cutaneous manifestations, involvement of the central nervous system (CNS) is rarely reported. We describe a case of Sweet syndrome involving the CNS in a 46-year-old male with a disturbance of consciousness following fever and erythematous skin plaques in the extremities. Cerebrospinal fluid examination disclosed neutrophilic pleocytosis without decreased glucose and protein levels. HLA typing showed B54, which is frequently seen in Sweet syndrome. Brain magnetic resonance imaging showed abnormal signal intensity lesions in the left temporal lobe. Skin biopsy revealed a dense dermal infiltration of neutrophils, which is compatible with Sweet syndrome. The confused mentality, fever, and erythematous skin plaques resolved after the administration of systemic corticosteroids.

A Case of Tenosynovitis Due to Mycobacterium intracellulare in a Patient with Rheumatoid Arthritis / 감염과화학요법

Nontuberculous mycobacteria are ubiquitous organisms that are frequently present in the water, soil and animal reservoirs. Nontuberculous mycobacterial infections of the musculoskeletal system are rare and usually associated with predisposing factors, such as prior joint disease, trauma, use of intraarticular or oral corticosteroids, or an immunocompromised state. A sixty five-year-old patient with rheumatoid arthritis was hospitalized due to swelling on the left wrist. M. intracellulare was cultured from the aspirated joint fluid. The patient was successfully treated with clarithromycin, ethambutol, and rifampin. We report this case with review, emphasizing high suspicion for nontuberculous mycobacterial infection in patients with predisposing risk factors.

A Case of Tenosynovitis Due to Mycobacterium intracellulare in a Patient with Rheumatoid Arthritis / 감염과화학요법

Nontuberculous mycobacteria are ubiquitous organisms that are frequently present in the water, soil and animal reservoirs. Nontuberculous mycobacterial infections of the musculoskeletal system are rare and usually associated with predisposing factors, such as prior joint disease, trauma, use of intraarticular or oral corticosteroids, or an immunocompromised state. A sixty five-year-old patient with rheumatoid arthritis was hospitalized due to swelling on the left wrist. M. intracellulare was cultured from the aspirated joint fluid. The patient was successfully treated with clarithromycin, ethambutol, and rifampin. We report this case with review, emphasizing high suspicion for nontuberculous mycobacterial infection in patients with predisposing risk factors.

A case of recurrent Kikuchi-Fujimoto disease with SLE / 대한내과학회지

Kikuchi-Fujimoto disease (KFD) is a rare self-limiting necrotizing lymphadenitis found mainly in young women. Patients typically present with lymphadenopathy and often with a high temperature. The etiology of the disease remains unknown, but various viral infection and autoimmune processes have been postulated to be the cause. One theory proposes that KFD may be a self-limiting form of systemic lupus erythematosus (SLE). This theory is strongly supported by the fact that microscopic features of KFD can be very similar to those found in lupus lymphadenitis. The diagnosis of KFD can precede, postdate or coincide with the diagnosis of SLE. No treatment is usually needed and relapse, although possible, is uncommon. Our case describes a young woman, originally diagnosed as having SLE, who subsequently developed recurrent KFD with axillary lymphadenopathy and fever.

A Case of Acute Leukoencephalopathy in a Patient with Systemic Lupus Erythematosus / 대한류마티스학회지

Up to 70% of patients with systemic lupus erythematosus (SLE) are afflicted with neurologic manifestations. However, there are only a few reports documenting acute leukoencephalopathy in SLE. We describe a 20-year-old woman who was recently diagnosed as SLE, suffering from headache, fever and arthritis. She developed an acute onset of consciousness disturbance with seizure followed by prolonged coma, which recovered completely after 1 month of steroid therapy. Her brain MRI showed diffuse high signal intensity in the periventricular and subcortical white matter on T2-weighted and FLAIR images, whereas cerebral cortex, basal ganglia, and thalamus were spared. Acute leukoencephalopathy may be recognized as a subtype of lupus involving the central nervous system.

Expression of Cell Surface Marker on Human Bone Marrow Derived Stromal Cells during Chondrogenic Differentiation / 대한류마티스학회지

OBJECTIVE: Multipotent bone marrow stromal cells have the ability to differentiate toward a variety of connective tissue lineages including cartilage. The future use of adult mesenchymal stem cells (MSCs) for human therapies depends on the establishment of preclinical studies. Therefore, in this preclinical study we demonstrated the expression of MSC surface markers CD29, CD105, and CD44 on human bone marrow derived stromal cells during chondrogenic differentiation. METHODS: Adult human bone marrow was collected from the iliac crest of 7 donors following informed consent. Mononuclear cells were isolated, incubated in monolayers, and embedded in alginate beads for three-dimensional cultures. Cellualr viability was assessed by MTT assay. Flow cytometry of alginate bead cultures was performed on days 0, 7, 14, 21, and 28 using monoclonal antibody against surface molecules, CD105, CD29, CD44, CD34 and CD45. Total contents of collagen and glycosaminoglycan (GAG) of the alginate beads was measured. SPSS 11.0 was used for data analysis. RESULTS: After 7 days of culture, 89% of the cells expressed the human integrin beta 1 antibody, CD29. The CD29-positive cells remained elevated at 83% on days 28. However, while only 18% expressed the type II TGF-beta receptor endoglin, CD105 on day 7, the CD105-positive cells increased abruptly 65% on day 14 remaining elevated up to day 28. The expression of CD44 was maximal in the first passage cell (63%). High concentration of TGF-beta 3 (10 ng/mL) was more favorable for sustaining cell viability than a low concentration (0.5 ng/mL)(n=4, p= 0.002, day 21). The total contents of collagen and GAG in the MSC-alginate beads increased during the three-dimensional culture (n=4, p=0.02, p=0.006) suggesting its differentiation into a chondrogenic lineage. CONCLUSION: CD29 was expressed earlier than CD105 during chondrogenic differentiation of human bone marrow MSC. CD44 expression was highest in the first passage cells and gradually decreased afterwards.

Combination treatment with leflunomide and methotrexate in patients with rheumatoid arthritis: the efficacy, safety, and predisposing factors for treatment response / 대한내과학회지

BACKGROUND: Leflunomide, a novel immunoregulatory drug, has been shown to be effective in rheumatoid arthritis (RA) as monotherapy and as combination therapy with methotrexate (MTX). The aims of this study were to investigate the efficacy and safety of combination therapy with leflunomide and MTX in active RA patients and to identify the patients with a better response to this combination. METHODS: The patients received a maintenance dose of 20 mg of leflunomide with or without a loading dose. Parameters for disease activity in RA were measured at baseline and at 12 and 24 weeks after initiation of leflunomide. At 24 weeks, the baseline data from the patients classified as leflunomide responders were compared with data from nonresponders and analyzed to determine the potential predisposing factors for treatment response. RESULTS: A total of 103 patients with RA were included and 93 (90.3%) patients received leflunomide for 24 weeks. At 24 weeks, 67 (65.1%) patients were DAS28 responders; 14 (13.6%) were good responders and 53 (51.5%) moderate responders. At 12 weeks, significant improvements were noticeable in the individual efficacy measures of diseases activity. There were also significant improvements between 12 and 24 weeks in swollen joint count, tender joint count, HAQ disability index, and patients' and physicians' global assessments of diseases activity; but no further improvements in ESR or CRP could be seen after the first 12 weeks. When comparing the baseline data from responders with the nonresponders, patients on a higher MTX dose and patients with a higher disease activity at baseline responded better to leflunomide. However, age, sex, disease duration of RA, functional status, loading dosage of leflunomide, and previous number of DMARDs used did not affect the patients' response to leflunomide. CONCLUSION: Combination therapy with leflunomide and MTX is effective and safe across a wide range of patients, especially those with a high disease activity in spite of treatment with other traditional DMARDs.

The Prevalence of Metabolic Syndrome in Patients with Gout: A Multicenter Study

It has been suggested that hyperuricemia and possibly gout are associated with the metabolic syndrome, but there have been no direct studies. This study was undertaken to obtain the prevalence of the metabolic syndrome in patients with gout and to compare it with those from the general population studies. This was a 4-institutional case-historical control study composed of 168 patients with gout. We assessed the prevalence of metabolic syndrome according to the ATP III criteria and compared the prevalence with that of the historical controls. To elucidate the factors in gout that were associated with metabolic syndrome, a multivariate analysis was done. The age-adjusted prevalence of metabolic syndrome in gout patients was 43.6%, which was significantly higher than that of the Korean control population (5.2%) from the previous studies. Patients with gout had more components of metabolic syndrome than did the controls. Body mass index (BMI, OR=1.357 (95%CI 1.111-1.657)) and high density lipoprotein (HDL, OR=0.774 (95%CI 0.705-0.850)) were the variables most significantly associated with the occurrence of metabolic syndrome in gout, but alcohol consumption did not show such associations. Gout is associated with the metabolic syndrome, and furthermore, obesity and dyslipidemia were the factors most associated with the syndrome in these patients.

A Case of Overlap Syndrome of Dermatomyositis and Sjogren's Syndrome associated with IgA Nephropathy / 대한류마티스학회지

An "overlap syndrome" is used to describe patients who have two or more well-defined connective tissue diseases such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and polymyositis. Their coexistence is defined clinically and often by specific serologic tests. We report a case of dermatomyositis associated with IgA nephropathy that had overlapping features of Sjogren's syndrome. This dermatomyositis and Sjogren's overlap is a rarely reported overlap syndrome worldwide.

A Case of Relapsing Polychondritis Associated with Myelodysplastic Syndrome with Erythroid Hypoplasia/Aplasia

Relapsing polychondritis (RP) is a rare multisystem disorder. Myelodysplastic syndrome (MDS) with erythroid hypoplasia/aplasia is a rare form of myelodysplasia. Several cases of RP associated with MDS have recently been described. However, RP associated with MDS with erythroid hypoplasia/aplasia has never been reported. There was only one case report of polymyalgia rheumatica associated with MDS with erythroid hypoplasia/aplasia. In this study, we report a 79-year-old patient with RP, who developed MDS subtype refractory anemia (RA) with erythroid hypoplasia/aplasia, a very characteristic subtype of MDS.

A Case of Mallory-Weiss Syndrome Complicating Pregnancy in a Patient with Scleroderma

The majority of patients with scleroderma have gastrointestinal involvement, and a few experience gastrointestinal hemorrhage, however, gastrointestinal hemorrhage due to Mallory-Weiss syndrome is very rare. We report upon a 24-year-old pregnant woman with scleroderma who had gastrointestinal hemorrhage due to Mallory-Weiss syndrome.

Spontaneous Aortic Dissection in a Patient with Neurofibromatosis

Neurofibromatosis type 1 (NF-1), or von Recklinhausen's disease, is an autosomal dominant disorder that primarily affects the skin, the central and peripheral nervous systems, and the skeleton. Vasculopathy, such as, aneurysm or stenosis of large vessels is a less common manifestation of NF-1, but spontaneous aortic dissection in NF-1 is very rare. We report a case of acute aortic dissection with incidental aneurysm of the right femoral artery in NF-1.

A Case of Pulmonary Alveolar Proteinosis Associated with Pulmonary Tuberculosis / 결핵

Pulmonary alveolar proteinosis(PAP) is a disorder in which an insoluble, proteinaceous material, rich in phospholipids, is deposited in the alveoli and bronchioles. The deficiency in the clearance and degradation of the i ntra-alveolar phospholipoproteinaceous material in PAP most likely represents a dysfunction of the type II pneumocytes. Although the pathogenesis and causative treatment of PAP is unclear a whole lung bronchopulmonary lavage is a relatively safe and effective treatment. Here we experienced a case of pulmonary alveolar proteinosis in a 62 year old female patient who had pulmonary tuberculosis approximately 20 years ago. She complained of aggravated dyspnea and chronic cough, and presented fine inspiratory crackles at both lung fields. diffuse ground glass opacity with some area of consolidation and smooth interlobular septal thickenings in both upper, right middle lobes, and a portion of right lower lobe. Optical microscopy of the lung tissue obtained by an open lung biopsy many granulomas containing acid-fast smear positive bacilli and diffuse homogeneous PAS-positive fluid in the alveolar space. Immunohistochemical stain showed surfactant A in the alveolar space. Antituberculosis drugs with bronchoalveolar lavage were used to treat the disease. There after she showed improvement in her symptoms and a partial improvement in the chest X-ray and HRCT findings. We present a case of PAT associated with pulmonary tuberculosis.

A Case of Pulmonary Alveolar Proteinosis Associated with Pulmonary Tuberculosis / 결핵

Pulmonary alveolar proteinosis(PAP) is a disorder in which an insoluble, proteinaceous material, rich in phospholipids, is deposited in the alveoli and bronchioles. The deficiency in the clearance and degradation of the i ntra-alveolar phospholipoproteinaceous material in PAP most likely represents a dysfunction of the type II pneumocytes. Although the pathogenesis and causative treatment of PAP is unclear a whole lung bronchopulmonary lavage is a relatively safe and effective treatment. Here we experienced a case of pulmonary alveolar proteinosis in a 62 year old female patient who had pulmonary tuberculosis approximately 20 years ago. She complained of aggravated dyspnea and chronic cough, and presented fine inspiratory crackles at both lung fields. diffuse ground glass opacity with some area of consolidation and smooth interlobular septal thickenings in both upper, right middle lobes, and a portion of right lower lobe. Optical microscopy of the lung tissue obtained by an open lung biopsy many granulomas containing acid-fast smear positive bacilli and diffuse homogeneous PAS-positive fluid in the alveolar space. Immunohistochemical stain showed surfactant A in the alveolar space. Antituberculosis drugs with bronchoalveolar lavage were used to treat the disease. There after she showed improvement in her symptoms and a partial improvement in the chest X-ray and HRCT findings. We present a case of PAT associated with pulmonary tuberculosis.

Sequential Development of Systemic Lupus Erythematosus in a Patient with Juvenile Rheumatoid Arthritis / 대한류마티스학회지

Overlap syndrome is used to describe patients who have two or more well-defined connective tissue diseases. Although a variety of overlap syndromes are now recognized, the coexistence of the progression of juvenile rheumatoid arthritis (JRA) to systemic lupus erythematosus (SLE) is uncommon. We describe a patient who had typical deforming polyarthritis, who years later developed SLE.

A case of adult extrarenal Wilms' tumor originated from retroperitoneum / 대한내과학회지

Wilms' tumor is the most common malignant abdominal tumor in the adolescent and pediatric period, whereas adult Wilms' tumor is uncommon. Wilms' tumor often originates in the kidney. Extrarenal Wilms' tumor is rare and has been considered "unstageable". Therefore, treatment and long-term survival have not been uniformly reported. We report a 24 year-old female who developed extrarenal Wilms' tumor, in the retroperitoneal space. She was misdiagnosed for ovarian neoplasm and underwent debulking operation, by which Wilms' tumor was confirmed. Palliative chemotherapy and radiotherapy were performed.

A Case of Malignant Lymphoma during Treatment with Methotrexate for Rheumatoid Arthritis / 대한류마티스학회지

Rheumatoid arthritis (RA)is a multisystemic inflammatory disease with a prevalance of approximately 1%of the population.The use of disease modifying anti-rheumatic drug (DMARD)is an essential regimen for the treatment of RA. Among DMARDs,methotrexate (MTX)is used worldwide with confirmed effectiveness.However,cytopenia,pulmonary injury,and hepatic toxicity are a few side effects limiting its use.In addition,although the oncogenic potential of MTX is low,several cases have been reported.Recently an increased risk of developing lymphoproliferative disorders has been reported in patients with RA. The incidence is higher in elderly individuals with severe longstanding seropositive RA,those with Sjogren's or Felty's syndrome,and in patients with RA treated with prolonged low dose MTX.We describe a case of RA who developed non-Hodgkin's lymphoma during low dose MTX therapy.