Musculoskeletal Manifestation in Inflammatory Bowel Disease / 대한소화기학회지

Almost 50% of patients with inflammatory bowel disease (IBD) exhibit at least one extra-intestinal manifestation in their lifetime. Extra-intestinal manifestations of IBD are often associated with the intestinal disease activity, reducing the quality of life of the patient but rarely leading to fatal complications. Musculoskeletal involvement is the most frequent extra-intestinal manifestation of patients with IBD but this rarely occurs before IBD is diagnosed. They are manifested in various forms, such as arthropathy, fibromyalgia, and osteoporosis. Therefore, a multidisciplinary team approach including gastroenterologists and rheumatologists are necessary for optimal treatment. This review focuses on the diagnosis and treatment of musculoskeletal manifestations of IBD from the perspectives of rheumatologists who can assist gastroenterologists.

Musculoskeletal Manifestation in Inflammatory Bowel Disease / 대한소화기학회지

Almost 50% of patients with inflammatory bowel disease (IBD) exhibit at least one extra-intestinal manifestation in their lifetime. Extra-intestinal manifestations of IBD are often associated with the intestinal disease activity, reducing the quality of life of the patient but rarely leading to fatal complications. Musculoskeletal involvement is the most frequent extra-intestinal manifestation of patients with IBD but this rarely occurs before IBD is diagnosed. They are manifested in various forms, such as arthropathy, fibromyalgia, and osteoporosis. Therefore, a multidisciplinary team approach including gastroenterologists and rheumatologists are necessary for optimal treatment. This review focuses on the diagnosis and treatment of musculoskeletal manifestations of IBD from the perspectives of rheumatologists who can assist gastroenterologists.

Standardized, musculoskeletal ultrasonographic reference values for healthy Korean adults

BACKGROUND/AIMS@#To define standard reference values for musculoskeletal ultrasonography (MSUS) in Korea.@*METHODS@#A total of 251 healthy adults were recruited for this study. Ultrasonography was performed by experienced rheumatologists who had undergone four appropriate training programs in Korea. A General Electric LOGIQ electronic ultrasound device fitted with a 12 MHz linear transducer was employed. Mean values ± standard deviations (SDs) were defined as standard reference values. Intraclass correlation coefficients was employed to evaluate the extent of inter- and intraobserver agreement when MSUS measurements were made.@*RESULTS@#The 251 study participants included 122 males. Mean subject age was 28.6 years. The average bone-to-capsule distance of the right-side second and third metacarpophalangeal (MCP) joints were 0.68 and 0.72 mm respectively, and those of the left-side joints 0.62 and 0.68 mm. The cartilage thicknesses of the right-side second and third MCP joints were 0.55 and 0.55 mm, and those of the left-side joints were 0.55 and 0.56 mm, respectively. The bone-to-capsule distances of the right and left wrists were 0.80 and 0.82 mm. In 12.4% of participants (31/251), the erosion score of the humeral head was 1.71. In the right-side knee joint, mean cartilage thicknesses of the medial and lateral condyles were 1.86 and 2.03 mm in longitudinal scans. High overall interobserver agreement was evident after appropriate training that included instruction on standard MSUS methodology.@*CONCLUSIONS@#We defined standard reference values for MSUS in healthy Korean adults. The reliabilities of interobserver agreements were high after appropriate training program.

Systemic sclerosis due to crystalline silica exposure among jewelry workers in Korea: two case reports

BACKGROUND: Occupational exposure to crystalline silica is a potential risk factor for various systemic autoimmune diseases including systemic sclerosis. The etiology of systemic sclerosis is not conclusively known, but there are epidemiological studies that show the relationship between exposure to crystalline silica and risk of systemic sclerosis. Here we report, for the first time, two cases of crystalline silica-related systemic sclerosis in patients who worked in crystal processing in the jewelry-manufacturing field. CASE PRESENTATION: Case 1 is a 57-year-old man who had worked mainly in crystal processing for multiple jewelry-processing companies for 17 years, since the age of 15 years. He contracted tuberculosis at the age of 25 years and showed Raynaud's phenomenon of both the hands and feet at age 32 years. Digital cyanosis and sclerosis developed at approximately age 41 years. The patient was diagnosed with systemic sclerosis at age 48 years. Case 2 is a 52-year-old man who worked in crystal processing for various jewelry-processing companies for 7 years, since the age of 23 years. He first showed signs of cyanosis in the third and fourth digits of both hands at age 32 years, was diagnosed with Raynaud's syndrome at age 37 years, and was diagnosed with systemic sclerosis at age 38 years. Crystal processing is a detailed process that involves slabbing and trimming the selected amethyst and quartz crystals, which requires close proximity of the worker's face with the target area. In the 1980s and 1990s, the working hours were 12 h per day, and the working environment involved 15 workers crowded into a small, 70-m2 space with poor ventilation. CONCLUSION: Two workers who processed crystals with a maximum crystalline silica content of 56.66% developed systemic sclerosis. Considering the epidemiological and experimental evidence, exposure to crystalline silica dust was an important risk factor for systemic sclerosis. An active intervention is necessary to reduce exposure in similar exposure groups in the field of jewelry processing.

Cyclophosphamide therapy for secondary amyloidosis in a patient with juvenile idiopathic arthritis unresponsive to tumor necrosis factor α inhibitor therapy

No abstract available.

Genetic analysis of ABCG2 and SLC2A9 gene polymorphisms in gouty arthritis in a Korean population

BACKGROUND/AIMS: Gout is a common inf lammatory arthritis triggered by the crystallization of uric acid in the joints. Serum uric acid levels are highly heritable, suggesting a strong genetic component. Independent studies to confirm the genetic associations with gout in various ethnic populations are warranted. We investigated the association of polymorphisms in the ABCG2 and SLC2A9 genes with gout in Korean patients and healthy individuals. METHODS: We consecutively enrolled 109 patients with gout and 102 healthy controls. The diagnosis of gout was based on the preliminary criteria of the America College of Rheumatology. Genomic DNA was extracted from whole blood samples. We identified single nucleotide polymorphism (SNP) changes in the ABCG2 and SLC2A9 genes using a direct sequencing technique. rs2231142 in ABCG2 and rs6449213 and rs16890979 in SLC2A9 and nearby regions were amplified by polymerase chain reaction. RESULTS: Patients with gout had significantly higher A/A genotype (29.3% vs. 4.9%, respectively) and A allele (52.8% vs. 26.5%, respectively) frequencies of rs2231142 in ABCG2 than did controls (chi2 = 29.42, p G and c.1002+78G>A) in the SLC2A9 gene. The univariate logistic regression analysis revealed that the c.881A>G and c.1002+78G>A SNPs were significantly higher in patients than in controls. CONCLUSIONS: We demonstrated a significant association between rs2231142 in the ABCG2 gene and gout and identified novel SNPs, c.881A>G and c.1002+78G>A, in the SLC2A9 gene that may be associated with gout in a Korean population.

Unilateral Pedicle Fracture Accompanying Spondylolytic Spondylolisthesis

Unilateral pedicle stress fracture accompanying spondylolytic spondylolisthesis is rare even in the elderly. Most are associated with major trauma, previous spine surgery, or stress-related activity. Here, the authors describe an unique case of unilateral pedicle fracture associated with spondylolytic spondylolisthesis at the L5 level, which was successfully treated by posterior lumbar interbody fusion with screw fixation at the L5-S1 level. As far as the authors' knowledge, no such case has been previously reported in the literature. The pathophysiological mechanism of this uncommon entity is discussed and a review of relevant literature is included.

The Ultrasonographic Features of Periarticular Diseases / 대한내과학회지

Ultrasonography (US) is a cost effective diagnostic imaging tool suitable for routine clinical use. High frequency US is a well-established method for both the diagnosis and monitoring of inflammatory arthritis, capable of detecting a wide range of joint pathologies, including effusion, synovitis, and extra-articular abnormalities. However, to ensure an accurate diagnosis, it is also important to consider periarticular disorders that could clinically mimic joint pathology. Such a diagnosis requires special attention to be paid to the periarticular structures, include the tendon, ligament, muscle, and bursa. Due to the close proximity of these structures to potentially affected joints, periarticular inflammation is often clinically misdiagnosed as articular in origin. In these cases, the processes driving pathology are the result of localized inflammation that is secondary to inflammatory arthritis, such as excessive friction or direct trauma. Therefore, when using US to diagnose inflammatory arthritis, it is important to understand the causative pathology of the periarticular disorders, as well as their clinical presentation.

Brown-Sequard Syndrome Caused by a Cervical Synovial Cyst

Synovial cysts are recognized as an uncommon cause of radicular and myelopathic symptoms. They are most frequently found in the lumbar region. The cervical spine or cervicothoracic junction is a rare location for a degenerative intraspinal synovial cyst as compared with the lumbar spine. At given cervical spinal levels, synovial cysts probably share clinical features with disc herniation and stenosis. However, the pathogenesis of synovial cysts remains still controversial. Here, we report a rare case of a synovial cyst in the lower cervical spine presented as Brown-Sequard syndrome and include a brief review of the literature. To the best of our knowledge, no previous report has been issued in the English literature on a synovial cyst presenting with Brown-Sequard syndrome. Neurologic function recovered completely after complete removal of the cyst and expansive laminoplasty.

A Case of Sick Sinus Syndrome in a Patient with Systemic Sclerosis / 대한내과학회지

Systemic sclerosis (SSc) is characterized by the presence of microvascular disease and various patterns of cutaneous and parenchymal fibrosis. Manifestation of SSc may occur in numerous tissues and organs and can be particularly problematic when present in the lungs, kidneys or heart. Cardiac involvement in SSc includes pericarditis, myocardial disease, conduction abnormalities, and arrhythmia. Sick sinus syndrome is described as a combination of symptoms (dizziness, confusion, fatigue, syncope and congestive heart failure) caused by sinus node dysfunction and manifested by marked sinus bradycardia, sinoatrial block, or sinus arrest. Sinus node dysfunction is most often seen in the elderly but also, rarely, in systemic amyloidosis and connective tissue disease. Sick sinus syndrome is rarely found in patients with SSc, of which there has been only one case report, according to a review of the literature. Therefore, we report a case of sick sinus syndrome in a 71-year-old female with SSc.

Clinical Analysis of Microscopic Removal of Discal Cyst

OBJECTIVE: The purpose of this study was to evaluate the clinical presentation and surgical outcome in patients with symptomatic discal cyst. METHODS: The authors reviewed consequent 9 patients in whom microscopic excision of the discal cyst with or without additional discectomy for discal cyst from 2005 to 2012. Diagnostic imagings including simple radiographs, computed tomography with discogram and magnetic resonance images were performed in each case. The patients were reviewed to evaluate the clinical presentation, surgical outcome and related complications. RESULTS: In all patients, discal cyst was located in the lumbar region and they presented with back pain and unilateral radiating pain. The preoperative magnetic resonance images (MRI) and computed tomography (CT) scan with discogram showed a connection between the cyst and the involved intervertebral disc. All patients obtained immediate relief of symptoms after microscopic excision of discal cyst. There were no recurrent lesions during follow-up period. The mean preoperative visual analogue scale (VAS) was 7.8 when compared with 2.6 in preoperative assessment. All patients obtained excellent or good outcome according to modified MacNab's criteria. CONCLUSION: Discal cysts are rare lesions that can lead to back pain and refractory sciatica. Microscopic excision of the cyst can achieve remarkable improvement of symptoms.

A Case of Psoriasiform Dermatitis Followed by Tumor Necrosis Factor Inhibitor Treated with Phototherapy

Although tumor necrosis factor (TNF)-alpha antagonist is a successful treatment modality for various autoimmune diseases, including rheumatoid arthritis (RA), ankylosing spondylitis and psoriatic arthritis, many adverse effects have been reported. Cutaneous adverse reactions of TNF-alpha antagonist include skin rash, urticaria, lupus like rash, seborrheic dermatitis and different kinds of psoriasiform dermatitis. We report a case of psoriasiform dermatitis during TNF-alpha antagonist treatment in a 50-year-old woman with RA. The patient has been treated with adalimumab. After 2 months, she developed pruritic erythematous eruption and desquamative lesions on the head and limbs, which were defined as psoriasiform change by a skin biopsy. These skin lesions are successfully treated with combination therapy, including cessation of adalimumab, corticosteroid and phototherapy.

Diffuse Alveolar Hemorrhage after Adalimumab Treatment of Rheumatoid Arthritis / 대한내과학회지

Diffuse alveolar hemorrhage (DAH) is a life-threatening condition involving hemorrhage originating in the pulmonary microvasculature. The clinical syndrome is characterized by hemoptysis, hypoxemic respiratory failure, low-grade fever, and diffuse pulmonary infiltrates. The majority of cases of DAH are caused by pulmonary renal syndromes, connective tissue disorders, and drugs. Here, we present a case of DAH associated with adalimumab treatment in a patient with rheumatoid arthritis.

A Case of Refractory Behcet's Uveitis Improving after Insertion of Fluocinolone Acetonide Implant

Behcet's disease (BD) is systemic vasculitis that can manifest severely debilitating. Despite the understanding mechanisms of overall BD, there are remains many questions in various critical manifestations and treatments. The ocular manifestation is characterized by a prototype of chronic relapsing and persistent uveitis. The main treatment is topical corticosteroid, and topical nonsteroidal anti-inflammatory drugs in mild uveitis. The recurrent and severe uveitis could be treated with ocular corticosteroid injections, and systemic corticosteroid for inducing long-lasting suppression of the inflammation. Systemic corticosteroids should rapidly be tapered within weeks for avoiding side effects. Recent advances have led to the development of sustained-release corticosteroid devices using different corticosteroids. We present a case of 67-year-old woman who received a fluocinolone acetonide implant for recurrent Behcet's uveitis. She was successfully treated with implant and the uveitis became quiescent within a month.

A Case of Gouty Arthritis Involving the Hip Joint Diagnosed Using Ultrasonography / 대한내과학회지

Gout is a clinical syndrome resulting from the deposition of monosodium urate monohydrate. Gouty arthritis is characterized by hyperuricemia and recurrent attacks of typical mono-or polyarthritis. Urate deposition frequently involves the articular cartilage, subchondral bone, synovial membrane, joint capsule, and periarticular tissues. The first metatarsophalangeal joint is most commonly involved, and other frequently affected joints include the ankle, knee, and tarsal area. However, the hip joint is very rarely affected. We herein report a case of gouty arthritis presenting with hip synovitis diagnosed using ultrasonography.

A Case of Sweet's Syndrome Presenting as Subdermal Cystic Lesions and Arthritis / 대한내과학회지

Sweet's syndrome is characterized by a combination of clinical and pathologic findings including fever, neutrophilia, tender erythematous skin lesions, and a diffuse infiltration of mature neutrophils in the upper dermis. Numerous diseases and clinical manifestations have been associated with the disease; however, Sweet's syndrome associated with subdermal cystic skin lesions and arthritis is rare. A 71-year-old female patient presented with fever, erythematous plaques, multiple hypoglossal ulcers, and arthritis in both ankles. The skin lesions were variously sized areas of erythematous swelling on the forehead, back, and left shoulder. Musculoskeletal sonography revealed hypervascularity and a subdermal cyst in the erythematous plaque on her back. The results of a skin biopsy indicated the presence of mature neutrophilic infiltration in the dermis and thus led to the diagnosis of Sweet's syndrome. We herein present an unusual case of Sweet's syndrome presenting as erythematous subdermal cystic lesions, multiple hypoglossal ulcers, and bilateral ankle arthritis with a literature review.

Usefulness and Limitation of 2010 ACR/EULAR Classification Criteria in Korean Patients with Early RA

OBJECTIVE: The 2010 New American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria for rheumatoid arthritis (RA) was raised to identify patients with early RA and replaced the 1987 ACR classification criteria. The aims of this study are to assess the availability of new classification criteria and to evaluate its potential limitation. METHODS: A total of 408 patients with newly diagnosed RA were included from 13 secondary or tertiary hospitals in South Korea. The symptom duration was less than 12 months before the diagnosis of RA. RA was defined as either 1987 ACR classification criteria or new 2010 ACR/EULAR criteria. We compared the full details of both classification criteria. RESULTS: The mean symptom duration was 5.1 months. The majority (76.2%) of the patients were female. Two hundred and seventy three patients (66.9%) fulfilled both of the 2010 and 1987 classification criteria. Forty-seven (14.7%) of the 320 patients fulfilling the 1987 criteria did not fulfill the new classification criteria. On the other hand, eighty-eight (24.4%) of the 361 patients fulfilling the 2010 ACR/EULAR classification criteria did not fulfill the 1987 ACR criteria. Thirty-six (55.4%) of the 65 patient with seronegative RA failed to meet the 2010 classification criteria. In case of seropositive RA (n=343), 85 additional patients (24.8%) could be diagnosed as RA using new classification criteria. CONCLUSION: The new 2010 ACR/EULAR classification criteria enable physicians to diagnose more patients with early RA via the help of serology. However, the sensitivity for the diagnosis of seronegative RA is projected to decrease.

Neuropsychiatric Lupus Presenting as a Thalamic Infarct in a Male Patient / 대한내과학회지

Systemic lupus erythematosus (SLE) is an autoimmune disease that can manifest systemically in many organs. It is most common in women of child-bearing age. Neuropsychiatric SLE is characterized by central nervous system (CNS) symptoms. CNS manifestations of SLE have been discovered in all parts of the brain, although thalamic infarcts associated with SLE are rare, especially in males. Here, we report a thalamic infarction in a 22-year-old male SLE patient.

A Case of Wegener's Granulomatosis Presenting as a Diplopia

Wegener's granulomatosis (WG) is a multisystemic granulomatous inflammatory disorder which is presumably caused by an autoimmune response. It typically targets the upper and lower respiratory tract and the kidney. Ocular involvement occurs in 50 to 60% of WG patients. However, orbital myositis and diplopia as a presenting symptom is a rare ocular manifestation. We present the case of a 49-year-old man who was admitted with bilateral diplopia due to orbital myositis under a diagnosis of WG. He was successfully treated with high dose corticosteroid therapy.

Prognostic Factors and Scoring Systems for Non-Small Cell Lung Cancer Patients Harboring Brain Metastases Treated with Gamma Knife Radiosurgery / 결핵및호흡기질환

BACKGROUND: The survival of non-small cell lung cancer (NSCLC) patients with brain metastases is reported to be 3~6 months even with aggressive treatment. Some patients have very short survival after aggressive treatment and reliable prognostic scoring systems for patients with cancer have a strong correlation with outcome, often supporting decision making and treatment recommendations. METHODS: A total of one hundred twenty two NSCLC patients with brain metastases who received gamma knife radiosurgery (GKRS) were analyzed. Survival analysis was calculated in all patients for thirteen available prognostic factors and four prognostic scoring systems: score index for radiosurgery (SIR), recursive partitioning analysis (RPA), graded prognostic assessment (GPA), and basic score for brain metastases (BSBM). RESULTS: Age, Karnofsky performance status, largest brain lesion volume, systemic chemotherapy, primary tumor control, and medication of epidermal growth factor receptor tyrosine kinase inhibitor were statistically independent prognostic factors for survival. A multivariate model of SIR and RPA identified significant differences between each group of scores. We found that three-tiered indices such as SIR and RPA are more useful than four-tiered scoring systems (GPA and BSBM). CONCLUSION: There is little value of RPA class III (most unfavorable group) for the same results of 6-month and 1-year survival rate. Thus, SIR is the most useful index to sort out patients with poorer prognosis. Further prospective trials should be performed to develop a new molecular- and gene-based prognostic index model.