A Case Report of SAPHO Syndrome Treated with Oral Alendronate

SAPHO syndrome, characterized by synovitis, acne, pustulosis, hyperostosis, and osteitis is rare compared to other spondyloarthropathies. It is also difficult to diagnose, and treatment methods have not yet been fully identified. Approximately 72% of patients are diagnosed with at least one other disease before a final diagnosis of SAPHO syndrome. In addition, SAPHO syndrome is subject to a delayed diagnosis period of 4.5 to 9.1 years. Medications such as non-steroidal anti-inflammatory drugs, disease-modifying anti-rheumatic drugs, and tumor necrosis factor inhibitors are used in treatment of SAPHO syndrome. Bisphosphonate is also used for refractory SAPHO syndrome; however, most reports on this relate to intravenous injection of medication. The authors experienced and subsequently reported on a case involving a patient with SAPHO syndrome accompanied by fracture and infection of the left second finger who was treated with the oral biphosphonate, alendronate.

Early effects of tumor necrosis factor inhibition on bone homeostasis after soluble tumor necrosis factor receptor use

BACKGROUND/AIMS: Our aim was to assess whether short-term treatment with soluble tumor necrosis factor (TNF) receptor affects circulating markers of bone metabolism in rheumatoid arthritis (RA) patients. METHODS: Thirty-three active RA patients, treated with oral disease-modifying antirheumatic drugs (DMARDs) and glucocorticoids for > 6 months, were administered etanercept for 12 weeks. Serum levels of bone metabolism markers were compared among patients treated with DMARDs at baseline and after etanercept treatment, normal controls and naive RA patients not previously treated with DMARDs (both age- and gender-matched). RESULTS: Bone-specific alkaline phosphatase (BSALP) and serum c-telopeptide (CTX)-1 levels were lower in RA patients treated with DMARDs than in DMARD-naive RA patients. After 12 weeks of etanercept treatment, serum CTX-1 and sclerostin levels increased. In patients whose DAS28 improved, the sclerostin level increased from 1.67 +/- 2.12 pg/mL at baseline to 2.51 +/- 3.03 pg/mL, which was statistically significant (p = 0.021). Increases in sclerostin levels after etanercept treatment were positively correlated with those of serum CTX-1 (r = 0.775), as were those of BSALP (r = 0.755). CONCLUSIONS: RA patients treated with DMARDs showed depressed bone metabolism compared to naive RA patients. Increases in serum CTX-1 and sclerostin levels after short-term etanercept treatment suggest reconstitution of bone metabolism homeostasis.

Digital Thermography of the Fingers and Toes in Raynaud's Phenomenon

The aim of this study was to determine whether skin temperature measurement by digital thermography on hands and feet is useful for diagnosis of Raynaud's phenomenon (RP). Fifty-seven patients with RP (primary RP, n = 33; secondary RP, n = 24) and 146 healthy volunteers were recruited. After acclimation to room temperature for 30 min, thermal imaging of palmar aspect of hands and dorsal aspect of feet were taken. Temperature differences between palm (center) and the coolest finger and temperature differences between foot dorsum (center) and first toe significantly differed between patients and controls. The area under curve analysis showed that temperature difference of the coolest finger (cutoff value: 2.2degrees C) differentiated RP patients from controls (sensitivity/specificity: 67/60%, respectively). Temperature differences of first toe (cutoff value: 3.11degrees C) also discriminated RP patients (sensitivity/specificity: about 73/66%, respectively). A combination of thermographic assessment of the coolest finger and first toe was highly effective in men (sensitivity/specificity : about 88/60%, respectively) while thermographic assessment of first toe was solely sufficient for women (sensitivity/specificity: about 74/68%, respectively). Thermographic assessment of the coolest finger and first toe is useful for diagnosing RP. In women, thermography of first toe is highly recommended.

Prevention of Comorbidity and Acute Attack of Gout by Uric Acid Lowering Therapy

The object of this study was to evaluate the effect of uric acid lowering therapy in reducing the new development of comorbidities and the frequency of acute attacks in gout patients. We retrospectively reviewed patients who were diagnosed to have gout with at least 3 yr of follow up. They were divided into 2 groups; 53 patients with mean serum uric acid level (sUA) or =6 mg/dL. Comorbidities of gout such as hypertension (HTN), type II diabetes mellitus (DM), chronic kidney disease, cardiovascular disease (CVD) and urolithiasis were compared in each group at baseline and at last follow-up visit. Frequency of acute gout attacks were also compared between the groups. During the mean follow up period of 7.6 yr, the yearly rate of acute attack and the new development of HTN, DM, CVD and urolithiasis was lower in the adequately treated group compared to the inadequately treated group. Tight control of uric acid decreases the incidence of acute gout attacks and comorbidities of gout such as HTN, DM, CVD and urolithiasis.

Extensive Thrombosis in a Patient with Familial Mediterranean Fever, Despite Hyperimmunoglobulin D State in Serum: First Adult Case in Korea

Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent episodes of fever accompanied by peritonitis, pleuritis, arthritis, or erysipelas-like erythema. It is known to occur mainly among Mediterranean and Middle Eastern populations such as non-Ashkenazi Jews, Arabs, Turks, and Armenians. FMF is not familiar to clinicians beyond this area and diagnosing FMF can be challenging. We report a 22-yr old boy who presented with fever, arthalgia and abdominal pain. He had a history of recurrent episodes of fever associated with arthalgia which would subside spontaneously or by antipyretics. Autosomal recessive periodic fever syndromes were suspected. Immunoglobulin D (IgD) level in the serum was elevated and DNA analysis showed complex mutations (p.Glu148Gln, p.Pro369Ser, p.Arg408Gln) in the MEFV gene. 3D angio computed tomography showed total thrombosis of splenic vein with partial thrombosis of proximal superior mesenteric vein, main portal vein and intrahepatic both portal vein. This is a case of FMF associated with multiple venous thrombosis and elevated IgD level. When thrombosis is associated with elevated IgD, FMF should be suspected. This is the first adult case reported in Korea.

A Case Report of Renal Involvement in Adult-Onset Still's Disease over Age 70

Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disease characterized by spiking fever, arthralgia, salmon pink rash, neutrophilic leukocytosis, and multi-organ involvement. Although renal involvement may appear in some cases of adult Still's disease, onset over 70 years of age with renal involvement has not been described. We report a 73-years-old woman whose illness manifested with fever of unknown origin, massive proteinuria, and multiple lymph nodes enlargement. With proteinuria of 2,650 mg/day, a renal biopsy was performed, and histopathological evaluation yielded the diagnosis of chronic glomerulonephritis (CGN). After excluding infectious disease, malignancy, and other rheumatic disease, AOSD was diagnosed with symptoms including fever over 39.0degrees C for more than a week, leukocytosis, generalized lymphadenopathy, and negative autoantibodies. Proteinuria and fever were improved markedly by high dose glucocorticoids and methotrexate therapy.

Erratum: Correction of Title

This erratum is being published to correct of title.

Safety and Efficacy Evaluation for the Addition of Either Etanercept or Leflunomide in Korean Rheumatoid Arthritis Patients Inadequately Responding to Methotrexate

OBJECTIVE: To compare the safety and efficacy associated with the addition of etanercept (ETN) with that of leflunomide (LEF) in Korean rheumatoid arthritis (RA) patients, who inadequately respond to methotrexate (MTX) in a randomized, open-label study. METHODS: Twenty-nine subjects suffering moderate to severe RA, despite MTX treatment were randomly assigned to a combination therapy with either ETN or LEF. The primary end-point was the proportion of subjects achieving American College of Rheumatology (ACR20) criteria at week 16. RESULTS: Ninety percent (n=18) of the ETN+MTX group (n=20) and 22.2% (n=2) of the LEF+MTX group (n=9) achieved an ACR20 response (p=0.001). All patients (n=20) in the ETN+MTX group showed moderate or good EULAR response as compared with 55.6% (n=5) in the LEF+MTX group (p=0.012). All of the ETN+MTX subjects completed the study without adverse events. Adverse events occurred in 77.8% (n=7) of cases in the LEF+MTX group; significantly elevated serum AST/ALT levels in 6 subjects and mild neutropenia (ANC < 1,500/microL) in 1 subject. CONCLUSION: The ETN+MTX combination therapy was effective and safe, whereas the LEF+MTX combination therapy resulted in moderate efficacy in only half of the cases, and was accompanied by a high rate of adverse events. Elevated AST/ALT was the most common adverse event causing dose adjustment or discontinuation of therapeutic agent in the LEF+MTX group.

Detection of an Infected N-butyl-2-cyanoacrylate Plug by F-18 FDG PET/CT Scan in a Patient Who Received Endoscopic Intervention for Gastric Variceal Bleeding / 감염과화학요법

Injection of N-butyl-2-cyanoacrylate has been used successfully for treatment of gastric variceal bleeding. Bacteremia after injection of N-butyl-2-cyanoacrylate is well known, however, the method for diagnosis of infected endovascular injected material has remained uncertain. This is the first case reporting use of F-18 FDG PET/CT in detection of the source of infection after control of endoscopic bleeding with N-butyl-2-cyanoacrylate.

Erratum: Figure Correction

No abstract available.

Serum Procalcitonin for Differentiating Bacterial Infection from Disease Flares in Patients with Autoimmune Diseases

Early differentiation between bacterial infections and disease flares in autoimmune disease patients is important due to different treatments. Seventy-nine autoimmune disease patients with symptoms suggestive of infections or disease flares were collected by retrospective chart review. The patients were later classified into two groups, disease flare and infection. C-reactive protein (CRP) and serum procalcitonin (PCT) levels were measured. The CRP and PCT levels were higher in the infection group than the disease flare group (CRP,11.96 mg/dL +/- 9.60 vs 6.42 mg/dL +/- 7.01, P = 0.003; PCT, 2.44 ng/mL +/- 6.55 vs 0.09 ng/mL +/- 0.09, P < 0.001). The area under the ROC curve (AUC; 95% confidence interval) for CRP and PCT was 0.70 (0.58-0.82) and 0.84 (0.75-0.93), which showed a significant difference (P < 0.05). The predicted AUC for the CRP and PCT levels combined was 0.83, which was not significantly different compared to the PCT level alone (P = 0.80). The best cut-off value for CRP was 7.18 mg/dL, with a sensitivity of 71.9% and a specificity of 68.1%. The best cut-off value for PCT was 0.09 ng/mL, with a sensitivity of 81.3% and a specificity of 78.7%. The PCT level had better sensitivity and specificity compared to the CRP level in distinguishing between bacterial infections and disease flares in autoimmune disease patients. The CRP level has no additive value when combined with the PCT level when differentiating bacterial infections from disease flares.

Carrier Woman of Duchenne Muscular Dystrophy Mimicking Inflammatory Myositis

Carrier woman of Duchenne muscular dystrophy (DMD) can mimic the inflammatory myositis in presenting symptoms. Two diseases should be differentiated by the clinical history, muscle biopsy and genetic study. There are few reports in which both histochemical and genetic study showed the possible link of overlapping inflammatory pathophysiology with dystrophinopathy. We report a 40-yr-old woman who presented with subacute proximal muscle weakness and high serum level of creatine kinase. She had a history of Graves' disease and fluctuation of serum liver aminotransferase without definite cause. MRI, EMG and NCV were compatible with proximal muscle myopathy. Muscle biopsy on vastus lateralis showed suspicious perifascicular atrophy and infiltration of mono-macrophage lineage cells complicating the diagnosis. Dystrophin staining showed heterogeneous diverse findings from normal to interrupted mosaic pattern. Multiple ligation probe amplification and X chromosome inactivation test confirmed DMD gene deletion mutation in exon 44 and highly skewed X inactivation.

A case report of alverine-citrate-induced acute hepatitis / 대한간학회지

Alverine citrate is one of the most commonly used antispasmodic drugs for patients with irritable bowel syndrome. Alverine-citrate-induced hepatotoxicity is extremely rare, with only a few cases having been reported worldwide. We present a case of a 75-year-old female patient who experienced complicated jaundice and abdominal discomfort after taking alverine citrate. Other causes of hepatitis were ruled out and the results of the liver function test returned to normal after ceasing the drug. This is the first case report in Korea of alverine-citrate-induced hepatotoxicity.

NK-T cell lymphoma manifesting as acute respiratory distress syndrome / 대한내과학회지

Primary pulmonary lymphoma is a rare disease, and non-B cell lymphomas (T-cell and natural killer cell lymphomas) involving the lung parenchyma are uncommonly reported. The most common radiological feature of pulmonary parenchymal lymphoma is a single mass or nodule. A 49-year-old woman with dyspnea was referred with suspicion of severe pneumonia. A chest radiograph showed diffuse nodular infiltration in both lungs. Acute respiratory failure was severe and rapidly progressive, so she was managed with a mechanical ventilator under the impression of acute respiratory distress syndrome (ARDS). A bronchoalveolar lavage and lung biopsy by video-assisted thoracic surgery revealed NK-T cell lymphoma. We report a case of extranodal NK-T cell lymphoma presenting as ARDS.

A Comparison of Three Different Guidelines for Osteoporosis Treatment in Patients with Rheumatoid Arthritis in Korea

BACKGROUND/AIMS: Osteoporotic fractures are an important comorbidity with rheumatoid arthritis (RA). We determined the overall fracture risk as assessed by the World Health Organization (WHO)'s FRAX(R) tool in Korean patients with seropositive RA. Additionally, we compared treatment eligibility according to the criteria of the Korean Health Insurance Review Agency (HIRA), FRAX, and the National Osteoporosis Foundation (NOF). METHODS: Postmenopausal women and men > or = 50 years of age with seropositive RA were recruited from one rheumatism center in Korea. The FRAX score was estimated using the Japanese model. Patients were classified as eligible for treatment using the HIRA, NOF, and FRAX thresholds for intervention. RESULTS: The study of 234 patients included 40 men (17%). The mean age was 60 +/- 9 years, and 121 (52%) patients had osteoporosis according to the WHO criteria. The overall median 10-year fracture risk was 13% for major osteoporotic fractures and 3.5% for hip fractures. HIRA guidelines identified 130 patients (56%) eligible for treatment, FRAX included 126 patients (54%), and 151 patients (65%) were included according to NOF guidelines. Older patients with a greater number of risk factors were included by FRAX compared to HIRA. The overall concordance between HIRA and FRAX, expressed as the kappa index, was 0.67, but was as low as 0.44 when limited to patients > or = 60 years of age. CONCLUSIONS: One-half of the patients had osteoporosis requiring treatment. RA patients have a high risk of fracture, and the adoption of a risk-scoring system should be considered.

Cholestatic Hepatitis Caused by Tongyeong Strain of Orientia tsutsugamushi / 감염과화학요법

In Korea, the clinical manifestation of scrub typhus in humans appears to be severe in the northern regions of the country and mild in the southern areas. Studies on the geographic distribution of the serotype of Orientia tsutsugamushi revealed that the Boryong serotype is predominant in the southern Korean peninsula, whereas the Gilliam, Karp, and Gilliam-Karp serotypes prevail in the northern or central peninsula. We report a case of severe scrub typhus caused by the Japanese Gilliam type of Orientia tsutsugamushi in a 66-year-old woman from Tongyeong, a city located in the southern part of Korean peninsula. The patient was admitted to the hospital with fever on August 29th, 2001. Scrub typhus complicated by shock, pneumonitis, and encephalitis was diagnosed. Additionally, markedly elevated alkaline phosphatase levels was observed, which necessitated an ultrasonographic examination, which showed an ill-defined hyperechogenic mass on the right hepatic lobe. Liver biopsy showed cholestatic hepatitis and blood culture showed growth of O. tsutsugamushi. Gene sequencing of the amplicons of a polymerase chain reaction using primers for the amplification of the gene encoding the 56-kDa protein of O. tsutsugamushi revealed a nucleotide sequence that matched Yonchon strain of O. tsutsugamushi.

Cholestatic Hepatitis Caused by Tongyeong Strain of Orientia tsutsugamushi / 감염과화학요법

In Korea, the clinical manifestation of scrub typhus in humans appears to be severe in the northern regions of the country and mild in the southern areas. Studies on the geographic distribution of the serotype of Orientia tsutsugamushi revealed that the Boryong serotype is predominant in the southern Korean peninsula, whereas the Gilliam, Karp, and Gilliam-Karp serotypes prevail in the northern or central peninsula. We report a case of severe scrub typhus caused by the Japanese Gilliam type of Orientia tsutsugamushi in a 66-year-old woman from Tongyeong, a city located in the southern part of Korean peninsula. The patient was admitted to the hospital with fever on August 29th, 2001. Scrub typhus complicated by shock, pneumonitis, and encephalitis was diagnosed. Additionally, markedly elevated alkaline phosphatase levels was observed, which necessitated an ultrasonographic examination, which showed an ill-defined hyperechogenic mass on the right hepatic lobe. Liver biopsy showed cholestatic hepatitis and blood culture showed growth of O. tsutsugamushi. Gene sequencing of the amplicons of a polymerase chain reaction using primers for the amplification of the gene encoding the 56-kDa protein of O. tsutsugamushi revealed a nucleotide sequence that matched Yonchon strain of O. tsutsugamushi.