The relationship between anti-C-reactive protein and disease activity in patients with systemic lupus erythematosus

BACKGROUND/AIMS: Anti-C-reactive protein (CRP) antibody has been introduced as a potential biologic marker in Systemic lupus erythematosus (SLE). The aim of study is to evaluate the level of anti-CRP antibody in patients with SLE. METHODS: This study investigated the relationship between levels of anti-CRP antibodies and disease activity markers, such as complement, anti-double-stranded DNA antibody, and SLE disease activity index in 34 patients with SLE. RESULTS: The serum anti-CRP antibody levels of the patients with SLE were significantly higher than those of the healthy controls (11.3 ± 5.6 µg/mL vs. 9.1 ± 2.8 µg/mL). The percentages of the positive anti-CRP antibody were 52.9% in SLE and 27.8% in controls. Disease duration of SLE showed significant correlation with the anti-CRP antibody (r = 0.234, p = 0.026). However no significant relationship was observed between the levels of anti-CRP antibodies and disease activity markers. CONCLUSIONS: These data show that the anti-CRP antibody levels of the patients with SLE were significantly higher than those of healthy controls. We observed that the presence of the anti-CRP anti-CRP antibody was not associated with disease activity of SLE.

Erratum: Title, Introduction: A Case of Propylthiouracil induced Anti-neutrophil Cytoplasm Antibody Positive Pyoderma Gangrenosum

This correction is being published to correct the typo in title and introduction.

Two Cases of Hypophosphatemic Osteomalacia After Long-term Low Dose Adefovir Therapy in Chronic Hepatitis B and Literature Review

Adefovir dipivoxil (ADV) is a nucleotide used as long-term therapy of chronic hepatitis B. Many published reports have shown that long-term high-dose therapy with adefovir can be associated with proximal renal tubular dysfunction resulting in significant hypophosphatemia, renal insufficiency and osteomalacia. We have encountered two patients who developed evidence of hypophosphatemic osteomalacia while on long-term low-dose adefovir therapy for chronic hepatitis B. We report on its clinical features and its potential resolution with cessation of the drug and supplementation with phosphate. We also reviewed the other published cases associated with hypophosphatemic osteomalacia after low-dose adefovir therapy. The symptoms and the hypophosphatemia improved after cessation of the drug and supplementation with phosphate in most cases. Patients taking adefovir long-term should receive regular investigation of the phosphate level and renal function.

A Case of Propylthiouracil induced Antineutrophil Cytoplasmic Antibody Positive Pyoderma Gangrenosum

Pyoderma gangrenosum (PG) is a rare disease that causes chronic skin ulcers, and it has recently been known to be related to changes in the immune system such as dysfunction of neutrophils. Over 50% of patients with PG have an underlying systemic disease, and rarely are there various medicines causing the disease, including iodide, bromide, and isotretinoin. A 49-year-old man had recurrent skin ulcers in his lower extremities. He had a history of taking propylthiouracil (PTU) for 10 years as a treatment for Graves' disease. Here, we describe a case of PTU-induced ANCA positive PG in a patient with hyperthyroidism, who has been improved after the cessation of PTU.

Subpopulations of Regulatory T Cells in Rheumatoid Arthritis, Systemic Lupus Erythematosus, and Behcet's Disease

Recently, subpopulations of regulatory T (Treg) cells, resting Treg (rTreg) and activated Treg (aTreg), have been discovered. The authors investigated the relationship between the change of Treg, aTreg and rTreg and autoimmune diseases. Treg cells and those subpopulations were analyzed by using the human regulatory T cell staining kit and CD45RA surface marker for 42 rheumatoid arthritis (RA), 13 systemic lupus sclerosis (SLE), 7 Behcet's disease (BD), and 22 healthy controls. The proportion of Treg cells was significantly lower in RA (3.8% +/- 1.0%) (P < 0.001) and BD (3.3% +/- 0.5%) (P < 0.01) compared to healthy controls (5.0% +/- 1.3%). The proportion of aTreg cells was also significantly lower in RA (0.4% +/- 0.2%) (P = 0.008) and BD (0.3% +/- 0.1%) (P = 0.013) compared to healthy controls (0.6% +/- 0.3%). The rTreg cells showed no significant differences. The ratio of aTreg to rTreg was lower in RA patients (0.4% +/- 0.2%) than that in healthy controls (0.7% +/- 0.4%) (P = 0.002). This study suggests that the decrement of aTreg not rTreg cells contributes the decrement of total Treg cells in peripheral blood of RA and BD autoimmune diseases. Detailed analysis of Treg subpopulations would be more informative than total Treg cells in investigating mechanism of autoimmune disease.

Transverse Myelitis in Patient with Behcet's Disease

Behcet's disease (BD) is a multisystem disorder presenting recurrent oral and genital ulcerations as well as ocular lesions, involving the nervous system in a subgroup of patients. BD develops at a young age and is frequently presented with an acute or subacute brainstem syndrome or hemiparesis, as well as with other various neurological manifestations, the syndrome is often included in the differential diagnosis of multiple sclerosis, stroke of the young adult, and other neurological disorders. Transverse myelitis (TM) is a clinical syndrome in which an immune-mediated process causes neural injury to the spinal cord, resulting in varying degrees of weakness, sensory alterations and autonomic dysfunction. Spinal Neuro-behcet's disease is rare case. We reported a 33-year old man who had been treated for BD for 3 years.

Attritional Rupture of Ulnar Nerve in a Patient with Rheumatoid Arthritis

Ulnar neuropathy around the elbow in patients with rheumatoid arthritis can occur by synovial invasion, flexion contracture or valgus deformity of the elbow. However, attritional rupture of the ulnar nerve by medial osteophyte with synovial invasion of the rheumatoid elbow is extremely rare. We reported a case of attritional rupture of the ulnar nerve by medial osteophyte in 62 years-old male with rheumatoid arthritis. Exact diagnosis and proper treatment are required if symptoms of ulnar neuropathy present in rheumatoid arthritis with contracture and valgus deformity of the elbow.

Factors Related to Exertional Oxygen Desaturation in Patients with COPD / 결핵및호흡기질환

BACKGROUND: The causes of exertional desaturation in patients with COPD can be multifactorial. We aimed to investigate factors predict exertional desaturation in patients with moderate to severe COPD. METHODS: We tested 51 consecutive patients with stable COPD (FEV1/FVC, 40+/-13% predicted). Patients performed a six minute walk test (6MWT). Pulse oxymetric saturation (SpO2) and pulse rate were recorded. RESULTS: Oxygen desaturation was found in 15 subjects after 6MWT, while 36 subjects were not desaturated. Lung diffusing capacity was significantly lower in desaturation (DS) group (62+/-18% predicted) compared with not desaturated (ND) group (84+/-20, p<0.01). However there was no statistical difference of FEV1/FVC ratio or residual volume between two groups. The pulse rate change was significantly higher in the desaturated compared with the not desaturated group. Six minute walking distance, subjective dyspnea scale, airflow obstruction, and residual volume did not predict exertional oxygen desaturation. Independent factors assessed by multiple logistic regression revealed that a pulse rate increment (odd ratio [OR], 1.19; 95% confidence interval [CI], 1.01~1.40; p=0.02), a decrease in baseline PaO2 (OR, 1.105; 95% CI, 1.003~1.218; p=0.04) and a decrease in lung diffusing capacity (OR, 1.10; 95% CI, 1.01~1.19; p=0.01) were significantly associated with oxygen desaturation. Receiver operator characteristic (ROC) analysis showed that an absolute increment in pulse rate of 16/min gave optimal discrimination between desaturated and not desaturated patients after 6MWT. CONCLUSION: Pulse rate increment and diffusion capacity can predict exertional oxygen desaturation in stable COPD patients with moderate to severe airflow obstruction.

CT Radiologic Findings in Patients with Tuberculous Destroyed Lung and Correlation with Lung Function / 결핵및호흡기질환

BACKGROUND: A tuberculous destroyed lung is sequelae of pulmonary tuberculosis and causes various respiratory symptoms and pulmonary dysfunction. The patients with a tuberculous destroyed lung account for a significant portion of those with chronic lung disease in Korea. However, few reports can be found in the literature. We investigated the computed tomography (CT) findings in a tuberculous destroyed lung and the correlation with lung function. METHODS: A retrospective analysis was carried out for 44 patients who were diagnosed with a tuberculous destroyed lung at the Keimyung University Dongsan Hospital between January 2004 and December 2009. RESULTS: A chest CT scan showed various thoracic sequelae of tuberculosis. In lung parenchymal lesions, there were cicatrization atelectasis in 37 cases (84.1%) and emphysema in 13 cases. Bronchiectasis (n=39, 88.6%) was most commonly found in airway lesions. The mean number of destroyed bronchopulmonary segments was 7.7 (range, 4~14). The most common injured segment was the apicoposterior segment of the left upper lobe (n=36, 81.8%). In the pulmonary function test, obstructive ventilatory defects were observed in 31 cases (70.5%), followed by a mixed (n=7) and restrictive ventilatory defect (n=5). The number of destroyed bronchopulmonary segments showed a significant negative correlation with forced vital capacity (FVC), % predicted (r=-0.379, p=0.001) and forced expiratory volume in one second (FEV1), % predicted (r=-0.349, p=0.020). After adjustment for age and smoking status (pack-years), the number of destroyed segments also showed a significant negative correlation with FVC, % predicted (B=-0.070, p=0.014) and FEV1, % predicted (B=-0.050, p=0.022). CONCLUSION: Tuberculous destroyed lungs commonly showed obstructive ventilatory defects, possibly due to bronchiectasis and emphysema. There was negative correlation between the extent of destruction and lung function.

Acute Pulmonary Embolism: Clinical Characteristics and Outcomes in a University Teaching Hospital / 결핵및호흡기질환

BACKGROUND: Pulmonary embolism (PE) is a common clinical problem in the West that is associated with substantial morbidity and mortality. The diagnostic modality has been changed since 2001. This study retrospectively reviewed the PE mortality with the aim of identifying the risk factors associated with mortality since the multidetector computed tomography (MDCT) was introduced. METHODS: We analyzed 105 patients with acute PE proven by multidetector CT or ventilation perfusion scan. The primary outcome measure was the all-cause mortality at 3 months. The prognostic effect of the baseline factors on survival was assessed by multivariate analysis. RESULTS: The main risk factors were prolonged immobilization, stroke, cancer and obesity. Forty nine percent of patients had 3 or more risk factors. The overall mortality at 3 months was 18.1%. Multivariate analysis revealed low diastolic blood pressure and the existence of cancer to be independent factors significantly associated with mortality. Forty two PE patients were examined for the coagulation inhibitors. Four of these patients had a protein C deficiency (9.5%), and 11 had a protein S deficiency (26%). CONCLUSION: PE is an important clinical problem with a high mortality rate. Close monitoring may be necessary in patients with the risk factors.

Critical Care Medicine / 결핵및호흡기질환

The year of 2009~2010 brought a number of concepts and new ideas were evaluated with promising results. However, some studies that challenged many beliefs. In acute respiratory distress syndrome (ARDS), recent clinical studies took into consideration of pathophysiologic changes of respiratory system compliance. Meta-analysis of positive end-expiratory pressure trials showed survival benefit of high positive end-expiratory pressure in ARDS. Until now, prone positioning did not show survival benefit in patients with ARDS. Extracorporeal membrane oxygenation (ECMO) based management improved survival in patients with severe ARDS. ECMO can be a management option in severe ARDS. Sedation is a standard practice in critically ill patients needing mechanical ventilation. However, Danish group reported less sedation of critically ill patients receiving mechanical ventilation was associated with an increase in days without ventilation. Although this single center study has some limitations, the overall results are promising. Use of maximal sterile barrier precautions (mask, sterile gown, sterile gloves, and large sterile drapes) with chlorhexidine-impregnated dressing reduced central venous catheter related infection. Selective oropharyngeal decontamination (application of topical antibiotics in the oropharynx) reduced the mortality rate of an intensive care unit (ICU) population. Normoglycemia in Intensive Care Evaluation and Survival Using Glucose Algorithm Regulation (NICE-SUGAR) trial reported intensive glucose control increased mortality among adults in the ICU. Some of the results of above papers are promising. However, some ideas may need for more frequent individual assessment and increase the workload of ICU staffs. Before implementation of new practice in ICU, we should take into consideration of individual hospital situation including human and material resources.

Churg-Strauss Syndrome Presenting as Transverse Myelitis / 대한류마티스학회지

Churg-Strauss syndrome (CSS), also known as allergic granulomatous angiitis, is a rare disorder characterized by the presence of asthma, eosinophilia and small-to-medium-sized vessels vasculitis. The peripheral nervous system is frequently involved in CSS, but central nervous system (CNS) involvement is rare. Furthermore, transverse myelitis (TM) as a presenting symptom in patients with CSS is extremely rare. We report here on a 60-year-old female who presented peripheral eosinophilia, lung eosinophilic infiltration, lung vasculitis, and TM. She was diagnosed as CSS based on clinical manifestation, pathologic findings, and the use of magnetic resonance imaging (MRI).

Assessment of Two Clinical Prediction Models for a Pulmonary Embolism in Patients with a Suspected Pulmonary Embolism / 결핵및호흡기질환

BACKGROUND: Estimation of the probability of a patient having an acute pulmonary embolism (PE) for patients with a suspected PE are well established in North America and Europe. However, an assessment of the prediction rules for a PE has not been clearly defined in Korea. The aim of this study is to assess the prediction rules for patients with a suspected PE in Korea. METHODS: We performed a retrospective study of 210 inpatients or patients that visited the emergency ward with a suspected PE where computed tomography pulmonary angiography was performed at a single institution between January 2005 and March 2007. Simplified Wells rules and revised Geneva rules were used to estimate the clinical probability of a PE based on information from medical records. RESULTS: Of the 210 patients with a suspected PE, 49 (19.5%) patients had an actual diagnosis of a PE. The proportion of patients classified by Wells rules and the Geneva rules had a low probability of 1% and 21%, an intermediate probability of 62.5% and 76.2%, and a high probability of 33.8% and 2.8%, respectively. The prevalence of PE patients with a low, intermediate and high probability categorized by the Wells rules and Geneva rules was 100% and 4.5% in the low range, 18.2% and 22.5% in the intermediate range, and 19.7% and 50% in the high range, respectively. Receiver operating characteristic curve analysis showed that the revised Geneva rules had a higher accuracy than the Wells rules in terms of detecting PE. Concordance between the two prediction rules was poor (kappa coefficient=0.06). CONCLUSION: In the present study, the two prediction rules had a different predictive accuracy for pulmonary embolisms. Applying the revised Geneva rules to inpatients and emergency ward patients suspected of having PE may allow a more effective diagnostic process than the use of the Wells rules.

A Case of Pulmonary Embolism in a Patient with a Factor VII Gene Promoter -401G/A Polymorphism / 결핵및호흡기질환

A factor VII gene -401 G/A polymorphism was identified in a patient with a pulmonary embolism. The patient was a 71-year-old woman who presented with acute-onset dyspnea. A chest CT scan revealed a pulmonary embolism. Despite the administration of low-dose warfarin as anticoagulation therapy, there was an excessively prolonged prothrombin time (PT). The blood tests revealed lower factor VII activity than normal. Full factor VII gene sequencing revealed a G to A substitution at ?401 in the promoter region. There were no other gene sequence anomalies. PCR-based analysis indicated lower factor VII gene expression in the patient than in a control subject. The data suggested the promoter polymorphism to be responsible for the lower transcription level. In conclusion, we encountered a case of Factor VII DNA polymorphism in a patient with a pulmonary embolism showing significantly reduced Factor VII activity.