Giant Coronary Artery Aneurysm with Thrombosis Complicated in a Patient with Idiopathic Hypereosinophilic Syndrome

Idiopathic hypereosinophilic syndrome (iHES) is a rare systemic disease that is characterized by persistent peripheral eosinophilia (absolute eosinophil count ≥1500/uL) for more than 6 months, with end-organ damage and absence of a primary cause for eosinophilia. Coronary artery aneurysm (CAA) is a rare but life-threatening complication. Here, we report a case of CAA with thrombosis in a patient with iHES in whom the disease activity was well-controlled (eosinophil count <500/uL) for several years. Despite modest control of the disease activity, giant CAA can be associated with iHES; and therefore, close surveillance and monitoring for the development of complications is warranted.

Incidence of adverse drug reaction among 6 iodinated contrast media

Purpose@#Contrast media is one of the most common cause of adverse drug reaction (ADR) in adult. However, there was little data reported about differences in ADR ratio and severity among iodinated contrast media (ICM). @*Methods@#Medical records of 627,049 patients who performed computed tomography scan using low-osmolar nonionic iodinated contrast media from January 2015 to December 2018 were retrospectively reviewed. A total of 6 ICMs including iomeprol, iohexol, iopromide, iobitridol, ioversol, and iopamidol were used in this period. The incidence of ADR was compared to their total usage and dosage between each contrast media. @*Results@#The incidence of ADR of iopromide (1.36%) and iomeprol (1.27%) was the highest when compared with the average incidence of 1% of 6 ICMs. Ioversol (0.67%), iohexol, and iobitridol (0.74%) had the lower incidence of ADR. The order of results adjusted by actual administered dosage, the use of premedication, and the prior exposure history of ICMs was similar. The fraction of moderate and severe ADR in overall ADR was slightly different, but not proportional to the incidence of ADR. @*Conclusion@#The incidence of ADR among 6 low-osmolar nonionic ICMs was significantly different when compared by the total number of usage and the total volume of dose. The incidence of ADR varied by nearly 2-fold depending on ICMs. Further study might need to explore the reason for the difference of incidence.

Effect of omalizumab as add-on therapy to Quality of Life Questionnaire for Korean Asthmatics (KAQLQ) in Korean patients with severe persistent allergic asthma

Background/Aims@#Omalizumab is the first biologic known to be effective in patients with severe allergic asthma. @*Methods@#This study was conducted as a multicenter, single-group, open trial to evaluate the improvement in the quality of life with the additional administration of omalizumab for 24 weeks in Korean patients with severe persistent allergic asthma. @*Results@#Of the 44 patients, 31.8% were men and the mean age was 49.8 ± 11.8 years. A score improvement of 0.5 points or more in the Quality of Life Questionnaire for Korean Asthmatics (KAQLQ) was noted in 50.0% (22/44) of the patinets. In the improved group, the baseline total immunoglobulin E (IgE) level and the amount of omalizumab used were higher, and the day and night asthma symptoms were more severe, compared to those in the non-improved group. According to the Global Evaluation of Treatment Effectiveness, favorable outcomes were found in 78.6% of patients. The Korean asthma control test (p < 0.005) and forced expiratory volume in 1 second % predicted (FEV1%; p < 0.01) improved significantly in patients who received omalizumab treatment, compared to that at week 0, and the total dose of rescue systemic corticosteroids significantly decreased (p < 0.05). The improved group on KAQLQ showed a significant improvement in FEV1% (p < 0.001). @*Conclusions@#Omalizumab can be considered a biological treatment for Korean patients with severe allergic asthma. It is recommended to consider omalizumab as add-on therapy in patients with high baseline total IgE levels and severe asthma symptoms.

Effect of omalizumab as add-on therapy to Quality of Life Questionnaire for Korean Asthmatics (KAQLQ) in Korean patients with severe persistent allergic asthma

Background/Aims@#Omalizumab is the first biologic known to be effective in patients with severe allergic asthma. @*Methods@#This study was conducted as a multicenter, single-group, open trial to evaluate the improvement in the quality of life with the additional administration of omalizumab for 24 weeks in Korean patients with severe persistent allergic asthma. @*Results@#Of the 44 patients, 31.8% were men and the mean age was 49.8 ± 11.8 years. A score improvement of 0.5 points or more in the Quality of Life Questionnaire for Korean Asthmatics (KAQLQ) was noted in 50.0% (22/44) of the patinets. In the improved group, the baseline total immunoglobulin E (IgE) level and the amount of omalizumab used were higher, and the day and night asthma symptoms were more severe, compared to those in the non-improved group. According to the Global Evaluation of Treatment Effectiveness, favorable outcomes were found in 78.6% of patients. The Korean asthma control test (p < 0.005) and forced expiratory volume in 1 second % predicted (FEV1%; p < 0.01) improved significantly in patients who received omalizumab treatment, compared to that at week 0, and the total dose of rescue systemic corticosteroids significantly decreased (p < 0.05). The improved group on KAQLQ showed a significant improvement in FEV1% (p < 0.001). @*Conclusions@#Omalizumab can be considered a biological treatment for Korean patients with severe allergic asthma. It is recommended to consider omalizumab as add-on therapy in patients with high baseline total IgE levels and severe asthma symptoms.

Anaphylaxis due to polyethylene glycol: A case report

Polyethylene glycol (PEG) is a major component of bowel preparation solution for colonoscopy. It has been recognized as a safe and effective osmotic laxative that is rarely immunogenic. We here report a case of anaphylaxis due to PEG. A 49-year-old female came to the outpatient clinic wanting to find the cause of skin rash, nausea and respiratory difficulty after ingesting bowel evacuant solution (Clicool). She had visited local Emergency Department at the time of event and was diagnosed with anaphylaxis. We performed skin tests with components of Clicool to identify the cause of anaphylactic reactions. The result showed a positive skin response only to PEG. In contrast, skin test done in the control showed no reactions to PEG. This is the first case that revealed PEG to be an exact cause of anaphylaxis after ingesting a bowel evacuant.

Clinical Characteristics and Treatment Response of Peripheral Neuropathy in the Presence of Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Experience at a Single Tertiary Center

BACKGROUND AND PURPOSE: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic small-vessel vasculitis accompanied by asthma, eosinophilia, and eosinophilic inflammation of various tissues including the peripheral nerves. This study investigated the clinical course and long-term outcomes of peripheral neuropathy in patients with EGPA. METHODS: Seventy-one patients with physician-diagnosed EGPA were identified at Samsung Medical Center between January 1995 and April 2014. Sixty-one of these patients were followed-up for more than 1 year and received corticosteroid therapy with or without intravenous cyclophosphamide pulse therapy for 6 to 18 months. Medical records of the 61 patients including demographic data, clinical features, laboratory and pathological findings, treatments, and outcomes were reviewed. RESULTS: Peripheral neuropathy as a manifestation of EGPA was present in 46 (75%) of the 61 patients. The mean follow-up duration of the patients with neuropathy was 6.4 years (range 1.2–18.8 years). The scores on the neurological functional disability scale before and after the combination treatment with corticosteroid and cyclophosphamide were 2.43±0.86 and 0.54±0.95 (mean±SD; p<0.001), respectively. The peripheral neuropathy relapsed in one patient. CONCLUSIONS: The long-term clinical outcome of peripheral neuropathy in patients with EGPA receiving initial corticosteroid and cyclophosphamide combination therapy was favorable with a very low relapse rate.

The Hidden Culprit: A Case of Repeated Anaphylaxis to Cremophor

Drug-induced anaphylaxis is a big pitfall in patients receiving antineoplastic chemotherapy. We report a case of lung cancer patient who experienced two near-fatal anaphylactic reactions that resulted from paclitaxel and multivitamin, seperately. Recurrent severe reactions to different agents led to further investigation to which material the patient was hypersensitive. The skin prick test revealed sensitization to cremophor, which is a commonly used emulsifying agent. This case emphasizes the importance of correctly identifying the culprit drug of anaphylaxis to avoid potentially fatal reaction.

Fractional Exhaled Nitric Oxide: Comparison Between Portable Devices and Correlation With Sputum Eosinophils

This study was performed to compare the 2 different portable devices measuring fractional exhaled nitric oxide (FeNO) and to see the correlation between FeNO and induced sputum eosinophil count (ISE). Forty consecutive subjects clinically suspected to have asthma underwent FeNO measurement by NIOX-MINO(R) and NObreath(R) concurrently. All also had induced sputum analysis, methacholine provocation test or bronchodilator response test, and spin prick test. Agreement between the 2 devices was evaluated. The correlation between FeNO and ISE was assessed, as well as the cut-off level of FeNO to identify ISE > or =3%. The intraclass correlation coefficient (ICC) between FeNO levels measured by NIOX-MINO(R) (FeNO(NIOX-MINO)) and NObreath(R) (FeNO(NObreath)) was 0.972 with 95% confidence interval of 0.948-0.985. The 95% limits of agreement were -28.9 to 19.9 ppb. The correlation coefficient between ISE and FeNO(NIOX-MINO) was 0.733 (P or =3% with 90% sensitivity and 81% specificity. Age, sex, body mass index, smoking history, atopy, and the presence of asthma did not affect the FeNO level and its correlation with ISE. The NIOX-MINO (R) and NObreath(R) agree with each other to a high degree. Both devices showed close correlation with ISE with similar cut-off value in identifying ISE > or =3%.

Chronic eosinophilic leukemia with a FIP1L1-PDGFRA rearrangement: Two case reports and a review of Korean cases

No abstract available.

The Prevalence of Toxocariasis and Diagnostic Value of Serologic Tests in Asymptomatic Korean Adults

PURPOSE: Toxocariasis is the most common cause of peripheral blood eosinophilia in Korea and produces eosinophilic infiltration in various organs, including the lung. However, the prevalence of toxocariasis in the general population is rarely reported. METHODS: We investigated the seroprevalence of Toxocara larval antibody among asymptomatic people who attended Samsung Medical Center for a health checkup, including low-dose chest computed tomography (CT) between March 2012 and December 2013. A total of 633 people (400 men and 233 women) were prospectively recruited. RESULTS: The Toxocara-seropositive rate was 51.2% using the current cutoff value based on Toxocara enzyme-linked immunosorbent assay (ELISA) (67.0% for men and 24.0% for women). In the multivariate-adjusted model, age (odds ratio [OR], 1.08; 95% confidence intervals [CI], 1.04-1.11), male sex (OR, 3.47; 95% CI, 2.26-5.33), rural residence (OR, 1.55; 95% CI, 1.05-2.30), and history of raw liver intake (OR, 8.52; 95% CI, 3.61-20.11) were significantly associated with Toxocara seropositivity. When subjects were divided into 3 groups using cutoff values base on weak positive and strong positive control optical densities (ODs), the ORs for peripheral blood eosinophilia and serum hyperIgEaemia were 0.31 (95% CI, 0.02-2.89) in the weakpositive group and 36.64 (95% CI, 11.73-111.42) in the strong positive group compared to the seronegative group. Similarly, ORs for the solid nodule with surrounding halo were 2.54 (95% CI, 0.60-10.84) in the weak positive group and 15.08 (95 CI 4.09-55.56) in the strong positive group compared to the seronegative group. CONCLUSIONS: The study indicated that the Toxocara-seropositive rate obtained by using the current cutoff value based on ELISA was high in the asymptomatic population in Korea. The results of this study suggest that active toxocariasis may be more frequently seen in the Toxocara-strong positive group than in the Toxocara-weak positive group.

A Case of Idiopathic Hypereosinophilic Syndrome Presenting With Acute Respiratory Distress Syndrome

Although idiopathic hypereosinophilic syndrome(IHES) commonly involves the lung, it is rarely associated with acute respiratory distress syndrome (ARDS). Here we describe a case of IHES presented in conjunction with ARDS. A 37-year-old male visited the emergency department at Samsung Medical Center, Seoul, Korea, with a chief complaint of dyspnea. Blood tests showed profound peripheral eosinophilia and thrombocytopenia. Patchy areas of consolidation with ground-glass opacity were noticed in both lower lung zones on chest radiography. Rapid progression of dyspnea and hypoxia despite supplement of oxygen necessitated the use of mechanical ventilation. Eosinophilic airway inflammation was subsequently confirmed by bronchoalveolar lavage, leading to a diagnosis of IHES. High-dose corticosteroids were administered, resulting in a dramatic clinical response.

Potential Masking of Airway Eosinophilic Inflammation by Combination Therapy in Asthma

PURPOSE: Long-acting beta2 agonists (LABA) may mask ongoing bronchial inflammation, leaving asthmatic patients at greater risk of severe complications. The aim of this study was to compare the effect of combination therapy using low-dose inhaled corticosteroids (ICS) plus LABA on airway inflammation in asthma to the effect of medium-dose ICS alone. METHODS: Twenty-four patients with asthma not controlled by low-dose (400 microg per day) budesonide alone were enrolled in this prospective crossover study. Patients were randomized into 2 treatment phases: one receiving medium-dose (800 microg per day) budesonide (ICS phase), and the other receiving a combination therapy of low-dose budesonide/formoterol (360 microg/9 microg per day) delivered by a single inhaler (LABA phase). Each treatment phase lasted for 6 week, after which patients were crossed over. Asthma symptoms, lung function, and airway inflammation were compared between the 2 phases. RESULTS: Twenty-three patients completed the study; adequate sputum samples were collected from 17 patients. Asthma symptoms and lung function remained similar between the 2 phases. However, the mean sputum eosinophil percentage was higher in the LABA phase than in the ICS phase (5.07+/-3.82% vs. 1.02+/-1.70%; P or =3%) was more frequently observed in the LABA phase than in the ICS phase (six vs. two). CONCLUSION: Addition of LABA may mask airway eosinophilic inflammation in asthmatic patients whose symptoms are not controlled with low-dose ICS.

Lung Abscess During the Treatment of Relapsed Churg-Strauss Syndrome / 대한내과학회지

Churg-Strauss syndrome (CSS) is a rare disease characterized by asthma, peripheral eosinophilia and vasculitis. A quarter of CSS patients with clinical remission have experienced a relapse. We report here a case of lung abscess in a patient with relapsed CSS. A 46-year-old man who achieved clinical remission of CSS was confirmed for relapse by the presence of peripheral eosinophillia, pulmonary lesions and perivascular infiltrate of eosinophils in colon pathology. After administration of systemic glucocorticoid and one cycle of cyclophosphamide pulse therapy, he complained of dyspnea, sputum and chest pain. There were necrotic masses containing internal air-fluid levels in the right upper and the left lower lobes of the lung on chest radiography. Percutaneous needle aspiration culture specimens revealed the presence of K. pneumoniae. The patient was successfully treated with a 5-week course of antibiotics.

Churg-Strauss syndrome presenting as acute acalculous cholecystitis

Churg-Strauss syndrome (CSS) is a rare systemic vasculitis. It is characterized by peripheral eosinophilia, asthma, neuropathy, skin manifestation, and less frequently gastrointestinal tract symptoms. Here we report a case of CSS which is initially manifested as acute acalculous cholecystitis. A 67-year-old asthmatic woman visited Emergency Room because of acute abdominal pain. Computed tomography showed acalculous cholecystitis and laparoscopic cholecystectomy was done. Pathology of gallbladder showed marked eosinophilic infiltration with necrotizing vasculitis and granuloma. On the basis of asthma, sinusitis, lung infiltration, combined peripheral eosinophilia and neuropathy, the patient was diagnosed as CSS. To our knowledge, this is the first case of pathologically proven eosinophilic cholecystitis in Korea.

Transmission of Toxocara canis via Ingestion of Raw Cow Liver: A Cross-Sectional Study in Healthy Adults

The aim of this study is to ascertain the relationship between ingestion of raw cow liver and Toxocara canis infection. A total of 150 apparently healthy adults were divided into 2 groups; 1 group consisted of 86 adults with positive results of Toxocara ELISA, and the other group of 64 adults with negative results. One researcher collected the history of ingestion of raw cow liver within 1 year and recent history of keeping dogs. Among 86 seropositive adults for T. canis, 68 (79.1%) had a recent history of ingestion of raw cow liver. Multivariate statistical analysis showed that a recent ingestion of raw cow liver and keeping dogs were related to an increased risk of toxocariasis (odds ratios, 4.4 and 3.7; and 95% confidence intervals, 1.9-10.2 and 1.2-11.6, respectively). A recent history of ingestion of raw cow liver and keeping dogs was significantly associated with toxocariasis.

A Case of Hypereosinophilic Syndrome Presenting With Multiorgan Infarctions Associated With Disseminated Intravascular Coagulation

Thromboembolism is one of the most critical complications of hypereosinophilic syndrome (HES). We report here a case of multi-organ infarctions related to HES. A 23-year-old woman was referred to our hospital with hemoptysis. Not only pulmonary, but also renal and splenic infarctions were detected on computed tomography images. Blood tests showed profound peripheral eosinophilia. She was diagnosed with HES with disseminated intravascular coagulation (DIC). We initiated infusion of corticosteroids, which effectively suppressed peripheral eosinophilia. However, consumptive coagulopathy did not improve and intracerebral hemorrhage related to thrombosis then developed. Addition of interferon-alpha resulted in the correction of the DIC associated with HES.

A case of cardiac involvement in Churg-Strauss syndrome presenting without cardiomegaly in an asymptomatic patient / 대한내과학회지

We report a case of Churg-Strauss syndrome with cardiac involvement presenting without cardiomegaly or cardiopulmonary symptoms. A 47-year-old woman was referred to our institution for myalgia, peripheral numbness, and eosinophilia. She had been diagnosed with bronchial asthma and allergic rhinitis four years ago. The patient exhibited eosinophilia (71%) and elevated cardiac enzymes (cTnI, 2.977 ng/mL). Cardiomegaly was not observed on chest radiography, but nonspecific ST segment changes were observed on electrocardiography. A transthoracic echocardiography revealed a dilated left ventricular cavity, a decreased left ventricle (42%), and diastolic dysfunction. Contrast-enhanced cardiac magnetic resonance imaging revealed delayed hyperenhancement 10 minutes after injecting gadolinium. An endomyocardial biopsy showed eosinophilic myocarditis associated with vasculitis. The patient was diagnosed with Churg-Strauss syndrome and received combination therapy with steroid and cyclophosphamide. After the second treatment cycle, the blood eosinophilia disappeared and the vasculitis and infiltration of eosinophils into the endomyocardial tissue had completely resolved.

Role of inducible nitric oxide synthase on the development of virus-associated asthma exacerbation which is dependent on Th1 and Th17 cell responses

Asthma is characterized by airway inflammation induced by immune dysfunction to inhaled antigens. Although respiratory viral infections are the most common cause of asthma exacerbation, immunologic mechanisms underlying virus-associated asthma exacerbation are controversial. Clinical evidence indicates that nitric oxide (NO) levels in exhaled air are increased in exacerbated asthma patients compared to stable patients. Here, we evaluated the immunologic mechanisms and the role of NO synthases (NOSs) in the development of virus-associated asthma exacerbation. A murine model of virus-associated asthma exacerbation was established using intranasal challenge with ovalbumin (OVA) plus dsRNA for 4 weeks in mice sensitized with OVA plus dsRNA. Lung infiltration of inflammatory cells, especially neutrophils, was increased by repeated challenge with OVA plus dsRNA, as compared to OVA alone. The neutrophilic inflammation enhanced by dsRNA was partly abolished in the absence of IFN-gamma or IL-17 gene expression, whereas unaffected in the absence of IL-13. In terms of the roles of NOSs, dsRNA-enhanced neutrophilic inflammation was significantly decreased in inducible NOS (iNOS)-deficient mice compared to wild type controls; in addition, this phenotype was inhibited by treatment with a non-specific NOS inhibitor (L-NAME) or an specific inhibitor (1400 W), but not with a specific endothelial NOS inhibitor (AP-CAV peptide). Taken together, these findings suggest that iNOS pathway is important in the development of virus-associated exacerbation of neutrophilic inflammation, which is dependent on both Th1 and Th17 cell responses.

Comparison of the Causes and Clinical Features of Drug Rash With Eosinophilia and Systemic Symptoms and Stevens-Johnson Syndrome

PURPOSE: Drug rash with eosinophilia and systemic symptoms (DRESS) and the Stevens-Johnson syndrome (SJS) are both severe drug reactions. Their pathogenesis and clinical features differ. This study compared the causes and clinical features of SJS and DRESS. METHODS: We enrolled 31 patients who were diagnosed with DRESS (number=11) and SJS (number=20). We retrospectively compared the clinical and laboratory data of patients with the two disorders. RESULTS: In both syndromes, the most common prodromal symptoms were itching, fever, and malaise. The liver was commonly involved in DRESS. The mucosal membrane of the oral cavity and eyes was often affected in SJS. The most common causative agents in both diseases were antibiotics (DRESS 4/11 (37%), SJS 8/20 (40%)), followed by anticonvulsants (DRESS 3/11 (27%), SJS 7/20 (35%)). In addition, dapsone, allopurinol, clopidogrel, sulfasalazine and non-steroidal anti-inflammatory drugs (NSAIDs) were sporadic causes. CONCLUSIONS: The most common causes of DRESS and SJS were antibiotics, followed by anticonvulsants, NSAIDs and sulfonamides. The increase in the use of antibiotics in Korea might explain this finding.

Comparison of the Causes and Clinical Features of Drug Rash With Eosinophilia and Systemic Symptoms and Stevens-Johnson Syndrome

PURPOSE: Drug rash with eosinophilia and systemic symptoms (DRESS) and the Stevens-Johnson syndrome (SJS) are both severe drug reactions. Their pathogenesis and clinical features differ. This study compared the causes and clinical features of SJS and DRESS. METHODS: We enrolled 31 patients who were diagnosed with DRESS (number=11) and SJS (number=20). We retrospectively compared the clinical and laboratory data of patients with the two disorders. RESULTS: In both syndromes, the most common prodromal symptoms were itching, fever, and malaise. The liver was commonly involved in DRESS. The mucosal membrane of the oral cavity and eyes was often affected in SJS. The most common causative agents in both diseases were antibiotics (DRESS 4/11 (37%), SJS 8/20 (40%)), followed by anticonvulsants (DRESS 3/11 (27%), SJS 7/20 (35%)). In addition, dapsone, allopurinol, clopidogrel, sulfasalazine and non-steroidal anti-inflammatory drugs (NSAIDs) were sporadic causes. CONCLUSIONS: The most common causes of DRESS and SJS were antibiotics, followed by anticonvulsants, NSAIDs and sulfonamides. The increase in the use of antibiotics in Korea might explain this finding.