Comparison of the 2017 EULAR/ACR Criteria with Clinicoserologic Criteria for the Classification of Idiopathic Inflammatory Myopathies in Korean Patients

Purpose@#To investigate correlations between myositis-specific autoantibodies (MSA) or myositis-associated antibodies (MAA) and clinical features, thereby demonstrating the utility of clinicoserologic classification in idiopathic inflammatory myopathies (IIM) patients. @*Materials and Methods@#We conducted a multicenter study of 108 adult patients (age ≥18 years) who were diagnosed with IIM by Peter and Bohan criteria or 2004 European Neuromuscular Centre (ENMC) criteria. Clinical data were obtained by medical record review. Immunoblot assay with Euroline strip (EUROIMMUN, Germany) was performed using the sera of dermatomyositis (DM, n=56), polymyositis (PM, n=45), amyopathic DM (n=5), DM sine dermatitis (n=1), and immune mediated necrotizing myopathy (n=1) patients. Patients were classified based on two classifications: 2017 EULAR/ACR and novel clinicoserologic classification. @*Results@#According to 2017 EULAR/ACR criteria, DM and PM were the most and the second most frequent entities. Overlap myositis was the major entity of IIM, and the frequency of PM was significantly lower when applying clinicoserologic classification criteria. Sixty-nine (63.9%) patients had one or more MSA, and 61 (56.5%) patients had one or more MAA. Interstitial lung disease was closely associated with anti-MDA5 and anti-ARS, and DM-specific skin lesions were frequently observed in patients with antiTIF1γ, anti-SRP, and anti-MDA5. @*Conclusion@#The clinicoserologic criteria based on MSA/MAA positivity could reflect more precise clinical features of IIM. Establishment of a laboratory system routinely available to screen for MSA/MAA status will be beneficial to provide precise diagnosis and proper management of IIM patients.

Comparison of the 2017 EULAR/ACR Criteria with Clinicoserologic Criteria for the Classification of Idiopathic Inflammatory Myopathies in Korean Patients

Purpose@#To investigate correlations between myositis-specific autoantibodies (MSA) or myositis-associated antibodies (MAA) and clinical features, thereby demonstrating the utility of clinicoserologic classification in idiopathic inflammatory myopathies (IIM) patients. @*Materials and Methods@#We conducted a multicenter study of 108 adult patients (age ≥18 years) who were diagnosed with IIM by Peter and Bohan criteria or 2004 European Neuromuscular Centre (ENMC) criteria. Clinical data were obtained by medical record review. Immunoblot assay with Euroline strip (EUROIMMUN, Germany) was performed using the sera of dermatomyositis (DM, n=56), polymyositis (PM, n=45), amyopathic DM (n=5), DM sine dermatitis (n=1), and immune mediated necrotizing myopathy (n=1) patients. Patients were classified based on two classifications: 2017 EULAR/ACR and novel clinicoserologic classification. @*Results@#According to 2017 EULAR/ACR criteria, DM and PM were the most and the second most frequent entities. Overlap myositis was the major entity of IIM, and the frequency of PM was significantly lower when applying clinicoserologic classification criteria. Sixty-nine (63.9%) patients had one or more MSA, and 61 (56.5%) patients had one or more MAA. Interstitial lung disease was closely associated with anti-MDA5 and anti-ARS, and DM-specific skin lesions were frequently observed in patients with antiTIF1γ, anti-SRP, and anti-MDA5. @*Conclusion@#The clinicoserologic criteria based on MSA/MAA positivity could reflect more precise clinical features of IIM. Establishment of a laboratory system routinely available to screen for MSA/MAA status will be beneficial to provide precise diagnosis and proper management of IIM patients.

Correction to: The Expression of Immunomodulation-Related Cytokines and Genes of Adipose- and Bone Marrow-Derived Human Mesenchymal Stromal Cells from Early to Late Passages

Unfortunately, the online published article has error in Figure 3.

Immunoglobulin G4-Related Disease Presenting with Isolated, Intractable Trigeminal Neuralgia

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated systemic condition affecting diverse organs which lately has been increasingly recognized. A 40-year-old male presents intractable trigeminal neuralgia lasting more than a year even with numerous pain medication, which lead further evaluation. The infiltrative lesion was found involving left pterygopalatine fossa where maxillary nerve locates. We report a case of IgG4-RD with a sole manifestation of intractable unilateral trigeminal neuralgia which was improved by steroid treatment.

Methimazole-Induced Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis in the Peripheral Nerves

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis has been reported in Graves' disease patients treated with antithyroid drugs (ATDs), especially propylthiouracil. ATD-induced ANCA-associated vasculitis usually involved the kidneys followed by the respiratory organs and skin. The treatment of ANCA-associated vasculitis induced by ATDs is to stop ATD therapy immediately, which often leads to an overall good prognosis. We report a case of ANCA-associated vasculitis in the peripheral nerves of the lower extremities in a 66-year-old woman who was treated with methimazole (MMI) for Graves' disease. To our knowledge, this is the third case of peripheral nervous system (PNS) involvement of ATD-induced vasculitis and the first case of PNS vasculitis associated with MMI.

The Expression of Immunomodulation-Related Cytokines and Genes of Adipose- and Bone Marrow-Derived Human Mesenchymal Stromal Cells from Early to Late Passages

BACKGROUND: Mesenchymal stromal cells (MSCs) are multipotent stem cells that can differentiate into several cell types. In addition, many studies have shown that MSCs modulate the immune response. However, little information is currently available regarding the maintenance of immunomodulatory characteristics of MSCs through passages. Therefore, we investigated and compared cytokine and gene expression levels from adipose (AD) and bone marrow (BM)-derived MSCs relevant to immune modulation from early to late passages. METHODS: MSC immunophenotype, growth characteristics, cytokine expressions, and gene expressions were analyzed. RESULTS: AD-MSCs and BM-MSCs had similar cell morphologies and surface marker expressions from passage 4 to passage 10. Cytokines secreted by AD-MSCs and BM-MSCs were similar from early to late passages. AD-MSCs and BM-MSCs showed similar immunomodulatory properties in terms of cytokine secretion levels. However, the gene expressions of tumor necrosis factor-stimulated gene (TSG)-6 and human leukocyte antigen (HLA)-G were decreased and gene expressions of galectin-1 and -3 were increased in both AD- and BM-MSCs with repeated passages. CONCLUSION: Our study showed that the immunophenotype and expression of immunomodulation-related cytokines of AD-MSCs and BM-MSCs immunomodulation through the passages were not significantly different, even though the gene expressions of both MSCs were different.

Sweet's Syndrome Associated with Graves' Disease

Sweet's syndrome, or acute febrile neutrophilic dermatosis, occurs in association with autoimmune diseases such as Hashimoto's thyroiditis but is rare in Graves' disease, in which all cases are induced by propylthiouracil (PTU). We report a case of Sweet's syndrome in a patient with Graves' disease treated with methimazole (MMI) during three weeks. A 34-year-old man presented with the acute onset of high fever, skin rashes on the whole body, arthralgia, and acroparesthesia. Laboratory results showed leukocytosis and elevated C-reactive protein. MMI first stopped and antibiotics and antihistamine therapy started, but his symptoms dramatically improved after oral prednisolone. Graves' disease has again been treated by MMI because of his aggravated ophthalmopathy. After one year of retreatment with MMI, there has been no recurrence of Sweet's syndrome, supporting that Sweet's syndrome in this case was not related to MMI exposure. To our knowledge, this is the first report of Sweet's syndrome associated with Graves' disease per se but not PTU or MMI use.

Dermatomyositis: A Rare Extra-intestinal Manifestation of Ulcerative Colitis

Inflammatory myositis as an extra-intestinal manifestation of inflammatory bowel disease (IBD) is rare. Coexistence of immuno-mediated diseases in patients with IBD and myositis suggests a common etiopathogenic mechanism underlying these conditions. The current report refers to a rare case of a 45-year-old Korean female with ulcerative colitis (UC) who developed dermatomyositis. She presented with skin rash and proximal muscle weakness, and her disease activity of UC was in remission state. Electromyography, magnetic resonance imaging, and deltoid muscle biopsy were performed. She was diagnosed with dermatomyositis associated with UC and treatment with glucocorticoids and azathioprine resulted in improvement in muscle power and skin rash. Clinicians should be aware of this unusual extra-intestinal manifestation.

Immunomodulatory Function of Mesenchymal Stem Cells for Rheumatoid Arthritis

Developments in our comprehension of the autoimmune and inflammation mechanisms in rheumatoid arthritis (RA) have produced targeted therapies that block aberrant immune cells and cytokine networks, and improved treatment of RA patients considerably. Nevertheless, limitations of these treatments include incomplete treatment response, adverse effects requiring drug withdrawal, and refractory cases. Hence, many researchers have redirected efforts towards investigation of other biological aspects of RA, including the mechanisms driving joint tissue repair and balanced immune regulation. This investigation focuses on mesenchymal stem cell (MSC) research, with the ultimate goal of developing interventions for immune modulation and repair of damaged joints. MSCs are multipotent cells capable of differentiating into mesodermal lineage cells. These cells have also attracted interest for their anti-inflammatory and immunomodulatory capacities. They have many distinctive immunological properties, inhibiting the proliferation and production of cytokines by T, B, natural killer, and dendritic cells. Indeed, MSCs have the capacity to regulate immunity-induced peripheral tolerance, suggesting they can be used as therapeutic tools in RA. This review discusses properties of MSCs, in vitro studies, animal studies, and clinical trials involving MSCs. Our review discusses the current knowledge of the mechanisms of MSC-mediated immunosuppression and potential therapeutic uses of MSCs in RA.

Successful Use of Extracorporeal Membrane Oxygenation in Diffuse Alveolar Hemorrhage Secondary to Systemic Lupus Erythematosus / 대한구급학회지

Diffuse alveolar hemorrhage (DAH) is an uncommon complication in patients with systemic lupus erythematosus (SLE), and mortality remains high. In recent years, cases of DAH due to SLE treated with extracorporeal membrane oxygenation (ECMO) have rarely been reported. The authors present a case of a 43-year-old woman with SLE who had rapidly aggravating dyspnea and hemoptysis. She was diagnosed as having DAH with refractory respiratory failure and was successfully managed with veno-venous ECMO. We propose ECMO as a useful salvage therapy in patients with alveolar hemorrhage secondary to SLE who are failing conventional ventilatory support.

Successful Use of Extracorporeal Membrane Oxygenation in Diffuse Alveolar Hemorrhage Secondary to Systemic Lupus Erythematosus / 대한중환자의학회지

Diffuse alveolar hemorrhage (DAH) is an uncommon complication in patients with systemic lupus erythematosus (SLE), and mortality remains high. In recent years, cases of DAH due to SLE treated with extracorporeal membrane oxygenation (ECMO) have rarely been reported. The authors present a case of a 43-year-old woman with SLE who had rapidly aggravating dyspnea and hemoptysis. She was diagnosed as having DAH with refractory respiratory failure and was successfully managed with veno-venous ECMO. We propose ECMO as a useful salvage therapy in patients with alveolar hemorrhage secondary to SLE who are failing conventional ventilatory support.

Differences in Clinical Manifestations and Outcomes between Adult and Child Patients with Henoch-Schonlein Purpura

We aimed to investigate differences in clinical manifestations and outcomes between adult and child patients with Henoch-Schonlein purpura (HSP), and to analyze the factors associated with poor prognosis for HSP nephritis. This retrospective 10-yr study enrolled 160 patients with HSP who visited Severance Hospital. Purpura was mostly detected in lower extremities, but purpura in upper extremities was more frequently observed in adults than children (41.7% vs 19.3%). Children had a greater frequency of arthralgia (55.4% vs 27.1%), while adults had a greater frequency of diarrhea (20% vs 1.6%). Anemia, elevated C-reactive protein, and level of IgA were more frequently observed in adults (25% vs 7.1%, 65.6% vs 38.4%, 26.3% vs 3.5%). Renal involvement in adults was more severe than in children (79.2% vs 30.4%). Chronic renal failure showed a significant difference in outcomes of HSP between adults (10.4%) and children (1.8%) after a follow up period of an average of 27 months. Furthermore, renal insufficiency at diagnosis was significantly related to the progression to chronic renal failure. Our results showed several differences in the clinical features of HSP between adults and children. Adults with HSP had a higher frequency of renal insufficiency and worse renal outcomes than children. Renal insufficiency at diagnosis might be of predictive value for the progression to chronic renal failure in HSP patients.

Typical 18-FDG-PET/CT Findings of Polymyalgia Rheumatica: A Case Report

Polymyalgia rheumatica (PMR) is an inflammatory rheumatic condition characterized by generalized pain and morning stiffness in the shoulders, hip girdle, and neck. Since the pathogenesis of PMR is still uncertain, the diagnosis of PMR depends on clinical features. There have been several studies regarding radiological tools for the diagnosis of PMR. Recent studies using 18-FDG-PET showed bursitis, synovitis, uptake in the spinous process and asymptomatic large-vessel vasculitis in PMR patients. However, there was no report on the efficacy of 18-FDG-PET for diagnosis of PMR in Korea. Here, we are first reporting a case of a Korean patient with PMR, who had radiological findings including bursitis, synovitis, uptake in the spinous process and asymptomatic large-vessel vasculitis on 18-FDG-PET/CT.

Two Cases of Refractory Thrombocytopenia in Systemic Lupus Erythematosus that Responded to Intravenous Low-Dose Cyclophosphamide

Treatment of thrombocytopenia in systemic lupus erythematosus (SLE) is considered in cases of current bleeding, severe bruising, or a platelet count below 50,000/microliter. Corticosteroid is the first choice of medication for inducing remission, and immunosuppressive agents can be added when thrombocytopenia is refractory to corticosteroid or recurs despite it. We presented two SLE patients with thrombocytopenia who successfully induced remission after intravenous administration of low-dose cyclophosphamide (CYC) (500 mg fixed dose, biweekly for 3 months), followed by azathioprine (AZA) or mycophenolate mofetil (MMF). Both patients developed severe thrombocytopenia in SLE that did not respond to pulsed methylprednisolone therapy, and started the intravenous low-dose CYC therapy. In case 1, the platelet count increased to 50,000/microliter after the first CYC infusion, and remission was maintained with low dose prednisolone and AZA. The case 2 achieved remission after three cycles of CYC, and the remission continued with low dose prednisolone and MMF.

A Case of Atypical Giant Cell Arteritis Presenting as a Fever of Unknown Origin

Giant cell arteritis (GCA) is a systemic vasculitis predominantly found in individuals of Northern European ancestry over 50 years of age. Typically it presents with new-onset persistent headache, claudication of jaws, and existence of an abnormal temporal artery. However, the diagnosis of GCA and the assessment of its activity remain challenging, especially in patients presenting with a variety of non-specific symptoms and laboratory tests. In those cases, 18F-fluorodeoxy-glucose positron emission tomography (18F-FDG-PET) is useful for the diagnosis of GCA. Recently, the number of foreign patients who visit domestic hospitals for medical care is increasing in Korea. Here-in, we report a Russian patient who was admitted to our hospital with fever of unknown origin (FUO). FUO study was performed to search for infection or malignancy and GCA was suspected by 18F-FDG-PET. The patient was eventually diagnosed with GCA by random temporal artery biopsy.

A Case of Neck Abscess Caused by Salmonella Serotype D in a Patient with Liver Cirrhosis

Non-typhoidal salmonellosis, which is increasing nowadays in Korea as well as in the developed countries, is manifested as enteritis in most cases, but it also encompasses bacteremia, intraabdominal infections, and bone, joint and soft tissue infections. These rare diseases are known to result from primary gastrointestinal infection and subsequent bacteremia with or without symptoms. We experienced a case of neck abscess caused by Salmonella serotype D, which is a rare but important differential diagnosis of neck abscess. We herein report it.

Molecular Epidemiology and Antimicrobial Resistance of Methicillin-resistant Staphylococcus aureus Isolated from Nasal Swab at Intensive Care Unit / 결핵및호흡기질환

BACKGROUND: Methicillin-resistant Staphylococcus aureus (MRSA) is the most common organism associated with nosocomial infections. MRSA infections are becoming increasing important because they have emerged no only as healthcare-associated (HA) infections but also as community-associated (CA) ones. This study examined the moleculo-epidemiology of MRSA, which was isolated from nasal swabs in the intensive care unit (ICU) at Konyang University Hospital. MRSA are classified into HA-MRSA and CA-MRSA. METHODS: From June to September 2006, 353 patients who were admitted to the ICU in Konyang University Hospital were enrolled in this study. Single nasal swabs were obtained for culture in the ICU on the 1st day. Pulsed-field gel electrophoresis and the antimicrobial resistant patterns were analyzed between HA- and CA-MRSA. An antimicrobial sensitivity test was also performed. RESULTS: Forty two strains of MRSA were isolated from 353 patients (11.9%). Among the 42 isolates, HA-MRSA and CA-MRSA were found in 33 (78.6%), and 9 (21.4%), respectively. Eleven different PFGE types (type A to K) were identified. Types A (n=9) and B (n=7) were the most common for HA-MRSA, and types A (n=2) and B (n=2) were identified in CA-MRSA. The proportion of types A and B in CA-MRSA (44.4%) was similar to that in HA-MRSA (48.5%). The rates of resistance rates to erythromycin and ciprofloxacin were higher in HA-MRSA than in CA-MRSA. CONCLUSION: The rate of isolation of MRSA in an ICU setting was 11.9%. HA-MRSA was isolated more frequently than CA-MRSA. The rate of resistance of HA-MRSA to erythromycin and ciprofloxacin was higher than that of CA-MRSA. Despite the small number of subjects, the main isolates (type A and B) of CA-MRSA were similar to those of HA-MRSA.

Peg-interferon induced autoimmune thyroiditis / 대한내과학회지

Peg-interferon is the main therapeutic agent in patients infected with the hepatitis C virus. It is rather safe, but it is known to induce production of autoantibodies and it can lead to the occurrence of autoimmune disease. Interferon induced thyroiditis is considered a major clinical problem for patients who receive interferon therapy. We recently experienced a 36 year-old woman who developed peg-interferon induced thyroiditis. To the best of our knowledge, this is a rare case report in Korea. We report here on this casea long with a review of the literature.

Synchronous Roentgenographically Occult Lung Carcinoma Treated with Argon Plasma Coagulation in a Patient with Resectable Primary Lung Cancer / 결핵및호흡기질환

We treated synchronous double primary lung cancers, where one site resulted from CIS disease, with lobectomy and argon plasma coagulation (APC) in a patient who couldn't tolerate pneumonectomy, which resulted in a reduction of the extent of surgery. APC could be a reasonable alternative for CIS disease of lung in inoperable patients.

A Case of Acute Lower Gastrointestinal Bleeding from Appendiceal Ulcer / 대한소화기내시경학회지

The common causes of acute lower gastrointestinal bleeding include diverticulosis, colonic vascular ectasia, neoplasm and colitis. Acute lower gastrointestinal bleeding from the appendix is a very rare malady and the causes are reported as angiodysplasia, diverticulum, appendicitis and endometriosis. We report here on the case of a 47-year-old man, who was taking enteric coated aspirin, with severe lower gastrointestinal bleeding that was due to appendiceal ulcer. An active bleeding was identified as coming from the appendiceal orifice during colonoscopy. He was treated by simple appendectomy. Histologic evaluation showed ulceration with both acute and chronic inflammation, along with thickened vessel walls in the submucosa of the appendix.