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.

The Role of Autoantibodies in Idiopathic Inflammatory Myopathies

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of autoimmune muscle diseases with systemic involvement. Patients with IIM present with varying degrees of muscle disease, cutaneous manifestations, and internal organ involvement. The diagnosis and classification of IIM is based primarily on the classification system composed of clinical features, laboratory value and muscle biopsy. In addition, the identification and characterization of myositis-related autoantibodies can help diagnosis and classification. Recently, many studies have also demonstrated that the physician can define the clinical syndromes, establish treatment strategy and predict outcomes based on the patients' myositis-specific autoantibodies (MSA) and myositis-associated antibodies (MAA) profiles. MSAs are found exclusively in IIMs and facilitate the identification of subsets of patients with relatively homogeneous clinical features. MAAs are frequently found in association with other MSA; however, they may also be detected in various connective diseases.

Neuromyositis: A Rare Extramuscular Manifestation of Dermatomyositis

Dermatomyositis (DM) and polymyositis (PM) are representative idiopathic inflammatory myopathies characterized by symmetric and progressive proximal muscle weakness. Especially, DM is identified by characteristic skin lesions and has many extramuscular manifestations including various cardiac abnormalities, interstitial lung disease, and malignancy. However, involvement of peripheral nervous system in DM/PM is very rare and less known. The term “Neuromyositis” was introduced by Senator in 1893 to describe the concomitant involvement of the peripheral nervous system in DM/PM. Since then, a very few cases of neuromyositis have been reported mainly in the United States and Europe. Therefore, the pathogenetic mechanism and disease progression are unclear. In recent years, a few more cases were reported in Asia, specifically, China and Japan; however, none in Korea. Here, we describe a case of DM-associated neuromyositis in a 42-year-old man in Korea and review previous publications through literature research.

Cancer of Unknown Primary Site Mimicking Retroperitoneal Fibrosis / 대한내과학회지

Retroperitoneal fibrosis (RPF) is a rare disease characterized by marked fibro-inflammatory tissue in the retroperitoneum. Approximately 70% of cases of RPF are idiopathic, while the rest can be secondary to several other causes. The diagnosis is mainly obtained by imaging modalities such as computed tomography (CT). However, histological examination should be considered when the clinical manifestations and imaging studies suggest malignancy. In particular, in malignant diseases with retroperitoneal metastases, abnormal collagen plaques are formed from an exuberant desmoplastic response, which may not be distinguishable from RPF on CT scans. Therefore, even if CT suggests RPF, biopsy is essential to identify malignant disease because it typically results in a fatal prognosis. Here, we report a case of metastatic adenocarcinoma of unknown primary site that developed only in the retroperitoneum and was initially diagnosed as RPF based on CT findings.

Prevalence of Pulmonary Arterial Hypertension in Korean Adult Patients with Systemic Sclerosis: Result of a Pilot Echocardiographic Screening Study

BACKGROUND: Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality among patients with systemic sclerosis (SSc). Early detection and prompt treatment of PAH associated with SSc (SSc-PAH) result in better prognosis. We conducted echocardiographic study to presume the prevalence of PAH in Korean adult SSc patients and to diagnose SSc-PAH in their early stages with right heart catheterization (RHC). METHODS: We performed free of charge echocardiographic study including 37 adult SSc patients at the Chungnam National University Hospital. The possibility of PAH is determined by the estimation of pulmonary arterial pressure by peak tricuspid regurgitation velocity of > 3.0 m/s. Patients with possible PAH were recommended to undergo RHC to confirm the diagnosis. RESULTS: In 37 patients, 8 patients were suspected with PAH. Among them, 6 patients agreed to be examined with RHC, and 4 were confirmed with PAH. The prevalence of possible PAH was 21.6% (8 of 37 patients), and that of confirmed PAH was 10.8% (4 of 37 patients). Four patients who were confirmed with SSc-PAH through RHC have been treated with specific pulmonary vasodilators and maintained stable. CONCLUSION: Eight patients (21.6%) were possible PAH and 4 (10.8%) were diagnosed as SSc-PAH by RHC after the echocardiographic screening study of 37 adult SSc patients.

Iliopsoas Bursitis after Transfemoral Coronary Angiography / 계명의대학술지

A 53-year-old male was admitted to our hospital for evaluation of the second kidney transplant. A Iliopsoas Bursitis was detected on diagnostic abdominopelvic computed tomography. He had undergone a coronary angiography (CAG) due to chest pain 1 month prior to his visit. At that time, he had experienced pain on his right back and flank for some time. We found no other causes or predisposing factors associated with that problem. Thus, we report on a case of iliopsoas bursitis after CAG.

Clinical Implication of 18F-FDG-PET in Diagnosing and Monitoring Disease Activity in a Case of Subclinical Stage of Giant Cell Arteritis

Giant cell arteritis (GCA) is a systemic vasculitis which typically occurs in persons over 50 years old. GCA is closely related to polymyalgia rheumatica (PMR). A temporal artery biopsy is the gold standard test for the diagnosis of GCA. Recently, there is increasing evidence for the role of 18F-fluorodeoxyglucose positron emission tomography (18F-FDG-PET) in diagnosis of vasculitis. Here, we report on a case of a 67-year-old Korean male who was diagnosed with atypical GCA in subclinical stage concomitant with PMR by 18F-FDG-PET. After treatment, abnormal findings of 18F-FDG-PET were improved.

A Case of Intravascular Large B-cell Lymphoma Mimicking Systemic Lupus Erythematosus / 대한내과학회지

Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of non-Hodgkin's lymphoma (NHL) and that progresses rapidly and is usually fatal. Because it usually presents with nonspecific symptoms, such as fever, the early diagnosis of IVLBCL is very difficult and it is often misdiagnosed as another disease. Systemic lupus erythematosus (SLE) is an autoimmune disease that affects various organs. The clinical manifestation of SLE ranges from rash and arthritis through anemia and thrombocytopenia to serositis, nephritis, seizures, and psychosis. Thus, it can be easily confused with many other disorders. We report a case of IVLBCL mimicking SLE in the initial diagnosis.

A Case of Hypertrophic Osteoarthropathy Associated with Lung Metastasis of Parotid Gland Mucoepidermoid Carcinoma

Hypertrophic osteoarthropathy is a syndrome characterized by periosteal new bone formation, arthritis, and clubbing of the fingers and toes. The majority of cases occur secondarily to the conditions associated with pulmonary, cardiac, gastrointestinal disorders or other systemic diseases. There are many cases with malignancy worldwide. We report the first patient who had hypertrophic osteoarthropathy due to metastatic cancer after surgical removal for mucoepidermoid carcinoma of the parotid gland.

Localized Mesenteric Vasculitis in a Patient with Polymyalgia Rheumatica

Polymyalgia rheumatica (PMR) is an uncommon disorder characterized by bilateral pain and stiffness in the shoulder and pelvic girdles. Polymyalgia rheumatica and giant cell arteritis (GCA) occur in the same patient population and share a common pathogenesis. Giant cell arteritis predominantly affects the cranial arteries and rarely involves the gastrointestinal tract. Moreover, giant cell arteritis has rarely been reported in Asians. Here, we present a case with 62-year-old Asian woman who developed polymyalgia rheumatica with localized vasculitis in the mesenteric arteries.

Osteonecrosis of the Humeral Head after Cerebral Angiography

A 79-year-old woman was admitted to our hospital for shoulder pain. A physical examination revealed a tender right shoulder with limitation of active, and preservation of passive, motion. She had undergone a cerebral angiography with coil embolization two months prior to admission. After the procedure, she was presented with pain in the right upper arm and shoulder. Due to persistent shoulder pain, an MRI of the shoulder was performed, and osteonecrosis of the humeral head was detected. We present a case of osteonecrosis of the humeral head after cerebral angiography.

Regulatory B Cells Are Inversely Associated with Disease Activity in Rheumatoid Arthritis

PURPOSE: The function of regulatory B lymphocytes is known to be abnormal in inflammatory diseases. However, a recent study indicates that IL-10+ B cells seem to be expanded in rheumatoid arthritis (RA). Therefore, the state of IL-10+ B cells in the peripheral blood from RA patients and healthy controls were investigated. MATERIALS AND METHODS: CD19+ cells in peripheral blood mononuclear cells were purified from blood samples of RA patients and age and gender-matched healthy controls, and stimulated with CD40 ligand and CpG for 48 hours. Then, intracellular IL-10 in CD19+ cells was analyzed using flow cytometry. RESULTS: There was no significant difference in the proportion of IL-10+ B cells between 10 RA patients and 10 healthy controls (RA, 0.300+/-0.07 vs. healthy control 0.459+/-0.07, p=0.114). The proportion of induced IL-10+ B cells to total B cells in RA patients was significantly higher than those in controls (RA, 4.44+/-3.44% vs. healthy control 2.44+/-1.64%, p=0.033). However, the proportion of IL-10+ B cells to total B cells correlated negatively with disease activity in RA patients (r=-0.398, p=0.040). Erythrocyte sedimentation rate or C-reactive protein or medication was not associated with the proportion of IL-10+ B cells. CONCLUSION: The proportion of induced IL-10+ B cell increased in RA patients compared to healthy control, however, negatively correlated with disease activity in RA.

Eosinophilic Fasciitis as the Initial Manifestation of Graft-Versus-Host Disease

Eosinophilic fasciitis (EF) is a relatively rare disorder characterized by eosinophilia of the peripheral blood and inflammation of fascia and skin. The etiology of EF is unknown but it can precede or occur concomitantly with hematologic disorders including malignancy. EF can rarely occur after bone marrow transplantation and is a feature of chronic graft-versus-host disease (cGVHD). We report a 49-year-old man who developed EF as the first manifestation of cGVHD after allogeneic hematopoietic stem cell transplantation (AlloHSCT).

Associations between TBX21 Gene Polymorphisms and Korean Patients with Behcet's Disease / 대한류마티스학회지

OBJECTIVE: Behcet's disease (BD) is a chronic systemic inflammatory disease with unknown etiology. A number of clinical and laboratory findings suggest a strongly polarized Th1 immune response in BD. T-bet is a newly identified Th1 specific T-box transcription factor selectively expressed in Th1 cells. However, it is not yet clear whether the T-bet protein is involved in the proposed Th1-mediated pathogenesis of BD at the transcriptional level. Therefore, this study investigated the potential associations of two single nucleotide polymorphisms (SNPs) at positions -99 (C/G) and -1993 (T/C) in the exon and promoter regions of the TBX21 gene with susceptibility to BD in the Korean population. METHODS: 105 patients with BD and 105 healthy controls were studied. All subjects were genotyped using restriction fragment length polymorphism analysis. The genotypes of the two groups were compared with the chi-square or Fisher's exact tests. RESULTS: The genotypic and allelic distributions of the two SNPs did not differ significantly between the two groups. Furthermore, no associations between the polymorphisms and clinical manifestations were found, except a central nervous system manifestation and arthritis. Furthermore, no associations between the polymorphisms and severity were identified. CONCLUSION: TBX21 gene polymorphisms were not associated with susceptibility, clinical manifestations, or severity of BD in the Korean population.

Interstitial pneumonitis associated with infliximab therapy in a rheumatoid arthritis patient / 대한내과학회지

Tumor necrosis factor (TNF)-alpha inhibitors are well-established biological agents for the treatment of a wide variety of chronic autoimmune diseases and inflammatory conditions, including rheumatoid arthritis (RA), ankylosing spondylitis, and psoriatic arthritis. Although these drugs have been noted to have good safety profiles, some important side effects, including infection, injection site reactions, lupus-like syndrome, congestive heart failure, and malignancies have been reported. Therefore, utilization of TNF-alpha inhibitors demands caution. Interstitial pneumonitis is a very rare complication of TNF-alpha inhibitors. We report here a 71-year-old man with RA who developed interstitial pneumonitis after the third infusion of infliximab.

Acute lymphoblastic leukemia initially presenting with polymyalgia rheumatica / 대한내과학회지

Polymyalgia rheumatica (PMR) is a clinical syndrome of unknown etiology characterized by aching and stiffness in the shoulder girdle, pelvic girdle, and neck that occurs in people over the age of 50 years. New-onset proximal muscle pain and stiffness in subjects older than 50 are the most common symptoms of PMR, but polymyalgic pain can also be present in patients with malignant neoplasms. The musculoskeletal system may be involved in malignancy either directly or indirectly, particularly in cases involving hematological malignancies. Only a few cases of cancer-associated PMR have been reported worldwide, and this is the first in Korea. We report the case of a 58-year-old man diagnosed with PMR associated with acute lymphoblastic leukemia.