Juvenile temporal arteritis (JTA) is a localized nodular arteritis confined to the temporal artery without evidence of systemic inflammation, and it occurs mainly in patients younger than 50 years. From the first case report, the pathological features of JTA have been suspected to be the morphological equivalent of Kimura disease (KD), which has been supported further by the concurrent cases of JTA with KD. We present the first case of bilateral JTA accompanying KD, which was confirmed by histological and ultrasound evaluations and supports the hypothesis that JTA is a manifestation of KD. The un-excised JTA lesion was resolved completely after corticosteroid therapy with no recurrence.
Adrenal Cortex Hormones ; Angiolymphoid Hyperplasia with Eosinophilia ; Arteritis ; Giant Cell Arteritis ; Humans ; Inflammation ; Recurrence ; Temporal Arteries ; Ultrasonography

BACKGROUND/AIMS: We investigated the electromyography (EMG) findings and demographic, clinical, and laboratory features that may predict the development of malignancy in patients with idiopathic inflammatory myopathy (IIM). METHODS: In total, 61 patients, 36 with dermatomyositis and 25 with polymyositis, were included. Patients were divided into those with and without malignancies, and comparisons were made between the groups in terms of their demographic, clinical, laboratory, and EMG findings. RESULTS: The frequencies of malignancies associated with dermatomyositis and polymyositis were 22% and 8%, respectively. Patients with malignancies showed a significantly higher incidence of dysphagia (odds ratio [OR], 21.50; 95% confidence interval [CI], 3.84 to 120.49), absence of interstitial lung disease (ILD; OR, 0.12; 95% CI, 0.01 to 0.98), and complex repetitive discharge (CRD) on the EMG (OR, 26.25; 95% CI, 2.67 to 258.52), versus those without. After adjustment for age, dysphagia and CRD remained significant, while ILD showed a trend for a difference but was not statistically significant. Multivariate analysis revealed that the CRD conferred an OR of 25.99 (95% CI, 1.27 to 531.86) for malignancy. When the frequency of malignancy was analyzed according to the number of risk factors, patients with three risk factors showed a significantly higher incidence of malignancy, versus those with fewer than two (p = 0.014). CONCLUSIONS: We demonstrated for the first time that CRD on the EMG was an additional independent risk factor for malignancy in IIM. Further studies on a larger scale are needed to confirm the importance of CRD as a risk factor for malignancy in IIM.
Action Potentials ; Adult ; Aged ; Dermatomyositis/complications/*diagnosis/physiopathology ; *Electromyography ; Female ; Humans ; Logistic Models ; Male ; Middle Aged ; Multivariate Analysis ; Muscle, Skeletal/*innervation/pathology ; Neoplasms/*etiology ; Odds Ratio ; Polymyositis/complications/*diagnosis/physiopathology ; Predictive Value of Tests ; Retrospective Studies ; Risk Factors

Paget's disease of bone (PDB) is a disorder featuring high-level bone turnover associated with the presence of disorganized and immature bone tissue with excessive levels of fibrosis. The risk of deformity is very high. The etiology of PDB is not well understood, but includes both genetic and environmental factors among which is bone trauma. Hypercalcemia can occur as a complication of PDB in patients who are immobilized and dehydrated. However, to date, no case of severe hypercalcemia with metastatic calcifications in multiple organs has been reported in any PDB patient. The drugs of choice for treatment of PDB are bisphosphonates. These drugs effectively suppress bone turnover. Patients with extensive PDB may require higher doses of bisphosphonates, and acquired resistance to a particular bisphosphonate may be overcome by use an alternative drug. Here, we report a case of suspicion of PDB. The patient presented with hypercalcemia and metastatic calcifications and his condition improved dramatically after treatment with zoledronic acid.
Bone and Bones ; Congenital Abnormalities ; Diphosphonates ; Fibrosis ; Humans ; Hypercalcemia* ; Imidazoles ; Osteitis Deformans ; Wounds and Injuries

Paget's disease of bone (PDB) is a disorder featuring high-level bone turnover associated with the presence of disorganized and immature bone tissue with excessive levels of fibrosis. The risk of deformity is very high. The etiology of PDB is not well understood, but includes both genetic and environmental factors among which is bone trauma. Hypercalcemia can occur as a complication of PDB in patients who are immobilized and dehydrated. However, to date, no case of severe hypercalcemia with metastatic calcifications in multiple organs has been reported in any PDB patient. The drugs of choice for treatment of PDB are bisphosphonates. These drugs effectively suppress bone turnover. Patients with extensive PDB may require higher doses of bisphosphonates, and acquired resistance to a particular bisphosphonate may be overcome by use an alternative drug. Here, we report a case of suspicion of PDB. The patient presented with hypercalcemia and metastatic calcifications and his condition improved dramatically after treatment with zoledronic acid.
Bone and Bones ; Congenital Abnormalities ; Diphosphonates ; Fibrosis ; Humans ; Hypercalcemia* ; Imidazoles ; Osteitis Deformans ; Wounds and Injuries

Dermatomyositis (DM) is a kind of systemic autoimmune disease characterized by chronic inflammation leading to progressive weakness of proximal muscles and typical cutaneous lesions. DM has been known to be strongly associated with malignancies, such as ovarian, lung, and gastric cancers. Prostate cancer is rarely associated with DM and, to our knowledge, no case of prostate cancer has been reported in patients with DM combined with interstitial lung disease in Korea. Here, we report a case of DM with bronchiolitis obliterans organizing pneumonia that was simultaneously diagnosed as advanced prostate cancer by a thorough evaluation for hidden malignancy.
Autoimmune Diseases ; Cryptogenic Organizing Pneumonia ; Dermatomyositis ; Humans ; Inflammation ; Korea ; Lung ; Lung Diseases, Interstitial ; Muscles ; Prostate ; Prostatic Neoplasms ; Stomach Neoplasms

A 73-year-old man with a history of hypertension and ascending aortic dissection was hospitalized for aggravated abdominal pain and general ache for 3 months. Follow-up CT showed aggravated abdominal aortic hematoma with aneurysm, atherosclerotic periaortitis and bilateral hydronephrosis. An initial laboratory finding showed elevated levels of inflammatory markers and renal dysfunction. Positron emission tomography-CT showed an increased standardized uptake values level in the aortic arch, descending thoracic aorta, major branch, abdominal aorta, and common iliac artery. For bilateral hydronephrosis, a double J catheter insertion was performed. Tissue specimens obtained from previous surgery on the aorta indicated the infiltration of lympho-plasma cells without granuloma formation in the aortic wall. After a combined therapy of high dose steroid therapy with azathioprine, the patient's initial complaints of abdominal pain, weakness and azotemia improved. This case was diagnosed as chronic periaortitis based on aortic inflammation at biopsy, which was complicated with retroperitoneal fibrosis and ureteric obstruction.
Abdominal Pain ; Aneurysm ; Aorta ; Aorta, Abdominal ; Aorta, Thoracic ; Azathioprine ; Azotemia ; Biopsy ; Catheters ; Electrons ; Follow-Up Studies ; Granuloma ; Hematoma ; Hydronephrosis ; Hypertension ; Iliac Artery ; Inflammation ; Retroperitoneal Fibrosis ; Ureter

OBJECTIVE: This study sought to investigate independent predictive factors for subclinical atherosclerosis in Korean patients with rheumatoid arthritis (RA). METHODS: We used high-resolution B-mode ultrasonography to measure the carotid artery intima-media thickness (IMT) and carotid plaque in 367 patients with RA. Detailed information on the demographic characteristics, cardiovascular (CV) risk factors, and RA disease characteristics were collected on all subjects. The relationship of the carotid artery IMT and carotid plaque to relevant clinical and laboratory variables were examined. RESULTS: Old age and male sex had the most significant association with increased IMT and presence of plaque than other factors. Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and mKHAQ (Korean version of modified health assessment questionnaire) were significantly associated with both increased IMT and presence of plaque after univariate analysis adjusting for age and sex. A multivariable logistic regression analysis revealed that ESR and TJC68 were independent factors associated with the presence of plaque (p<0.001 and p=0.019, respectively). There was a significant linear correlation between the number of plaques and ESR (p<0.001 and R2=0.07). CONCLUSION: Our results indicated that markers of systemic inflammation contributed significantly to subclinical atherosclerosis in patients with RA. We emphasize the need for aggressive control of RA disease activity in patients who persistently demonstrate highly elevated ESR levels.
Arthritis, Rheumatoid ; Atherosclerosis ; Blood Sedimentation ; C-Reactive Protein ; Cardiovascular Diseases ; Carotid Arteries ; Cohort Studies ; Humans ; Inflammation ; Logistic Models ; Male ; Risk Factors

May-Thurner syndrome, which refers to an iliofemoral venous thrombosis caused by chronic compression of the left common iliac vein by the right common iliac artery, usually manifests as left lower extremity pain and swelling. The syndrome is particularly evident in patients with health conditions including obesity, smoking, pregnancy, surgery, or prolonged immobility. Antiphospholipid syndrome consists of arterial or venous thrombosis and the association of lupus anticoagulant or anticardiolipin antibodies. Most common clinical manifestations include pregnancy loss and deep vein thrombosis. We experienced a rare case of May-Thurner syndrome concurrent with antiphospholipid syndrome, secondary to systemic lupus erythematosus. The patient was treated successfully by catheter-directed percutaneous thrombectomy, venous thrombolysis, and stent insertion, followed by oral anticoagulant therapy.
Antibodies, Anticardiolipin ; Antiphospholipid Syndrome ; Humans ; Iliac Artery ; Iliac Vein ; Lower Extremity ; Lupus Coagulation Inhibitor ; Lupus Erythematosus, Systemic ; May-Thurner Syndrome ; Obesity ; Postthrombotic Syndrome ; Pregnancy ; Smoke ; Smoking ; Stents ; Thrombectomy ; Venous Thrombosis

May-Thurner syndrome, which refers to an iliofemoral venous thrombosis caused by chronic compression of the left common iliac vein by the right common iliac artery, usually manifests as left lower extremity pain and swelling. The syndrome is particularly evident in patients with health conditions including obesity, smoking, pregnancy, surgery, or prolonged immobility. Antiphospholipid syndrome consists of arterial or venous thrombosis and the association of lupus anticoagulant or anticardiolipin antibodies. Most common clinical manifestations include pregnancy loss and deep vein thrombosis. We experienced a rare case of May-Thurner syndrome concurrent with antiphospholipid syndrome, secondary to systemic lupus erythematosus. The patient was treated successfully by catheter-directed percutaneous thrombectomy, venous thrombolysis, and stent insertion, followed by oral anticoagulant therapy.
Antibodies, Anticardiolipin ; Antiphospholipid Syndrome ; Humans ; Iliac Artery ; Iliac Vein ; Lower Extremity ; Lupus Coagulation Inhibitor ; Lupus Erythematosus, Systemic ; May-Thurner Syndrome ; Obesity ; Postthrombotic Syndrome ; Pregnancy ; Smoke ; Smoking ; Stents ; Thrombectomy ; Venous Thrombosis

The salient features of Sweet syndrome, also known as acute febrile neutrophilic dermatosis, are pyrexia, elevated neutrophil count, painful red papules, nodules, plaques, and an infiltrate that consists mainly of mature neutrophils diffusely distributed in the upper dermis. In addition to idiopathic forms of the syndrome, malignancies, inflammatory bowel disease, infections, pregnancy, and drugs have been associated with Sweet syndrome. Disseminated gonococcal infection (DGI) results from bacteremic dissemination of Neisseria gonorrhoeae. The most common presentation of DGI is arthritis-dermatitis syndrome. About 75% of DGI patients display a characteristic dermatitis consisting of discrete papules and pustules, often with a hemorrhagic component. We report a case of DGI presenting as Sweet syndrome.
Dermatitis ; Dermis ; Fever ; Humans ; Inflammatory Bowel Diseases ; Neisseria gonorrhoeae ; Neutrophils ; Pregnancy ; Sweet Syndrome