Bilateral Involvement of Juvenile Temporal Arteritis Associated with Kimura Disease

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.

Complex repetitive discharge on electromyography as a risk factor for malignancy in idiopathic inflammatory myopathy

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.

A Case of Prostate Cancer Associated with Dermatomyositis and Interstitial Lung Disease / 대한내과학회지

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.

Hypercalcemia Refractory to Pamidronate Associated with Metastatic Calcifications in Multiple Organs / 대한내과학회지

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.

Hypercalcemia Refractory to Pamidronate Associated with Metastatic Calcifications in Multiple Organs / 대한내과학회지

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.

A Case of Chronic Periaortitis with Retroperitoneal Fibrosis

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.

A Case of May-Thurner Syndrome with Systemic Lupus Erythematosus in Antiphospholipid Syndrome / 대한내과학회지

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.

Identification of Independent Predictive Factors for Atherosclerosis in Rheumatoid Arthritis: Based on KARRA Cohort Study

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.

A Case of May-Thurner Syndrome with Systemic Lupus Erythematosus in Antiphospholipid Syndrome / 대한내과학회지

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.

Disseminated Gonococcal Infection Presenting as Sweet Syndrome / 대한내과학회지

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.

Systemic Lupus Erythematosus Preceded by Distal Renal Tubular Acidosis / 대한내과학회지

Distal renal tubular acidosis (RTA) is characterized by a decreased net H+ secretion in the collecting tubules, which results in a failure of urine acidification and results in metabolic acidosis, hypokalemia, and nephrocalcinosis. The acquired form of distal RTA is associated with tubulointerstitial involvement of immune-mediated disorders such as Sjogren's syndrome and systemic lupus erythematosus (SLE). Only a few case reports have indicated that distal RTA precedes SLE by months to years. We present a 39-year-old woman who had manifestations of distal RTA for 21 years before the development of overt symptoms of SLE.

A Case of Henoch-Schonlein Purpura in a Patient withRheumatoid Arthritis and Chronic Lymphocytic Thyroiditis / 대한류마티스학회지

Henoch-Schonlein purpura (HSP) is a small-vessel vasculitis involving skin, gut, and kidney associated with arthralgia, arthritis and characterized by immunoglobulin A (IgA)-dominant immune deposits in target organs. This vasculitis is common in children and rarely reported to be associated with other autoimmune disease such as rheumatoid arthritis, autoimmune thyroiditis. We report the case of 72 year-old man who developed adult-onset HSP and also had rheumatoid arthritis and chronic lymphocytic thyroiditis. This is the first report in South Korea.

A Case of Renal Crisis in a Korean Scleroderma Patient with Anti-RNA polymerase I and III Antibodies

Scleroderma (SSc) renal crisis has been reported to be associated with anti-RNA polymerase I and III (RNAP I/III) antibodies in Caucasians and the Japanese. However, no report is available for Korean SSc patients. Here, we describe the case of a 65-yr-old female SSc patient who developed renal crisis and whose serum contained anti-RNAP I/III antibodies. She was finally diagnosed as having diffuse cutaneous SSc based on skin thickening proximal to the elbows and knees. Sudden hypertension, oliguria, and pulmonary edema were features of her renal crisis. Despite the use of captopril and adequate blood pressure control, her renal function deteriorated. Subsequent renal biopsy findings showed severe fibrinoid necrosis with luminal obliteration in interlobar arteries and arterioles consistent with SSc renal crisis. Serum anti-RNAP I/III antibodies were detected by radioimmunoprecipitation. This is the first report of a renal crisis in a Korean SSc patient with RNAP I/III antibodies.

Primary Sjogren's syndrome presenting as acute transverse myelitis / 대한내과학회지

Sjogren's syndrome is one of chronic inflammatory autoimmune diseases involving exocrine gland. Involvement of central nerve system was rarely reported. We report a case of primary Sjogren's syndrome presenting as transverse myelitis in 37-year-old female who complained of tingling sense in both legs and voiding difficulty. Magnetic resonance imaging shows high signal intensity with cord swelling from C6 to T7 in T2 weighted image. The patient received intravenous methylprednisolone (1,000 mg/day) for 10 days followed by prednisolone and intravenous monthly cyclophosphamide with subsequent clinical improvement.

A Case of Femoral Compressive Neuropathy in AL Amyloidosis

We describe a case of a 54-yr-old AL amyloidosis patient who developed femoral compressive neuropathy due to iliopsoas pseudohypertrophy. The patient, who presented with end stage renal disease, was referred to our clinic because of lower extremity weakness and polyarthritis. Finally, he was diagnosed as having kappa-AL amyloidosis, complicated by femoral compressive neuropathy, hypertrophic amyloid myopathy, amyloid arthropathy, carpal tunnel syndrome, and end stage renal disease. Femoral compressive neuropathy has never been reported in association with amyloid induced iliopsoas hypertrophic myopathy. This report expands the clinical spectrum of AL amyloidosis.

Clinical Analysis of Relapsing Polychondritis: 16 Cases in Korea / 대한류마티스학회지

OBJECTIVE: To investigate the clinical characteristics of relapsing polychondritis (RP) in Korea. METHODS: We retrospectively reviewed 16 patients with RP who were diagnosed at Seoul National University Hospital and Gyeongsang National University Hospital in the period from July 1987 to August 2003. RESULTS: There were 9 males and 7 females, the mean age was 45.4+/-12.8 years. The frequency of clinical manifestation was as follows; auricular chondritis (81.3%), nasal chondritis (68.8%), ocular inflammation (56.3%), respiratory tract involvement (56.3%), arthritis (50%) and audio-vestibular damage (25%). Westergren erythrocyte sedimentation rate (ESR) was elevated in 53.3% and antinuclear antibody (ANA) was positive in 20% of study patients. Rheumatoid factor was negative in all cases. CONCLUSION: Clinical manifestations were comparable to the reports of Western countries. However, the frequency of arthritis was lower in Korean patients with RP than Western reports.

A Case of Hypertrophic Osteoarthropathy Associated with Epithelioid Hemangioendothelioma

Epithelioid hemangioendothelioma is a rare vascular tumor, which occurs in the lung, liver, bone, and soft tissue. Hypertrophic osteoarthropathy is a syndrome characterized by subperiosteal new bone formation, joint effusion and clubbing, and may be associated with cyanotic heart disease, chronic pulmonary disease, liver disease, and other miscellaneous diseases. The activation of endothelium and platelets has been suggested to be involved in the development of hypertrophic osteoarthropathy. We report a rare case of hypertrophic osteoarthropathy, which developed in association with hepatic epithelioid hemangioendothelioma with pulmonary metastasis. We also discuss the role of vascular endothelial growth factor in its pathogenesis.

A case of relapsing polychondritis with vasculitis mimicking Takayasu's arteritis / 대한내과학회지

Relapsing polychondritis is a rare autoimmune disease of unknown etiology characterized by recurrent inflammation of cartilaginous structures of the ears, nose, respiratory tract and joints. Relapsing polychondritis may affect proteoglycan-rich tissues, such as the eyes, aorta, heart, and skin. Several cardiovascular complications have been reported in patients with relapsing polychondritis. The most common findings of cardiovascular manifestations are aortic or mitral regurgitation and aortic aneurysm. Aortitis is a very rare complication of relapsing polychondritis. We report a case of 48-year-old man who had relapsing polychondritis with aortitis mimicking Takayasu's arteritis.

A Case of Primary Sjogren's Syndrome with Protein-losing Enteropathy / 대한류마티스학회지

Protein-losing enteropathy is a rare complication of autoimmune diseases. We report a case of primary Sjogren's syndrome with protein-losing enteropathy in 50-year-old female who complained of generalized edema. Protein-losing enteropathy of the small intestine was demonstrated by Tc-99m labeled albumin abdominal scintigraphy. Duodenal biopsies showed chronic inflammatory cell infiltration without lymphangiectasis or vasculitis. The patient received oral prednisolone (60 mg/day) for 4 weeks with subsequent clinical improvement. Primary Sjogren's syndrome or other autoimmune diseases should be considered in cases of protein-losing enteropathy.

Seizure Attacks in a Diffuse Systemic Sclerosis Patients / 대한류마티스학회지

Systemic sclerosis is a rare, autoimmune, connective tissue disorder which involves skin and other internal organs. However, central nervous system involvement including seizure, is a very rare event. This 29-year-old woman experienced repeated episodes of typical generalized tonic clonic seizure. T2 weighted and flare images of magnetic resonance image showed a mass-like high signal intensity lesion in the left temporal lobe. Atypical spikes and waves were present in a eclectroencephalogram. Her seizure episodes were controlled with anticonvulsants, corticosteroids and immunosuppresants. We report a rare case of systemic sclerosis which showed central nervous system involvement.