Tumor-like Presentation of Cerebral Vasculitis in a Patient With Systemic Lupus Erythematosus: A Biopsy-confirmed Case

Central nervous system (CNS) manifestations of systemic lupus erythematosus (SLE) are diverse and often difficult to distinguish from SLE-unrelated events. CNS vasculitis is a rare manifestation, which is seen in less than 10% of post-mortem studies, and lesions with multifocal cerebral cortical microinfarcts associated with small-vessel vasculitis are the predominant feature. However, CNS vasculitis presenting as a tumor-like mass lesion in SLE has rarely been reported. Herein, we report a case of cerebral vasculitis mimicking a brain tumor in a 39-year-old female with SLE. A biopsy of the brain mass revealed fibrinoid necrosis and leukocytoclastic vasculitis. The neurological deficits and systemic symptoms improved after treatment with corticosteroids and immunosuppressive agents. To the best of our knowledge, there are no reports of biopsy-proven cerebral vasculitis presenting as a brain mass in patients with SLE in Korea.

Case of Renal Microaneurysms and Multiple Renal Infarctions in a Patient with Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is a chronic inflammatory, heterogeneous autoimmune disease characterized by autoantibody production and the potential involvement of almost every organ system. Although vasculitis usually confined to small vessels is a fairly common feature of SLE, ischemic vasculitis with an aneurysm is an uncommon feature. In particular, renal arterial microaneurysms and multiple renal infarctions are very rarely reported in patients with SLE. Furthermore, to the best of the authors' knowledge, there is no report on renal arterial microaneurysms associated with SLE in Korea. Here, this paper presents a case of renal microaneurysms and multiple renal infarctions in a 41-year-old woman with SLE.

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.

Focal Eosinophilic Myositis Associated with Behçet's Disease

Behçet's disease (BD) is a systemic vasculitis commonly accompanied by recurrent mucosal ulceration and other systemic manifestations, but rarely by myositis. Focal eosinophilic myositis is the most limited idiopathic eosinophilic myopathy characterized by peripheral blood eosinophilia and/or eosinophilic muscle infiltration. Clinical manifestations include myalgia, muscle weakness, and cutaneous lesions, such as subcutaneous induration and erythema. Given that BD can mimic deep vein thrombosis or pseudotumor, muscle biopsy should be performed to enhance the accuracy of diagnosis. Microscopic examination reveals extensive infiltration of eosinophils and mononuclear cells into muscle, myofiber necrosis, and regeneration. To the best of our knowledge, there have not been any published reports on MEDLINE regarding focal eosinophilic myositis associated with BD. Here, we presented a case of focal eosinophilic myositis associated with intestinal BD in a 23-year-old man who suffered from a large ulcer in the terminal ileum.

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.

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 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.

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.

Successful treatment of refractory livedoid vasculitis with rituximab and cyclophosphamide / 대한내과학회지

Livedoid vasculitis (LV) is a chronic recurrent cutaneous disease that is characterized by a netlike vascular pattern of purpuric lesions that progress to ulcers and atrophic white scars. The etiology of LV is unknown. Various therapies have been used for LV with inconsistent results, including antiplatelet agents, fibrinolytics, anticoagulants, prostaglandin E1, and immunosuppressive agents, such as systemic corticosteroids, azathioprine, methotrexate, cyclosporine, and mycophenolate mofetil. We present a patient with idiopathic LV refractory to immunosuppressive therapy who had a good response to treatment with rituximab and cyclophosphamide.