PURPOSE: A case of frosted branch angiitis in Kikuchi-Fujimoto disease is reported. CASE SUMMARY: A 33-year-old male complained of a sudden decrease in visual acuity that developed in both eyes 5 days prior. He suffered from a headache, chills, myalgia, and flank pain 1 week before. The initial best-corrected visual acuity (BCVA) was 0.1 in the right eye and 0.2 in the left eye. On slit lamp examination, no inflammatory finding was observed in the anterior chamber and vitreous body of both eyes. On fundus examination, a diffuse vascular sheathing-like frosted branch was found in the retinal vessels, and retinal hemorrhage was observed. Fluorescein angiography showed staining and leakage of dye along the vascular sheathing. Serological findings were negative, showing no evidence of an autoimmune disease or viral infection. Neck ultrasonography revealed non-tender left cervical lymph node enlargement >1 cm in diameter. Ultrasound-guided fine needle aspiration cytology showed findings compatible with Kikuchi-Fujimoto disease, including necrotic changes and pronounced karyorrhexis, plus histiocyte and lymphocyte infiltration without neutrophils. We started systemic steroid therapy. One month after treatment, the BCVA of both eyes improved to 1.0. CONCLUSIONS: In patients with frosted branch angiitis, systemic disease such as Kikuchi-Fujimoto disease should be considered.
Adult ; Anterior Chamber ; Autoimmune Diseases ; Biopsy, Fine-Needle ; Chills ; Flank Pain ; Fluorescein Angiography ; Headache ; Histiocytes ; Histiocytic Necrotizing Lymphadenitis ; Humans ; Lymph Nodes ; Lymphocytes ; Male ; Myalgia ; Neck ; Neutrophils ; Retinal Hemorrhage ; Retinal Vessels ; Slit Lamp ; Ultrasonography ; Vasculitis ; Visual Acuity ; Vitreous Body

Kawasaki disease (KD) is a systemic vasculitis that occurs predominantly in infants and young children. The etiology of KD is unknown and coronary heart disease is a major complication of KD. Acute scrotum is a rare complication of acute KD, and not as well recognized as other manifestations of the disease. We report a 2-month-old boy with acute scrotum in the acute phase of KD. He was treated with intravenous immunoglobulin (total 2 g/kg) and aspirin (50 mg/kg/day). The treatment was effective in resolving his fever and other clinical symptoms, but 2 days after starting treatment he experienced scrotal swelling. Scrotal ultrasound and transillumination were used in the diagnosis of acute scrotum. After 2 months, a follow-up testicular ultrasound revealed a remission of the acute scrotum. Subsequently, he has been followed up for KD.
Aspirin ; Child ; Coronary Disease ; Diagnosis ; Fever ; Follow-Up Studies ; Humans ; Immunoglobulins ; Infant* ; Male ; Mucocutaneous Lymph Node Syndrome* ; Scrotum* ; Systemic Vasculitis ; Testicular Hydrocele ; Transillumination ; Ultrasonography ; Urological Manifestations

Pulmonary artery sarcoma (PAS) is a rare and fatal disease that often mimics chronic thromboembolic pulmonary hypertension (CTEPH); therefore, diagnosis of PAS is often delayed. Herein, a healthy 74-year-old man was presented with a 4-month history of dyspnea. Chest computed tomography showed wall thickening and stenosis in the main pulmonary artery as well as in both proximal pulmonary arteries. In order to differentiate between unusual CTEPH, vasculitis, and PAS, we performed right heart catheterization and pulmonary angiography. The mean pulmonary arterial pressure was 21 mmHg, and there was severe pulmonary artery stenosis. Thrombi on the pulmonary arterial wall lesions were observed in intravascular ultrasound and optical coherence tomography. Furthermore, the patient had a history of deep vein thrombosis. Therefore, we diagnosed unusual CTEPH. After 6 months of rivaroxaban anticoagulation therapy, a chest X-ray revealed a left lower lobe lung mass, and a positron emission tomography later showed hypermetabolic lesions in the main pulmonary artery wall, in both pulmonary arteries walls, in the lung parenchyma, and in the bones. A biopsy of the right proximal humerus lesion revealed undifferentiated intimal sarcoma. Pulmonary sarcoma is rare, but should be considered when differentially diagnosing main pulmonary artery wall thickening and stenosis. A positron emission tomography may aid in this diagnosis.
Aged ; Angiography ; Arterial Pressure ; Biopsy ; Cardiac Catheterization ; Cardiac Catheters ; Constriction, Pathologic* ; Diagnosis ; Dyspnea ; Fluorodeoxyglucose F18* ; Humans ; Humerus ; Hypertension, Pulmonary ; Lung ; Positron-Emission Tomography ; Positron-Emission Tomography and Computed Tomography* ; Pulmonary Artery* ; Rivaroxaban ; Sarcoma* ; Thorax ; Tomography, Optical Coherence ; Ultrasonography ; Vasculitis ; Venous Thrombosis

Pulmonary artery sarcoma (PAS) is a rare and fatal disease that often mimics chronic thromboembolic pulmonary hypertension (CTEPH); therefore, diagnosis of PAS is often delayed. Herein, a healthy 74-year-old man was presented with a 4-month history of dyspnea. Chest computed tomography showed wall thickening and stenosis in the main pulmonary artery as well as in both proximal pulmonary arteries. In order to differentiate between unusual CTEPH, vasculitis, and PAS, we performed right heart catheterization and pulmonary angiography. The mean pulmonary arterial pressure was 21 mmHg, and there was severe pulmonary artery stenosis. Thrombi on the pulmonary arterial wall lesions were observed in intravascular ultrasound and optical coherence tomography. Furthermore, the patient had a history of deep vein thrombosis. Therefore, we diagnosed unusual CTEPH. After 6 months of rivaroxaban anticoagulation therapy, a chest X-ray revealed a left lower lobe lung mass, and a positron emission tomography later showed hypermetabolic lesions in the main pulmonary artery wall, in both pulmonary arteries walls, in the lung parenchyma, and in the bones. A biopsy of the right proximal humerus lesion revealed undifferentiated intimal sarcoma. Pulmonary sarcoma is rare, but should be considered when differentially diagnosing main pulmonary artery wall thickening and stenosis. A positron emission tomography may aid in this diagnosis.
Aged ; Angiography ; Arterial Pressure ; Biopsy ; Cardiac Catheterization ; Cardiac Catheters ; Constriction, Pathologic ; Diagnosis ; Dyspnea ; Fluorodeoxyglucose F18 ; Humans ; Humerus ; Hypertension, Pulmonary ; Lung ; Positron-Emission Tomography ; Positron-Emission Tomography and Computed Tomography ; Pulmonary Artery ; Rivaroxaban ; Sarcoma ; Thorax ; Tomography, Optical Coherence ; Ultrasonography ; Vasculitis ; Venous Thrombosis

Acute cholecystitis as a complication of malarial disease is a rare condition, especially with Plasmodium vivax infection. A 62 year-old-female was admitted via emergency room (ER) due to high fever (40.3degrees C) and epigastric pain. Initial abdominal ultrasound and computed tomography (CT) scan showed edematous gallbladder with stone, which suggested acute calculous cholecystitis. Emergency percutaneous transhepatic gallbladder drainage (PTGBD) was done with systemic antibiotic therapy. The clinical course, however, unusually worsened with hypotension and intensive care unit (ICU) management was done. Four days after admission multi-focal splenic infarction was developed and Plasmodium vivax infection was diagnosed afterward. The clinical symptoms and laboratory results, including fever and epigastric pain, improved dramatically after anti-malarial treatment and cholecystectomy was done. The resected gallbladder (GB) specimen shows vasculitis pattern with capillary red blood cell (RBC) engorgement, which suggests the cause of cholecystitis was due to Plasmodium vivax rather than GB stone.
Capillaries ; Cholecystectomy ; Cholecystitis ; Cholecystitis, Acute* ; Drainage ; Emergencies ; Emergency Service, Hospital ; Erythrocytes ; Fever ; Gallbladder ; Hypotension ; Intensive Care Units ; Plasmodium vivax* ; Plasmodium* ; Splenic Infarction ; Ultrasonography ; Vasculitis

Vessel wall imaging can depict the morphologies of atherosclerotic plaques, arterial walls, and surrounding structures in the intracranial and cervical carotid arteries beyond the simple luminal changes that can be observed with traditional luminal evaluation. Differentiating vulnerable from stable plaques and characterizing atherosclerotic plaques are vital parts of the early diagnosis, prevention, and treatment of stroke and the neurological adverse effects of atherosclerosis. Various techniques for vessel wall imaging have been developed and introduced to differentiate and analyze atherosclerotic plaques in the cervical carotid artery. High-resolution magnetic resonance imaging (HR-MRI) is the most important and popular vessel wall imaging technique for directly evaluating the vascular wall and intracranial artery disease. Intracranial artery atherosclerosis, dissection, moyamoya disease, vasculitis, and reversible cerebral vasoconstriction syndrome can also be diagnosed and differentiated by using HR-MRI. Here, we review the radiologic features of intracranial artery disease and cervical carotid artery atherosclerosis on HR-MRI and various other vessel wall imaging techniques (e.g., ultrasound, computed tomography, magnetic resonance, and positron emission tomography-computed tomography).
Arteries ; Atherosclerosis ; Carotid Arteries* ; Early Diagnosis ; Electrons ; Magnetic Resonance Imaging ; Moyamoya Disease ; Phenobarbital ; Plaque, Atherosclerotic ; Stroke ; Ultrasonography ; Vasculitis ; Vasoconstriction

The use of musculoskeletal ultrasound in rheumatology clinical practice has rapidly increased over the past decade. Ultrasound has enabled rheumatologists to diagnose, prognosticate and monitor disease outcome. Although international standardization remains a concern still, the use of ultrasound in rheumatology is expected to grow further as costs fall and the opportunity to train in the technique improves. We present a review of value of ultrasound, focusing on major applications of ultrasound in rheumatologic diseases.
Humans ; Magnetic Resonance Imaging ; Musculoskeletal System/ultrasonography ; Osteoarthritis/ultrasonography ; Rheumatic Diseases/*ultrasonography ; Sjogren's Syndrome/ultrasonography ; Spondylarthropathies/ultrasonography ; Synovitis/ultrasonography ; Tendinopathy/ultrasonography ; Vasculitis/ultrasonography

BACKGROUND/AIMS: Behcet's disease (BD) is a systemic disorder associated with a characteristic vasculitis that can involve both veins and arteries of all sizes. Endothelial activation or injury is a characteristic feature of BD. Endothelial dysfunction is widely regarded as being the initial lesion in the development of atherosclerosis. The carotid artery intima-media thickness (IMT) is a widely accepted marker of subclinical atherosclerosis. We aimed to determine the carotid IMT in BD patients with using high-resolution B-mode Doppler ultrasonography. METHODS: We studied 40 patients (24 males, mean age: 39.1+/-8.5 years) who were diagnosed by the international diagnostic criteria of Behcet's disease and 20 healthy controls (13 males, mean age: 40.2+/-5.1 years), and the two groups were matched by age and gender. No subject in either group had a history of atherosclerosis or its complications. The clinical data, including the age of onset, the duration of disease, a history of medication, the activity score and the laboratory data were analyzed. RESULTS: The carotid IMT in the BD group was significantly higher than that in the control group (0.71+/-0.22 mm vs. 0.59+/-0.09 mm, respectively, p<0.01). Cardiac and major vessel involvements were not identified in the BD group. However, minor vascular involvements were documented in 2 patients with deep vein thrombosis, in 4 patients with superficial thrombophlebitis and in 2 patients with pseudoaneurysm. The carotid IMT in the patients with posterior uveitis or retinal vasculitis was higher than that of the patients without these findings (0.85+/-0.21 mm vs. 0.64+/-0.10 mm, respectively, p=0.007), but there was no difference of the IMT according to minor vascular involvement. CONCLUSIONS: Despite that there was no significant cardiovascular involvement in the BD patients, the carotid IMT was significantly higher in the BD patients as compared with the healthy controls.
Adult ; Behcet Syndrome/complications/diagnosis/*pathology ; Carotid Arteries/*pathology/ultrasonography ; Case-Control Studies ; Endothelium, Vascular/pathology ; Female ; Humans ; Male ; Prognosis ; Prospective Studies ; Risk Factors ; Tunica Intima/*pathology/ultrasonography ; Tunica Media/*pathology/ultrasonography ; Ultrasonography, Doppler ; Vasculitis/pathology

Glomerulonephritis and pulmonary hemorrhage are features of Goodpasture's syndrome. Goodpasture's syndrome accompanied with central nervous system (CNS) vasculitis is extremely rare. Herein, we report a rare case of CNS vasculitis associated with Goodpasture's syndrome in a 34-year-old man, who presented with a seizure and sudden onset of right sided weakness. He also had recurrent hemoptysis of one month's duration. Goodpasture's syndrome is histologically diagnosed by intense linear deposits of IgG along the glomerular basement membrane in both renal and lung tissues.
Adult ; Anti-Glomerular Basement Membrane Disease/complications/*diagnosis/therapy ; Anti-Inflammatory Agents/administration & dosage ; Brain/*pathology ; Contrast Media/administration & dosage ; Diagnosis, Differential ; Fluorescent Antibody Technique ; Hemoptysis/etiology ; Humans ; Image Enhancement/methods ; Immunoglobulin G/immunology ; Kidney/ultrasonography ; Lung/pathology/*radiography ; Magnetic Resonance Imaging ; Male ; Methylprednisolone/administration & dosage ; Muscle Weakness/etiology ; Plasmapheresis ; Rare Diseases ; Seizures/etiology ; Tomography, X-Ray Computed ; Vasculitis, Central Nervous System/*diagnosis/etiology/therapy

BACKGROUND: Acute renal infarction is an uncommon disease which is often delyed or missed due to its rarity and unspecific clinical presentation. METHODS: In order to evaluate the clinical features and to elucidate diagnostic or therapeutic options, we analyzed the medical records of 23 patients who were admitted to Pusan National University Hospital from January, 1995 to July, 2004 and diagnosed as renal infarction. RESULTS: The mean age of the patients was 57.3 18.4 years and male to female ratio was 0.91:1. Underlying diseases were cardiovascular disease (n=18), tumor embolism (n=1), vasculitis (n=1), post kidney transplantation thrombosis (n=1), and trauma(n=1). One patient did not have any underlying disease. Initial symptoms were abdominal or flank pain (61%), fever (35%), anorexia (35%), nausea (26%), vomiting (17%), gross hematuria (9%), and oliguria (4%). On physical examination, costovertebral angle tenderness (43%), abdominal tenderness (9%), and hypertension (35%) were noted. Initial abnormal laboratory findings were elevated serum level of LDH (100%), AST (87%), ALT (83%), CK (22%), and creatinine (>1.4 mg/dL, 17%). Imaging diagnosis of renal infarction included renal angiography, isotope renal scan, computed tomography or ultrasonography. CT was done in 17/23 cases and useful in diagnosis of renal infarction. Nine patients were treated with heparin or warfarin. Thrombolysis was done in 3 patients. Others were treated conservatively. CONCLUSIONS: In a patients with an increased risk of thromboembolism, flank or abdominal pain, microscopic hematuria, and an elevated serum LDH are strongly supportive of diagnosis of renal infarction. Under such circumstances, enhanced CT is essential for the early diagnosis of renal infarction.
Abdominal Pain ; Angiography ; Anorexia ; Busan ; Cardiovascular Diseases ; Creatinine ; Diagnosis ; Early Diagnosis ; Female ; Fever ; Flank Pain ; Hematuria ; Heparin ; Humans ; Hypertension ; Infarction* ; Kidney Transplantation ; Male ; Medical Records ; Nausea ; Neoplastic Cells, Circulating ; Oliguria ; Physical Examination ; Renal Insufficiency ; Thromboembolism ; Thrombosis ; Ultrasonography ; Vasculitis ; Vomiting ; Warfarin