Behcet's disease is recognized as a multisystemic disease with various organ involvement including skin, mucous membrane, eyes, joints, veins, arteries, gastrointestinal tract, meninges, and brain. In this review, Behcet's disease has been approached from two rheumatologic perspectives--as an intermittent and periodic arthritic syndrome and as a vasculitic syndrome. Association with seronegative spondyloarthropathy and other autoimmune diseases will also be discussed.
Arthritis/etiology* ; Autoimmune Diseases/etiology* ; Behcet's Syndrome/complications* ; Human ; Vasculitis/etiology*

Arthritis, Rheumatoid/*complications ; Heart Diseases/etiology ; Lung Diseases/etiology ; Vasculitis/etiology

Central nervous system is often involved by herpes zoster but it is very rarely seen that contralateral hemiparesis or hemiplegia developed after herpes zoster ophthalmicus. We report a case of herpes zoster ophthalmicus followed by the delayed contralateral hemiparesis. A 33-year-old man developed acute cerebral infarction and resultant right hemiparesis 44 days after herpes zoster ophthalmicus in the left side. Brain CT disclosed hypodense area in the left basal ganglia. Cerebral angiography revealed segmental narrowing of M1 portion of the right middle cerebral artery.
Adult ; Cerebral Angiography ; Cerebral Arteries/pathology ; Cerebral Infarction/*etiology/radiography ; Dominance, Cerebral ; Hemiplegia/*etiology ; Herpes Zoster Ophthalmicus/*complications ; Humans ; Male ; Tomography, X-Ray Computed ; Vasculitis/etiology

Antibodies, Antineutrophil Cytoplasmic ; blood ; Child ; Female ; Hematuria ; etiology ; Humans ; Kidney ; pathology ; physiopathology ; Kidney Function Tests ; Proteinuria ; etiology ; Renal Insufficiency ; etiology ; Vasculitis ; blood ; complications ; pathology

The relationship between immune vasculitis and atherosclerosis was studied. The experimental model of weanling rabbits for immune vasculitis was reproduced by intravenous injection of 10% bovine serum albumin. There were 6 groups: group A, 25 weanling rabbits with immune vasculitis subject to coronary arteriography; group B, 10 normal mature rabbits subject to coronary arteriography; group C, 10 weanling rabbits subject to coronary arteriography; group D, 8 weanling rabbits with vasculitis and cholesterol diet; group E, 8 weanling rabbits receiving single cholesterol diet; group F: 8 weanling rabbits receiving basic diet. Four weeks later, coronary arteriography was performed in groups A, B and C. The rabbits in groups D, E and F were sacrificed for the study of pathological changes in the coronary artery after 12 weeks. The results showed that the dilatation of coronary artery occurred in 6 rabbits of group A, but in groups B and C, no dilatation of coronary artery appeared. In comparison with group E, more severe atherosclerosis occurred in group D, showing the thickened plaque, fibrous sclerosis and atherosclerotic lesion. Percentage of plaques covering aortic intima, incidence of atherosclerosis of small coronary arteries and degree of stenosis of coronary arteries were significantly higher in group D than in group E (P < 0.01). No atherosclerosis changes were found in group F. It was concluded that in the acute phase, the serum immune vasculitis can induce the dilatation of coronary artery of some weanling rabbits, and aggravate the formation of atherosclerosis in rabbits fed with cholesterol diet. Immune vasculitis is a new risk factor of atherosclerosis and ischemic heart disease.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/chemically induced ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/*complications ; Atherosclerosis/*etiology ; Cholesterol, Dietary/*administration & dosage ; Myocardial Ischemia/etiology ; Random Allocation ; Risk Factors ; Serum Albumin, Bovine

Bacterial Infections ; complications ; Diagnosis, Differential ; Factitious Disorders ; complications ; Fever of Unknown Origin ; diagnosis ; etiology ; psychology ; Humans ; Vasculitis ; complications ; Virus Diseases ; complications

Immunoglobulin A (IgA) vasculitis is the most common leukocytoclastic small-vessel vasculitis in children and mainly involves the small vessels in the skin, joints, digestive tract, and kidneys. Its pathogenesis is still unclear. Currently, it is believed that environmental factors can cause autoimmune dysfunction and lead to the deposition of IgA-containing immune complexes on the wall of arterioles on the basis of genetic factors. This article reviews the research advances in the role of immune factors in the pathogenesis of IgA vasculitis.
Autoantibodies ; analysis ; Complement System Proteins ; physiology ; Cytokines ; physiology ; Glycosylation ; Humans ; Immunoglobulin A ; analysis ; Immunoglobulin E ; metabolism ; Vasculitis ; etiology ; immunology

Atherosclerosis ; blood ; drug therapy ; etiology ; C-Reactive Protein ; analysis ; Humans ; Lipoproteins, LDL ; blood ; PPAR gamma ; agonists ; Vasculitis ; complications

Pseudotumor cerebri is an uncommon manifestation of neuropsychiatric systemic lupus erythematosus (SLE), and is characterized by an elevated intracranial pressure, papilledema with occasional abducens nerve paresis, absence of a space-occupying Iesion or ventricular enlargement, and normal cerebrospinal fluid chemical and hematological constituents. Pseudotumor cerebri has been reported in a few sporadic cases in patients with systemic lupus erythematosus. However, the recurrent pseudotumor cerebri in patients with systemic lupus erythematosus which has been rarely reported, has not been reported in Korea. We experienced a 30-yr-old female patient with SLE who was presented with second attack of severe intractable headache. She was diagnosed pseudotumor cerebri twice and successfully treated with corticosteroid. Headache is the common symptom in patients with neuropsychiatric SLE and attributable to various causes. We suggest that it is important to define the cause of headache in patients with SLE and pseudotumor cerebri should be included in the spectrum of clinical manifestations during the course of SLE as a cause of headache.
Adult ; Case Report ; Female ; Headache/etiology/pathology ; Human ; Lupus Vasculitis, Central Nervous System/*complications/pathology ; Magnetic Resonance Imaging ; Pseudotumor Cerebri/*etiology/pathology ; Recurrence

OBJECTIVETo study the roles of platelet (PLT) and its regulating factors, megakaryocyte, thrombopoietin (TPO) and transforming growth factor beta1 (TGF-beta1), in immune vasculitis in young rabbits.

METHODSAn experimental model of Kawasaki disease (KD) of weanling rabbits was reproduced by bovine serum. PLT count, total number and differentiating count of megakaryocyte, and serum TPO and TGF-beta1 levels were measured 0, 4, 8, 12, 16, 20, 24 and 28 days after KD induction. Pathological analysis of coronary artery, liver, spleen, kidney and brain was performed 17 and 28 days after KD induction.

RESULTSIn the KD group, PLT count, the total number of megakaryocyte, and the middle board megakaryocyte percentage increased 12, 16, 20, 24 and 28 days; serum TPO level increased 8, 12, 16, 20, 24 and 28 days; serum TGF-beta1 level increased 16, 20, 24 and 28 days after KD induction compared with those in the normal control group (p<0.05). The pathological examinations of coronary artery, liver, spleen, kidney and brain showed severe inflammatory injuries of tiny arteries and small/medium-sized arteries 17 and 28 days after KD induction, respectively in the KD group. The aortas were showed as mild inflammatory injuries.

CONCLUSIONSPLT, megakaryocyte, TPO and TGF-beta1 participate in the pathogenesis of KD, and they may play an important role in the injuries of immune vasculitis. This suggests that they may serve as markers for the assessment of severity in KD.

Animals ; Blood Platelets ; physiology ; Disease Models, Animal ; Humans ; Megakaryocytes ; physiology ; Mucocutaneous Lymph Node Syndrome ; etiology ; Rabbits ; Thrombopoietin ; physiology ; Transforming Growth Factor beta1 ; physiology ; Vasculitis ; etiology ; immunology ; pathology