In recent years the survival of patients with systemic lupus erythematosus has increased markedly. Consequently, long-term complications, such as osteoporosis, are currently of paramount importance. SLE is known to increase the risk of bone fractures, and numerous studies have found that SLE patients have osteoporosis. Of the various risk factors associated with osteoporosis in SLE, disease duration, the use of corticosteroids and chronic disease-related damage are consistently reported, with differences between studies probably due to the different populations studied. The role of chronic inflammation in osteoporosis is also important. On the other hand, little attention has been paid to osteoporotic fractures, especially of the vertebra, which are associated with reduced quality of life, increased mortality rates and increased risk of new vertebral and non-vertebral fractures in the general population.

Bechet disease (BD) is an inflammatory, multi systemic disease with spontaneous remissions and relapses similar to various autoimmune diseases. BD leads to organ damage, including the eyes, skin, joints, etc., which produce various clinical manifestations. The central histopathologic characteristic is systemic vasculitis with perivascular inflammatory infiltrates. The etiopathogenesis is unknown, although immunological abnormalities, possibly induced by susceptible microbiological pathogens, have been postulated.

The aim of the current study was to analyze the role of traditional and systemic lupus erythematosus (SLE)-related risk factors in the development of vertebral fractures. A cross-sectional study was performed in women with SLE attending a single center. A vertebral fracture was defined as a reduction of at least 20% of vertebral body height. Two hundred ten patients were studied, with median age of 43 years and median disease duration of 72 months. Osteopenia was present in 50.3% of patients and osteoporosis in 17.4%. At least one vertebral fracture was detected in 26.1%. Patients with vertebral fractures had a higher mean age (50 +/- 14 vs. 41 +/- 13.2 years, p = 0.001), disease damage (57.1% vs. 34.4%, p = 0.001), lower bone mineral density (BMD) at the total hip (0.902 +/- 0.160 vs. 982 +/- 0.137 g/cm(2), p = 0.002), and postmenopausal status (61.9% vs. 45.3%, p = 0.048). Stepwise logistic regression analysis revealed that only age (p = 0.001) and low BMD at the total hip (p = 0.007) remained as significant factors for the presence of vertebral fracture. The high prevalence of vertebral fractures in the relatively young population implies that more attention must be paid to detect and treat vertebral fractures.

Systemic lupus erythematosus (SLE) is a chronic, occasionally life threatening, multisystem disorder. Patients suffer from a wide group of symptoms and have a variable prognosis that depends of the severity and type of organ involvement. The clinical manifestations include fever, skin lesions, arthritis, neurologic, renal, cardiac, and pulmonary disease. The pathogenesis of this serious multisystem autoimmune disease is based on polyclonal B cell immunity, which involves connective tissue and blood vessels. The novel biologic therapies have raised hope for more effective and safer treatment for SLE. Although definitive studies are still under development, the impressive preliminary results of therapies specifically targeting B cells and the signaling pathways involved in B-T-cell interactions suggest that the depletion of memory cells accounts, at least in part, for the clinical efficacy of rituximab therapy in patients whose disease is resistant to other immunosuppressive therapies. However these findings, although provocative, require further investigation in larger cohorts.

Raynaud's phenomenon is an episodic vasospasm of the peripheral arteries, causing pallor followed by cyanosis and redness with pain and sometimes paraesthesia, and, rarely, ulceration of the fingers and toes. Primary or idiopathic Raynaud's phenomenon (Raynaud's disease) occurs without an underlying disease. Secondary Raynaud's phenomenon (Raynaud's syndrome) occurs in association with an underlying disease. Initially conservative, non-pharmacologic approach is important for these patients, although pharmacologic therapy may ultimately be necessary. Advances in vascular physiology have showed the role of the endothelium as well as endothelium-independent mechanisms in the altered vasoregulation of Raynaud's phenomenon. This has opened promising therapeutic avenues, and it is likely that therapies targeted towards specific pathophysiologic steps become available in the near future.

The aim of this study was to determine the prevalence and risk factors for low bone mineral density (BMD) in women with systemic lupus erythematosus (SLE). A cross-sectional study was conducted among 100 pre-menopausal patients with SLE. Patients were evaluated using a questionnaire about the following variables: age, disease duration, disease activity, chronic disease damage, cumulative corticosteroid dose, and history of fracture. Lumbar spine and hip measurements of BMD were performed by dual absorptiometry. Univariate and multivariate statistical analyses were used to assess the relationship between risk factors and BMD. The mean age was 32.8 +/- 8.7 years, and the median duration of SLE was 73.2 +/- 65 months. The mean cumulative corticosteroid dose was 20.0 +/- 21.3 g. The mean BMD was 1.09 +/-.18 g/cm(2) in the lumbar spine and 1.0 +/-.14 g/cm(2) in the hip. Osteopenia was present in 40% of patients and osteoporosis in 5%. In the multiple regression analysis, low BMD in the lumbar spine was associated with chronic disease damage and low body mass index (BMI). Low BMD in the hip was associated with cumulative corticosteroid dose and low BMI. Chronic disease damage, low BMI, and cumulative corticosteroid dose are risks factors for low BMD in pre-menopausal SLE patients. Osteopenia was found in 40% of patients, while osteoporosis was found in only 5%.

BACKGROUND Data on pediatric antiphospholipid syndrome (APS) are very sparse. OBJECTIVES To describe the main clinical characteristics, laboratory data and complications of pediatric APS patients, and to analyze the differences between primary APS and APS associated with systemic lupus erythematosus (SLE). METHODS We retrospectively reviewed clinical and laboratory data of 32 children at the Federico Gomez children's hospital in Mexico. Nineteen patients had SLE, 12 (37.5%) had primary APS and 1 (3%) had immune thrombocytopenic purpura. We collected information on sociodemographic variables, vaccinations, age at onset, and family history of rheumatic disease, hematological disorders, skin disorders and non-thrombotic neurological disorders. Immunological features included immunoglobulin (Ig) G and IgM anticardiolipin antibodies, IgG and IgM anti-beta2 glycoprotein I antibodies, lupus anticoagulant, and anti-dsDNA and antinuclear antibodies. RESULTS The patients included 24 females and 8 males. The most common thrombotic events were small vessel thrombosis (44%), venous thrombosis (28%) mainly deep venous thrombosis (DVT) in lower extremities, and arterial thrombosis (25%). The most common clinical non-thrombotic manifestations were hematological (53%) and neurological disorders (22%). There were no significant differences between groups with regard to the site of thrombosis, nonthrombotic clinical manifestations or laboratory features. CONCLUSIONS There were some important differences between the clinical manifestations of APS in children compared with adults, but we found no significant differences between patients with primary and APS associated with SLE. Larger studies in Latin American APS children are necessary to determine whether there are differences between ethnic groups.

BACKGROUND The prevalence of vertebral fractures in systemic lupus erythematosus (SLE) ranges between 20% and 21.4%, and patients with these fractures have impaired walking and activities of daily living. Moreover, clinical and radiological vertebral fractures have been associated with increased mortality. OBJECTIVES To compare the quality of life of patients with SLE with and without vertebral fractures. METHODS The study group comprised 140 women with SLE undergoing screening for vertebral fractures using a standardized method. SLE disease activity and organ damage were measured by the Mexican Systemic Lupus Erythematosus Disease Activity Index (MEX-SLEDAI) and the Systemic International Collaborating Clinics/American College of Rheumatology damage index (SLICC), respectively. The QUALEFFO and Center for Epidemiologic Studies Depression Scale were used to measure health-related quality of life and depression, respectively. RESULTS The median age of the 140 patients was 43 years (range 18-76); disease duration was 72 months (range 6-432); 49.7% were menopausal. Thirty-four patients (24.8%) had vertebral fractures (> or = 1), mostly in the thoracic spine. Patients with vertebral fractures had a higher mean age (49.5 +/- 13.4 vs. 41 - 13.2 years, P= 0.001) and disease damage (57.1% vs. 34.4%, P = 0.001). The global QUALEFFO score was not different between the vertebral fractures group and the non-vertebral group. The only significant difference in the QUALEFFO items was in physical function (P = 0.04). A significant correlation was found between the severity of vertebral fractures and the QUALEFFO pain (r = 0.27, P = 0.001) and physical function (r = 0.37, P = 0.02) scores. The number of vertebral fractures correlated only with physical function (r = 0.01). CONCLUSIONS The HRQOL of women with SLE is low, regardless of whether they have vertebral fractures or not, but patients with vertebral fractures have worse physical function compared to those without. Strategies to improve the HRQOL of patients with SLE with or without vertebral fractures are necessary.

Vitiligo is a common depigmenting disorder which may have devastating psychological and social consequences and is characterized by the presence of circumscribed white macules in the skin due to the destruction of melanocytes in the epidermis. Various hypotheses have been proposed to explain the pathomechanisms involved in this disease, and studies have shown the participation of autoimmune processes in the pathogenesis of vitiligo. Cellular and humoral immunities have been implicated in the development of vitiligo and their role continues to be investigated. Peripheral blood and skin biopsies of patients with vitiligo show that T-cells, mononuclear cells, various pro-inflammatory cytokines, and auto-antibodies can damage melanocytes. Further research is required to determine whether autoimmunity is the main mechanism of vitiligo or only a consequence.

Currently, the origin of autoimmune diseases is considered to be multifactorial. Genetic predisposition, immune system malfunction or even backfire, hormonal regulation, and environmental factors, i.e. infections, all play important roles in the pathogenesis of autoimmune diseases such as the antiphospholipid syndrome (APS). New drugs and strategies aimed at preventing infections could further improve the outcome of APS and other autoimmune diseases.

Susac syndrome was named after J.O. Susac who first described the syndrome in 1979. It is characterized by the clinical triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. It mainly occurs in young women. This underdiagnosed disease needs to be considered in the differential diagnosis of a broad variety of disorders. In Susac syndrome, autoimmune processes leading to damage and inflammation-related occlusion of the microvessels in brain, retina, and inner ear are thought to play a causal role. The diagnosis is based primarily on the clinical presentation, the documentation of branch retinal artery occlusion by fluorescence angiography, and characteristic findings on cerebral MRI, that help in distinguishing Susac syndrome from other inflammatory entities, like multiple sclerosis. Antiendothelial cell antibodies could be detected in some patients. Patients are successfully treated with immunosuppression, however, the best regimen still needs to be defined. As a result of the rarity of the disease, controlled therapeutic trials are missing so far. In this review, we want to demonstrate the clinical features, natural history, treatment, and clinical course of Susac syndrome, illustrated by a typical case history.

We report the first case of Susac syndrome in Koreans, in a 23-yr-old female patient who presented with sudden visual loss and associated neurological symptoms. Ophthalmic examination and fluorescein angiography showed multiple areas of branch retinal artery occlusion, which tended to recur in both eyes. Magnetic resonance imaging showed dot-like, diffusion-restricted lesions in the corpus callosum and left fornix, and audiometry showed low-frequency sensory hearing loss, compatible with Susac syndrome. She received immunosuppressive therapy with oral steroid and azathioprine. Three months later all the symptoms disappeared but obstructive vasculitis have been relapsing. This patient demonstrated the entire clinical triad of Susac syndrome, which tends to occur in young females. Although this disorder has rarely been reported in Asian populations, a high index of suspicion is warranted for early diagnosis and timely treatment.

OBJECTIVE Susac syndrome comprises a triad of vestibulocochlear dysfunction, retinopathy and multifocal encephalopathy, which is characterised pathophysiologically by microangiopathy of the ear, retina and brain. Diagnosis is confirmed by magnetic resonance imaging of the brain and ophthalmological examination, which reveals branch retinal artery occlusion. Hearing loss persists in 90 per cent of patients. We present a case of successful hearing rehabilitation by cochlear implantation in a young woman with this syndrome. CLINICAL PRESENTATION A 36-year-old woman presented with neurological symptoms suggestive of encephalitis. She subsequently developed vestibulocochlear symptoms. The diagnosis was confirmed upon magnetic resonance imaging and fluorescein angiography, which showed multiple peripheral retinal arterial occlusions. Hearing loss was fluctuant but gradually progressive over nine months, to bilateral profound sensorineural hearing loss. INTERVENTION A left cochlear implant was placed, with a good outcome. CONCLUSION In this Susac syndrome patient, the outcome of cochlear implantation was encouraging, notwithstanding the possible involvement of retrocochlear pathways.

Susac's syndrome (SS) is a rare microangiopathy affecting the precapillary arterioles of the brain, retina and inner ear, presumably resulting from an autoimmune endotheliopathy. We report the first case of SS with histologically proven skin involvement, in a 24-year-old male who presented a subacute encephalopathy, branch retinal artery occlusions and bilateral hearing loss, two weeks after the onset of a livedo racemosa of the flanks and feet. Skin biopsies revealed a thrombus in several dermal arterioles, endothelial cells swelling and a mild perivascular lymphocytic infiltrate, which correspond to the same histological findings as previously observed in brain but also in muscle biopsies of patients with SS. A complete recovery was achieved in 4 months with corticosteroids. Follow-up MRI showed centro-callosal "holes". Skin involvement in SS has pathological plausibility since serum antibodies directly binding to central nervous system but also to generic endothelium cells have been reported. Our report supports that SS is a systemic disease that could affect other organs in addition to the brain, retina and inner ear. We suggest careful skin examination should be considered in patients with a suspicion of SS.

Susac's Syndrome (SS) consists of the clinical triad of encephalopathy, branch retinal artery occlusion (BRAO), and hearing loss (HL). It is an autoimmune endotheliopathy affecting the precapillary arterioles of the brain, retina, and inner ear (cochlea and semicircular canals). The age range extends from 7 to 72 years, but young women (20-40) are most vulnerable. Headache routinely accompanies the encephalopathy and may be constant (best explained by leptomeningeal involvement), migrainous, or both. Multifocal neurological manifestations--particularly bilateral long-tract signs--commonly accompany the encephalopathy, which is laden with psychiatric features, confusion, memory loss and other cognitive changes. Left untreated, dementia can ensue. SS has an unexplained proclivity for attacking the central corpus callosum. In its encephalopathic form, pathognomonic callosal lesions permit an immediate diagnosis. We believe that the diagnosis of SS can be made when only the encephalopathy and pathognomonic MRI lesions are present; the BRAO and HL need not be present. We have also found the "string of pearls" MRI finding--the studding of the internal capsules with microinfarcts--to be most helpful--if not pathognomonic. This sign is always associated with the clusters of corpus callosum lesions, is especially striking on diffusion weighted imaging, and is associated with long-tract findings. We discuss the newly appreciated BRAO subset of SS and offer preliminary treatment suggestions for this subset. We also call attention to our development of an International Collaborative Study of SS and an educational website (http://www.ucalgary.ca/susac).

Susac's syndrome (SS) is a rare, immune-mediated endotheliopathy affecting the microvasculature of the brain, the inner ear and the retina. Clinical presentation is characterised by a triad: encephalopathy, hearing loss and branch retinal artery occlusion (BRAO). Given the rarity of this disease, its natural history still remains partially unknown, but lethal cases appear to be extremely rare since there has never been, to our knowledge, a report of SS leading to death. We report 2 cases of SS illustrating the multiplicity of neurological symptomatology and its unpredictable course. One case is particularly unusual due to its severe neurological evolution, leading to death despite treatments. This report presents clinical and paraclinical findings contributory to SS diagnosis and offers an innovative perspective on disease management. These cases represent the potential severity of this disease. Early, aggressive treatment strategies may be warranted for SS in order to avoid neurological deterioration and lethal evolution.

Ménière syndrome is an inner ear disorder characterized by spontaneous attacks of vertigo, fluctuating low-frequency sensorineural hearing loss, aural fullness and tinnitus. When the syndrome is idiopathic and cannot be attributed to any other cause (eg, syphilis, immune-mediated inner ear disease, surgical trauma), it is referred to as Ménière disease. This article reviews the physiologic effects of Ménière disease on vestibular function, as measured by caloric, head impulse, and vestibular-evoked myogenic potential testing.

Susac syndrome is a rare neurologic disorder first described by Susac et al. in 1979. Clinically, Susac syndrome consists of a triad including encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. Microinfarction is believed to be the basic histologic feature and MR is considered the best imaging modality. This case documents the typical imaging findings of Susac syndrome, as well as the unique MR imaging finding of cranial nerve enhancement.

A 52-year-old man developed transient, migratory polyarthralgias in the presence of hearing loss. He then developed persistent leukocytosis and thrombocytosis. His initial transient, bilateral visual obscurations happened in context with bilateral disk edema and an enlarged blind spot. Visual symptoms progressed to vision loss and multiple branch retinal artery occlusions. It was not until later in the disease progression that gastrointestinal symptoms occurred. Electron microscopy of duodenal biopsies confirmed a diagnosis of Whipple disease.

BACKGROUND Susac's syndrome (SS) is a rare arteriopathy affecting the microvasculature of the brain, retina, and inner ear, resulting in encephalopathy, branch retinal artery occlusion and hearing loss. Anecdotal reports exist on SS being associated with a humoral immune response against endothelial cells. However, no original data has ever been published. OBJECTIVE To analyze serum and CSF from a patient with SS for the presence of CNS auto-antibodies and, if present, to further characterize such antibodies immunologically. METHODS Serum and CSF samples were examined by indirect immunofluorescence on adult mouse cerebrum, cerebellum, brain stem, and inner ear tissue sections, and IgG subclasses were determined. RESULTS Anti-endothelial antibodies were found at a titre of 1:960 in serum but not CSF. Antibodies belonged to the complement activating IgG1 subclass. Glucocorticoid treatment resulted in a decrease of titres (1:480), though the antibodies remained clearly detectable. CONCLUSION Our finding of anti-endothelial cell antibodies in a patient with SS is important in the light of previous pathological data suggesting that SS is associated with endothelial damage. Larger serological studies are now warranted to assess systematically the frequency and relevance of auto-antibodies in SS.