Immune-Complex Allergic Vasculitis in Association with the Immune-Complex Allergic Vasculitis in Association with the Development of Transverse Myelitis: A Case Report

A severe vasculitis, probably therapy related, in a sixty-four-year-old man being treated for possible subacute bacterial endocarditis, was associated with the development of transverse myelitis. It is hypothesized that the vasculitis affected the small vessels to the spinal cord in the same way that systemic vasculitis can also cause a transverse myelitis

Autoantibodies in systemic vasculitis

Systemic vas culitis is a heterogeneous group of disorders characterized by inflammation and necrosis in the vessel wall. the diagnosis of a systemic vasculitis is challenging, because patients usually present a broad spectrum of manifestations that vary according to the predominant size of vessels affected, organs and systems involved, and the extent of the inflammatory process (I). in systemic vasculitis, disease manifestations usually cluster into clinical phenotypes and definite diagnosis rely on confirmation by tissue biopsy, angiography, or by serologic tests. However, when a systemic vasculitis is under investigation, it is of paramount importance to keep in mind vasculitis mimics (e.g., genetic vascular disorders and atheroembolic diseases) and secondary causes (i.e., infections, malignancy, connective tissue disorders, or drugs) (2).The main serologic tests for the diagnosis of primary systemic vasculitides are antineutrophil cytoplasmic antibodies (ANCA), cryoglobulins, anti-glomerular basement membrane (anti-GBM) antibodies, and anti-Clq antibodies (3). Although, several other autoantibodies have been investigated in systemic vasculitis, the clinical usefulness of these antibodies still needs further investigation (Table 1). Herein, the main autoantibodies and their clinical associations in systemic vasculitis are reviewed.Universidade Federal de São Paulo, Escola Paulista Med, Dept Internal Med, Div Rheumatol, São Paulo, BrazilUniversidade Federal de São Paulo, Escola Paulista Med, Dept Internal Med, Div Rheumatol, São Paulo, BrazilWeb of Scienc

Clinical Spectrum of Medium-Sized Vessel Vasculitis

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis of medium-sized visceral vessels. However, cutaneous arteritis (CA) and gastrointestinal (GI) vasculitis are forms of single-organ vasculitis having indistinguishable histopathologic findings from PAN. The aim of this study was to evaluate and compare the clinical characteristics, treatment, and outcomes of patients with systemic PAN, CA, and GI vasculitis

The clinical characteristics of retinal vasculitis in HLA-B27-positive patients

Purpose: To investigate the ocular and systemic manifestations of retinal vasculitis in HLA-B27-positive patients. Methods: Retrospective noncomparative case series of 9 HLA-B27-positive patients with uveitis and retinal vasculitis. Main outcome measures consisted of ocular and angiographic findings and assessment of any additional systemic disorders. Results: Three male and 6 female HLA-B27-positive patients with a median age of 32 years were diagnosed with retinal vasculitis. Concurrent intraocular inflammation was noted in all patients. All patients suffered from extensive vasculitis of the large retinal veins. Five patients developed retinal vasculitis at the onset of uveitis and the remaining 4 exhibited retinal vasculitis 1-15 years after the onset of uveitis. Vascular occlusions occurred in 4 patients and subsequent neovascularizations developed in 3. Three patients were diagnosed with an HLA-B27-associated systemic disease. Conclusion: Retinal vasculitis may develop in the wake of HLA-B27-associated uveitis and might represent a rare manifestation of HLA-B27-associated disease

Dermatological Lesions of Cholesterol Embolization Syndrome and Kaposi Sarcoma Mimic Primary Systemic Vasculitis: Case Report Study

Primary systemic vasculitis can present with a wide spectrum of manifestations ranging from systemic non-specific features such as fever, malaise, arthralgia, and myalgia to specific organ damage. We describe two cases of cholesterol embolization syndrome and Kaposi sarcoma mimicking primary systemic vasculitis, both of which were characterized by features such as livedo reticularis, blue toe syndrome, a brown, purpuric skin rash, and positive p-ANCA associated with Kaposi sarcoma. Establishing the right diagnosis was challenging, and thus we aim in this study to highlight the possible ways to distinguish them from primary systemic vasculitis. Keywords: Dermatological lesions, Cholesterol embolization syndrome, Kaposi sarcoma, vasculitis mimic

A sore red eye with systemic involvement

The study of happiness has long been a playground for philosophical speculation. By lack of empirical measures of happiness, it was not possible to check propositions about the matter. In the late 20th century, survey-research methods introduced by the social sciences have brought a break-through. Dependable measures of happiness have developed, by means of which a significant body of knowledge has evolved

Optimal management of Cogan’s syndrome: a multidisciplinary approach

Cogan's syndrome (CS) is a rare disorder characterized by nonsyphilitic interstitial keratitis (IK) and audio-vestibular symptoms. CS affects mainly young Caucasian adults, mostly during their first three decades of age, and may develop into typical and atypical variants. Typical CS manifests primarily with IK and hearing loss, whereas atypical CS usually presents with inflammatory ocular manifestations in association with audio-vestibular symptoms but mostly different Ménière-like symptoms and, more frequently, with systemic inflammation (70%), of which vasculitis is the pathogenic mechanism. CS is considered as an autoimmune- or immune-mediated disease supported mainly by the beneficial response to corticosteroids. Using well-developed assays, antibodies to inner ear antigens, anti-Hsp70, and antineutrophil cytoplasmic antibodies were found to be associated with CS. Corticosteroids represent the first line of treatment, and multiple immunosuppressive drugs have been tried with variable degrees of success. Tumor necrosis factor-alpha blockers and other biological agents are a recent novel therapeutic option in CS. Cochlear implantation is a valuable rescue surgical strategy in cases with severe sensorineural hearing loss unresponsive to intensive and/or innovative immunosuppressive regimens

Role of brain perfusion SPECT with 99mTc HMPAO in the assessment of response to drug therapy in patients with autoimmune vasculitis: a prospective study

Abstract BACKGROUND: The diagnosis of vasculitis in the brain remains a quite difficult achievement. To the best of our knowledge, there is no imaging method reported in literature which is capable of reaching to a diagnosis of vasculitis with very high sensitivity. AIM: The aim of this study was to determine whether perfusion brain single photon emission computed tomography (SPECT) can be usefully employed in monitoring the treatment of vasculitis, allowing treating only potentially responder patients and avoiding the side effects on patients who do not respond. MATERIALS AND METHODS: Twenty patients (two males and 18 females) suffering from systemic lupus erythematosus (SLE; n = 5), Behcet's disease (BD; n = 5), undifferentiated vasculitis (UV; n = 5), and Sjogren's syndrome (SS; n = 5) were included in the study. All patients underwent a wide neurological anamnestic investigation, a complete objective neurological examination and SPECT of the brain with 99mTc-hexamethyl-propylene-aminoxime (HMPAO). The brain SPECT was then repeated after appropriate medical treatment. The neurological and neuropsychiatric follow-up was performed at 6 months after the start of the treatment. RESULTS: Overall, the differences between the scintigraphic results obtained after and before the medical treatment indicated a statistically significant increase of the cerebral perfusion (CP). In 19 out of 200 regions of interest (ROI) studied, the difference between pre- and post treatment percentages had negative sign, indicating a worsening of CP. This latter event has occurred six times (five in the same patients) in the UV, 10 times (eight in the same patients) in the SLE, never in BD, and three times (two in the same patient) in the SS. CONCLUSION: The reported results seem to indicate the possibility of identifying, by the means of a brain SPECT, responder and nonresponder (unchanged or worsened CP) patients, affected by autoimmune vasculitis, to the therapy