1/26. interferon-alpha may exacerbate cryoblobulinemia-related ischemic manifestations: an adverse effect potentially related to its anti-angiogenic activity.The discovery of the strong association between hepatitis c virus (HCV) infection and the development of mixed cryoglobulinemia has motivated active testing of antiviral-directed alternative therapies. Several trials have demonstrated that classic cryoglobulinemia-associated manifestations improve with interferon-alpha (IFNalpha) treatment. Herein we report on 3 HCV-infected patients with severe cryoglobulinemia-related ischemic manifestations who were closely followed up during IFNalpha therapy. Clinical evaluations with special attention to ischemic lesions, liver function tests, and cryocrit determinations were serially performed. In addition to prednisone and immunosuppressive agents, the patients received IFNalpha at 3 x 10(6) units, 3 times per week for 2 months, 3 months, and 4 months, respectively. In all 3 patients, systemic features improved, liver function results returned to normal, and cryocrit values decreased. However, ischemic lesions became less vascularized and ischemia progressed, leading to transmetatarsal and subcondylar amputation, respectively, in 2 of the patients and fingertip necrosis and ulcer enlargement in the third. skin biopsies performed before IFNalpha therapy and after 2 months of IFNalpha therapy in the third patient showed a significant decrease in subepidermal microvessels. When IFNalpha was discontinued, the lesions finally healed. cryoglobulinemia-related ischemic lesions may worsen during IFNalpha treatment, presumably through a decrease in inflammation-induced angiogenesis. The anti-angiogenic activity of IFNalpha may delay the appropriate healing of ischemic lesions.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/26. Relapsing ischemic encephaloenteropathy and cryoglobulinemia.cryoglobulinemia is a rare cause of encephalopathy. The authors report three patients with strikingly similar clinical features of recurrent encephalopathy accompanied by symptoms of gastrointestinal ischemia. In only one patient was cryoglobulinemia ascertained in life during the final illness. The autopsy examinations all showed diffuse cerebral, enteral, and systemic small vessel lesions immunoreactive for immunoglobulins and typical of mixed essential cryoglobulinemia. This unusual relapsing clinical syndrome is readily misinterpreted as of nonorganic origin despite its potentially fatal prognosis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/26. hepatitis c virus infection with peripheral neuropathy is not always associated with cryoglobulinaemia.OBJECTIVES: To describe cases of peripheral neuropathy associated with chronic hepatitis c virus infection without mixed cryoglobulinaemia. methods: Four cases of peripheral neuropathy associated with chronic hepatitis c virus infection with persistent negativity of mixed cryoglobulinaemia were found. RESULTS: All patients had small increases of transaminase levels and a positive viraemia. Liver biopsy showed chronic active hepatitis in all but one case (Knodell 4-9, Metavir A0F0-A3F3). Neuromuscular biopsy showed axonal neuropathy associated with lymphoid infiltrates around small vessels in two cases. rheumatoid factor was always negative and C4 complement level was always normal. In three patients, neuropathy improved with interferon alpha, interferon alpha ursodesoxycholic acid, or steroids plasma exchange. CONCLUSION: Peripheral neuropathy may be associated with hepatitis c virus infection without mixed cryoglobulinaemia.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/26. Palpable purpura at the site of previous herpes zoster in association with mixed cryoglobulinemia and hepatitis c virus infection.A 70-year-old woman affected with chronic active hepatitis c and mixed cryoglobulinemia presented a palpable purpura on her abdominal skin in a metameric configuration, fourteen months after a herpes zoster in the same site. Histopathology showed a small vessel leukocytoclastic vasculitis in the superficial dermis. Post-zoster eruptions are variable, and their spectrum is still expanding, although the pathogenesis remains to be elucidated. Perhaps our case represents an isomorphic reaction, because this palpable purpura, probably related to HCV infection, occurred several months after herpes zoster.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/26. Type I cryoglobulinemia presenting as hemorrhagic crusted leg ulcers.cryoglobulins (CGs) are serum proteins that reversibly precipitate when the serum is cooled below 37 degrees C. Cryoglobulinemias are associated with a variety of diseases, including hematologic, autoimmune, and infectious. Isolation of CGs requires handling the specimen with extreme care. We describe a 70-year-old man, recently diagnosed with chronic lymphocytic leukemia, who developed hemorrhagic crusted skin ulcers on his legs that were pruritic and painful. Results of skin biopsies showed dilated superficial, mid-dermal and deep-dermal blood vessels containing pink amorphous material and red blood cells. cryoglobulinemia was suspected; however, an initial search for CGs was negative. There was concern about suboptimal handling of the specimen, and the test was repeated. Two percent CGs with IgM kappa specificity were detected. This case illustrates the importance of the proper handling of specimens for evaluation of cryoglobulinemia.- - - - - - - - - - ranking = 7.3124755825487keywords = blood vessel, vessel (Clic here for more details about this article) |
6/26. purpura with cold urticaria in a patient with hepatitis c virus infection-associated mixed cryoglobulinemia type III: successful treatment with interferon-beta.We describe a 54-year-old man with hepatitis c virus (HCV) infection-associated cryoglobulinemia type III. The patient had suffered from cold-induced urticaria that left purpuric eruptions up to 1 cm in diameter, intermittent migratory joint pain for seven years and mild liver dysfunction for nine years. hemophilia a was diagnosed when the patient was 26 years old, and he was then given infusions of factor viii for a short time. In both skin biopsy samples from urticarial and purpuric eruptions, mild inflammatory infiltration by polymorphonuclear leukocytes with nuclear dust, extravasation of erythrocytes and deposition of IgM and C3 in the superficial blood vessels were observed. After antiviral treatment with interferon-beta, the clinical symptoms and the cryoglobulin and HCV-rna in the serum disappeared. There has been no recurrence in the subsequent nine years.- - - - - - - - - - ranking = 7.3124755825487keywords = blood vessel, vessel (Clic here for more details about this article) |
7/26. Cerebral vasculitis in a patient with HCV-related type II mixed cryoglobulinemia.The peripheral nervous system is often involved in patients with mixed cryoglobulinemia (MC), while there are few reports of central nervous system involvement. We describe a case of HCV-related type II MC with peripheral and central nervous system involvement. A 61-year-old woman, suffering from flaccid tetraparesis, was referred to our department because of an increasing disability. The presence of delirium prompted us to also investigate the central nervous system. MMSE, EEG, EMG, brain CT-scan, color-Doppler of neck vessels, retinal fluorangiography and brain MRI were performed. These investigations suggested a cerebral vasculitis. The finding of very low C4 serum levels, together with high rheumatoid factor serum levels, suggested the search for cryoglobulins. The laboratory findings showed a HCV-related type II (IgMk) MC. A marked improvement of symptoms and of laboratory data was obtained by treatment with methylprednisolone cyclophosphamide.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/26. Initial increase in the cryoglobulin level after rituximab therapy for type II cryoglobulinemia secondary to waldenstrom macroglobulinemia does not indicate failure of response.Type II (mixed) cryoglobulinemia is a systemic vasculitis that may affect many organs, including the skin, leading to ulcerations secondary to immune complex deposition. We report a patient who presented with a large necrotic ulcerative lesion on the right ankle secondary to type II cryoglobulinemic vasculitis due to waldenstrom macroglobulinemia that was resistant to multiple modalities of therapy. amputation to prevent further necrosis and infections was almost performed. Treatment with rituximab was initiated and the patient had a dramatic response. This case report highlights the effectiveness and prompt response to rituximab when used in patients with small vessel vasculitis and skin ulceration. We describe a rise in the cryoglobulin level that may occur initially after rituximab therapy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/26. Cryoglobulinemic vasculitis and lymphocytic interstitial pneumonitis in a person with HIV infection.A wide variety of rheumatologic complications have been reported in the setting of hiv-1 infection. We present the case of an hiv-1-infected patient with small-vessel vasculitis and inflammatory lung disease. This case illustrates the complexity of the differential diagnosis of inflammatory and autoimmune disease states in hiv-1-infected patients.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/26. Successful management of cryoglobulinemia-induced leukocytoclastic vasculitis with thalidomide in a patient with multiple myeloma.Leukocytoclastic vasculitis (LV) is a systemic inflammatory disorder involving mostly the small vessels. It is characterised by segmental angiocentric neutrophilic inflammation, endothelial cell damage and fibrinoid necrosis. LV is related to a variety of clinical disorders including cryoglobulinemia and, very rarely, multiple myeloma (MM), among many others. The development of LV in patients with MM has been linked to cryoglobulinemia, infections, drugs and paraneoplasia. It has been speculated that myeloma patients with a poorer prognosis and progressive disease are more prone to develop LV. thalidomide is a rediscovered old drug with anti-angiogenic, immunomodulatory and anti-inflammatory properties. It is highly effective in the treatment of MM and other clinical disorders such as leprosy, various cancers, graft-versus-host disease and autoimmune diseases. We report here a female patient with Durie-salmon stage IIA MM who initially presented with cryoglobulinemia and LV. LV in this patient was primarily considered to be the result of progressive cryoglobulinemia, which was closely associated with MM. She was successfully managed with thalidomide and dexamethasone.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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