Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Chronic urticaria remains a major problem in terms of etiology, investigation, and management. It is important to identify patients in whom physical urticaria is the principal cause of disability. Once confirmed by appropriate challenge testing, no further investigation is required. Urticarial vasculitis (UV) is a major differential diagnosis of "idiopathic" urticaria (CIU). I perform biopsy of most patients in this category because UV cannot be considered confirmed in the absence of histologic evidence. Patients with confirmed UV need to be thoroughly investigated for paraproteins, lupus erythematosus hepatitis B and C, and inflammatory bowel disease. Of patients with CIU, a few (<5%) prove to have food additive reactivity confirmed by placebo-controlled challenge testing. There is no convincing evidence of the involvement of Helicobacter pylori or parasite infestation as a cause of chronic urticaria, although H pylori could have an indirect role. Recently it has become clear that 27% to 50% of patients with CIU have functional autoantibodies directed against the alpha-chain of the high-affinity IgE receptor or less commonly against IgG. These antibodies, whose involvement has now been independently confirmed in several centers, are identified by autologous serum skin testing and confirmed by histamine release studies or immunoblotting. Their removal (by intravenous Ig or plasmapheresis) or treatment by cyclosporine has proved highly beneficial in severely affected patients. However, the routine treatment of all CIU patients, irrespective of etiology, remains the judicious use of H(1) antihistamines.
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PMID:Chronic urticaria. 1075 14

Urticarial vasculitis can present in a variety of ways, ranging from a primarily cutaneous disease consisting of chronic urticaria to a lupus-like disease with severe cardiopulmonary disease. Low complement levels and positive anti-C1q antibodies are markers of more severe disease. Care must be taken to look for an underlying condition. The mainstay of therapy is treatment of any underlying condition. Therapies most often employed include corticosteroids, antihistamine, and dapsone, but many others have been utilized.
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PMID:Urticarial vasculitis. 1768 41

Urticarial vasculitis is an eruption of erythematous wheals that clinically resemble urticaria but histologically show changes of leukocytoklastic vasculitis. In association with connective tissue disease it is most commonly seen complicating Systemic lupus erythematous (SLE) and, less often, Sjogren's syndrome. Here, we report a 25-year-old woman who developed SLE in 1998. In May 2013 she presented with urticarial vasculitis; her skin biopsy was consistent with leukocytoclastic vasculitis. She also developed bilateral uveitis. She had most of the clinical and laboratory characteristics of hypocomplementic urticarial vasculitis syndrome (HUVS) which is difficult to be differentiated from SLE. She was treated with high-dose prednisone, Mycophenolate Mofetil (MMF), colchicine, and Dapsone but failed. We decided to give her Rituximab (RTX), her urticarial vasculitis and uveitis symptoms improved significantly. Unfortunately, later on she presented with severe discoid lupus. We started her on thalidomide and responded well. Our case highlights that Rituximab is a good option for severe refractory urticarial vasculitis and thalidomide is effective in treatment of discoid lupus erythematosus (DLE), and can be used safely in specialist rheumatological practice.
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PMID:Successful Treatment of Urticarial Vasculitis in a Patient With Systemic Lupus Erythematosus With Rituximab. 3319 8