Wegener’s granulomatosis

PA S1 S01.134 Myeloblastin Antigen against which the autoimmunity of Wegener s granulomatosis is directed... 

Pillinger, M.. and Staud, R., Wegener s granulomatosis in a patient receiving propylthiouracil for Graves disease, Semin. Arthritis Rheum., 28, 124, 1998. 

Miscellaneous Epistaxis dry nose or mouth transient chest pain (rarely persists after discontinuation) vasculitis (including Wegener s granulomatosis) anemia lymphadenopathy edema tinnitus hearing impairment weight loss. 

Azathioprine also has applications in certain disorders with autoimmune components, most commonly rheumatoid arthritis. It is as effective as cyclophosphamide in the treatment of Wegener s granulomatosis. It has largely been replaced by cyclosporine in immunosuppressive therapy. Relative to other cytotoxic agents, the better oral absorption of azathioprine is the reason for its more widespread clinical use. 

H. Other considerations Enbrel has been designated an orphan product for use in the treatment of juvenile rheumatoid arthritis and Wegener s granulomatosis. 

Cyclophosphamide is active against rheumatoid arthritis when given orally at dosages of 2 mg/kg/d but not when given intravenously. It is used regularly to treat systemic lupus erythematosus, vasculitis, Wegener s granulomatosis, and other severe rheumatic diseases. 

Cyclosporine is approved for use in rheumatoid arthritis and retards the appearance of new bony erosions. Its usual dosage is 3-5 mg/kg/d divided into two doses. Anecdotal reports suggest that it may be useful in systemic lupus erythematosus, polymyositis and dermatomyositis, Wegener s granulomatosis, and juvenile chronic arthritis. 

MMF is effective for the treatment of renal disease due to systemic lupus erythematosus and may be useful in vasculitis and Wegener s granulomatosis. Although MMF is occasionally used at a dosage of 2 g/d to treat rheumatoid arthritis, there are no well-controlled data regarding its efficacy in this disease. 

Other rheumatic diseases in which the corticosteroids potent anti-inflammatory effects may be useful include vasculitis, systemic lupus erythematosus, Wegener s granulomatosis, psoriatic arthritis, giant cell arteritis, sarcoidosis, and gout. 

Erban SB, Sokas RK. Kaposi s sarcoma in an elderly man with Wegener s granulomatosis treated with cyclophosphamide and corticosteroids. Arch Intern Med 1988 148(5) 1201-3. 

Although the most common methotrexate dosing regimens for the treatment of rheumatoid arthritis are 15 or 17.5 mg weekly, there is an increased effect up to 30 or 35 mg weekly. The drug decreases the rate of appearance of new erosions. Evidence supports its use in juvenile chronic arthritis, and it has been used in psoriasis, psoriatic arthritis, polymyositis, dermatomyositis, Wegener s granulomatosis, giant cell arteritis, subacute lupus erythematosus, and vasculitis. 

A 30-year-old man developed destructive rhinitis due to cocaine abuse after initially presenting with Henoch-Schonlein purpura (121). Cocaine use can mimic vasculitis and is often accompanied by positive ANCAs. Cocaine-induced midline destructive lesions are characterized by mucosal damage and ischemic necrosis of the nasal septum. Histopathological similarity to leukocytoclastic vasculitis and the presence of PR3-ANCA can lead to confusion between Wegener s granulomatosis and cocaine-induced midline destructive lesions. 

ANCA Specific for Wegener s granulomatosis, polyarteritis nodosa, and related vasculitis-associated diseases... 

Chest radiography Tuberculosis, sarcoidosis, Wegener s granulomatosis... 

Autoimmune hepatitis (see chapter 33) Autoimmune cholangitis (see chapter 32) Primary biliary cholangitis (see chapter 32) Primary sclerosing cholangitis (see chapter 32) Wegener s granulomatosis... 

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