1/784. Alternating antineutrophil cytoplasmic antibody specificity: drug-induced vasculitis in a patient with Wegener's granulomatosis. We describe a patient who presented with Wegener's granulomatosis associated with antineutrophil cytoplasmic antibodies (ANCA) directed against proteinase 3 (PR3) with a cytoplasmic immunofluorescence pattern (cANCA), whose ANCA type changed to antimyeloperoxidase antibodies with a perinuclear immunofluorescence pattern (pANCA) when treated with propylthiouracil, and changed back to anti-PR3 antibodies with cANCA after the medication was discontinued. The patient developed flares of vasculitis symptoms associated with rises in either type of ANCA. Tests for antimyeloperoxidase ANCA were repeatedly negative before the drug was started, strongly implicating the drug as the cause of the episode. This case demonstrates that patients with idiopathic ANCA-positive vasculitis may quickly develop a superimposed drug-associated ANCA-positive vasculitis. Iatrogenic vasculitis should be suspected when a patient with idiopathic vasculitis with one type of ANCA develops the other type of ANCA. ( info) |
2/784. An overlap syndrome with features of atypical cogan syndrome and Wegener's granulomatosis. A 48 year old women developed serous otitis, scleritis, myalgia, vertigo, polyneuropathy, crescentic glomerulonephritis, general cerebral dysrythmia, hilar adenopathy, and retroorbital granulomatous inflammation. Pulmonary manifestations were absent and antibodies against neutrophilic cytoplasmic antigens (ANCA) could not be detected. The clinical picture was classified as an overlap syndrome with features of both atypical cogan syndrome and Wegener's granulomatosis. The patient responded to treatment with high dose corticosteroids and pulse cyclophosphamide. ( info) |
3/784. Long-term follow-up of renal transplantation for Wegener's disease. Eight patients with end-stage renal failure due to Wegener's granulomatosis underwent renal transplantation at the University of minnesota. Seven patients were alive with a functioning graft 40 to 128 months posttransplant (mean follow-up: 91 months). One patient died 126 months posttransplant with a well-functioning graft. Posttransplant immunosuppression controlled primary disease in all but 1 patient, who presented with perisinusitis. Recurrent disease was not noted in any of the transplanted organs. We conclude that transplantation is an excellent treatment for renal failure secondary to Wegener's disease. ( info) |
4/784. Pituitary involvement by Wegener's granulomatosis: a report of two cases. We describe two cases of pituitary involvement by Wegener's granulomatosis. At initial presentation, or during subsequent disease "flares," a pattern of pituitary abnormality was suggested. During periods of remission, we found the pituitary returned to a nearly normal appearance. Loss of the normal posterior pituitary T1 hyper-intensity matched a clinical persistence of diabetes insipidus, suggesting there is permanent damage to this structure by the initial disease process. ( info) |
5/784. Takayasu's arteritis coexisting with Wegener's granulomatosis in a teenager with renal insufficiency: case report. A case of Wegener's granulomatosis (WG) coexisting with Takayasu's arteritis (TA) in a 12-year-old girl is presented. She presented with fulminant and severe renal insufficiency due to crescentic glomerulonephritis. At autopsy, aortic lesions of Takayasu's arteritis coexisted with pulmonary and renal findings of WG, and the patient's serum at autopsy had an elevated level of antineutrophil cytoplasmic antibodies (ANCA). Both forms of vasculitis have been thought to be triggered by infectious agents and it is postulated that this occurrence accounts for the coexistence of the two forms of vasculitis in this case. ( info) |
6/784. Wegener's granulomatosis causing laryngeal and tracheobronchial obstruction in an adolescent girl. A 12-year-old girl presenting with a painful neck mass and ulcerated gingival lesions was found to have Wegener's granulomatosis involving the gingivae, paranasal sinuses, ears, mastoids, larynx, trachea, bronchial tree, lungs, kidneys and joints. The initial illness and two subsequent exacerbations were treated with intravenous and oral cyclophosphamide and prednisone. Secondary infections have been managed with establishment of good drainage and use of appropriate antibiotics. The patient is well with a normal sedimentation rate 4 1/2 years after the onset of her disease. ( info) |
7/784. Antithymocyte globulin treatment of orbital wegener granulomatosis: a follow-up study. PURPOSE: To describe the follow-up of patients with orbital wegener granulomatosis after antithymocyte globulin treatment. methods: patients with ocular/orbital wegener granulomatosis refractory to standard treatment were selected for immunotherapy with rabbit antithymocyte globulin intravenously. The specific ocular/orbital symptoms were monitored in patients with a vision-threatening form of wegener granulomatosis or a life-threatening form with ocular symptoms. RESULTS: One patient had a complete remission, two patients had remissions but still needed additional treatment, and one patient remained refractory. CONCLUSIONS: In selected patients, antithymocyte globulin immunotherapy may present an alternative when vision or life is threatened by orbital wegener granulomatosis. ( info) |
8/784. Pulmonary manifestations of ulcerative colitis mimicking Wegener's granulomatosis. We describe 2 patients with quiescent ulcerative colitis who developed clinical syndromes suggesting Wegener's granulomatosis. Both patients had features of systemic illnesses, nodular lung lesions, and antineutrophil cytoplasmic antibodies. Although the inflammatory bowel disease of both patients was quiescent at the time of their pulmonary presentations, lung biopsy results were consistent with pulmonary complications of ulcerative colitis, an unusual manifestation of that disorder. These cases illustrate that pathological confirmation of the diagnosis is essential in cases of suspected vasculitis. The patients' pulmonary lesions resolved promptly after treatment with corticosteroids. ( info) |
9/784. Wegener's granulomatosis and alpha1-antitrypsin-deficiency emphysema: proteinase-related diseases. Wegener's granulomatosis (WG) and alpha1-antitrypsin (alpha1-AT)-deficiency emphysema are both uncommon disorders. A relationship may exist between these diseases involving the proteinase and antiproteinase balance in the lung. A case is presented of WG and alpha1-AT-deficiency emphysema occurring in the same patient. Previous studies concerning the correlation between abnormal alpha1-AT alleles and WG are discussed. Potential mechanisms for the relationship and recommendations for screening are given. ( info) |
10/784. Synovial actinomycosis of the hip: case report and review. A case of hematogenous spread of an actinomycotic granule to the left hip joint is presented. This occurred in a 62-year-old woman under immunosuppressive treatment for Wegener's disease. It was a chance finding on routine synovium biopsy during a total hip replacement. Treatment consisted of 1 g ampicillin I.M. a day for six weeks. The postoperative course was uneventful. ( info) |