21/784. Pial involvement in Wegener's granulomatosis shown on MRI. Involvement of the brain and meninges is rare in Wegener's granulomatosis (WG); it has been reported in 1.2-8 % of patients. Meningeal involvement in WG has been reported in imaging as being confined to the dura mater, and is thought to represent granulomatous infiltration. We present a case of WG with abnormal pial enhancement and involvement of the perivascular spaces on MRI, pathologically proven to represent granulomatous infiltration due to the primary disease rather than to infection. ( info) |
22/784. Wegener's granulomatosis with unusual cavernous sinus and sella turcica extension. Intracerebral extension of Wegener's granulomatosis (WG) is rare. We present a patient with oculomotor and trochlear nerve palsy with histologically proved WG. An MR examination revealed granulomatous tissue in nasal cavity, paranasal sinuses with meningeal infiltration, and uncommon penetration into cavernous sinus and sella turcica. The MR images before and during pharmacological therapy are presented. ( info) |
23/784. Extensive involvement of the myocardium and the cardiac conduction system in a case of Wegener's granulomatosis. A 61-year-old female who had shown Raynaud's phenomenon and articular swelling for 10 years, was admitted to hospital because of fever of unknown origin (FUO) and dry cough. She was diagnosed by skin biopsy to have a collagen disease or overlap syndrome. anemia developed rapidly and FUO persisted, but blood culture was negative. Although indomethacin and prednisolone were administered for the progression of clinical signs and symptoms, severe dyspnea developed, resulting in bradycardia, followed by recurrent episodes of ventricular tachycardia. In spite of extensive treatment for her arrhythmia she died on the 9th hospital day. An autopsy revealed generalized Wegener's granulomatosis with extensive cardiac involvement. Necrotizing angitis and severe granulomatous inflammatory foci affected characteristically the common bundle of his and right bundle branch in addition to the ordinary myocardium. ( info) |
24/784. Isolated abdominal vasculitis as an atypical presentation of Wegener's granulomatosis. Although current classifications characterize vasculitic syndromes based upon the size of the vessels involved, the histopathology, and the presence or absence of antineutrophil cytoplasmatic antibodies ANCA (1-3), those occasional patients with vasculitis whose features are not typical may evade diagnosis and effective treatment. We report one such patient who presented with bilateral refractory uveitis and abdominal angina who had a positive C-ANCA. Because of his atypical presentation, this patient's disease progressed over 8 yr despite an extensive gastrointestinal evaluation, before a diagnosis of vasculitis was established angiographically, and immunosuppressive therapy was begun. ( info) |
| This study reports on a first case of granulomatous pneumocystis carinii pneumonia (PCP) in a human immunodeficiency virus-negative patient with antineutrophil cytoplasmic antibody-positive Wegener's granulomatosis whilst receiving immunosuppressive treatment. The patient presented with diffuse alveolar haemorrhage, pauci-immune rapid progressive glomerulonephritis and leukocytoclastic vasculitis of the skin. Granulomatous pneumocystis carinii pneumonia developed under immunosuppressive treatment with cyclophosphamide and prednisone. At the time pneumocystis carinii pneumonia developed, there was a marked lymphopenia with a very low CD8 cell count in the blood. Grocott staining in bronchoalveolar lavage fluid revealed no pneumocystis carinii. The diagnosis was made via a video-assisted thoracoscopic lung biopsy which showed granulomas containing high numbers of pneumocystis carinii cysts. ( info) |
26/784. Lacrimal drainage surgery in Wegener's granulomatosis. AIM: To examine the results of open lacrimal surgery in patients with Wegener's granulomatosis. methods: A retrospective review of patients with Wegener's granulomatosis who underwent lacrimal surgery over a 17 year period. RESULTS: 11 patients were identified and a total of 14 primary dacryocystorhinostomies (DCR) and one revisional DCR were performed; symptomatic relief was achieved in 13/14 operations and one patient required revisional surgery for persistent symptoms. There were no intraoperative and few postoperative complications. CONCLUSIONS: In contrast with some previous reports, open DCR appears to be a safe procedure and it is recommended as a treatment for lacrimal obstruction in patients with Wegener's granulomatosis, but an increase of perioperative immunosuppression is recommended in certain cases. ( info) |
27/784. sarcoidosis presenting as necrotizing sinus destruction mimicking Wegener's granulomatosis. We describe a 26-year-old man who developed nasal stuffiness and palatal destruction. biopsy of a mass in the ethmoid sinus confirmed sarcoidosis. Treatment was initiated with oral steroid and methotrexate, with marked improvement in his symptoms. Although paranasal sinus involvement in sarcoidosis is rare it should be considered in differential diagnosis of diseases causing palatal or paranasal sinus destruction. ( info) |
| A case is described of a 28-year-old man who presented with a picture of classical Wegener's granulomatosis; and who, in the absence of fulminating renal or respiratory disease died from myocardial infarction. Other reports of cardiac involvement are discussed and compared with the picture presented by this patient. ( info) |
29/784. cytomegalovirus retinitis in a patient with Wegener's granulomatosis. cytomegalovirus retinitis (CMVR) is a rare complication of systemic immunosuppressive therapy in patients with rheumatic disorders. We describe a 44-year-old man with Wegener's granulomatosis who was treated with cyclophosphamide and methylprednisolone and who subsequently developed bilateral CMVR. In spite of discontinuation of immunosuppressive therapy and administration of systemic ganciclovir, retinitis recurred and maintenance therapy was needed. In conclusion, increased awareness of this potentially serious complication of immunosuppressive therapy is critical. If immune recovery is slow after discontinuation of immunosuppressive agents, prolonged antiviral therapy is required in order to prevent recurrences of CMVR. ( info) |
30/784. Trigeminal trophic syndrome mimicking Wegener's granulomatosis. A case report with a review of the literature. Trigeminal neuropathy with nasal ulceration, called trigeminal trophic syndrome, is an unusual complication of anesthesia in the trigeminal area. We present a case to illustrate the diagnostic and management problems that this syndrome presents. ( info) |