1/101. 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.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
2/101. Treatment of Wegener's granulomatosis with immune globulin: CNS involvement in an adolescent female.OBJECTIVE: To describe the use of intravenous immune globulin (IVIG) to treat Wegener's granulomatosis (WG) in an adolescent female with an abnormal magnetic resonance imaging (MRI) scan and electroencephalogram (EEG), as well as central nervous system involvement manifesting as generalized seizures. CASE SUMMARY: A 15-year-old white girl diagnosed with WG and receiving prednisone and cyclophosphamide was admitted with new-onset tonic-clonic seizures. The patient received phenobarbital and phenytoin to control seizures and was receiving cyclophosphamide and corticosteroids for WG. She developed cyclophosphamide-induced cystitis and was started on a four-day therapeutic course of IVIG following the discontinuation of cyclophosphamide. After 16 days of hospitalization, repeat EEG and MRI were within normal limits, and laboratory and clinical improvement was evident in at least nine of the affected organ systems including pulmonary, hematologic, renal, ocular, cutaneous, musculoskeletal, central nervous system, gastrointestinal, and genitourinary. The patient was discharged with clinical involvement of WG documented in two organ systems, hematologic and renal. DISCUSSION: WG is a form of vasculitis believed to develop due to an autoimmune disorder. The diagnosis is based on radiographic and histopathologic findings, as well as the presence of elevated antineutrophil cytoplasmic antibodies and a suggestive clinical presentation. The presentation is widely variable and is most commonly associated with upper-airway involvement such as sinusitis, cough, pulmonary infiltrates, and cavitary nodules. Renal involvement signifies generalized disease. Conventional treatment for WG includes cyclophosphamide and prednisone. Little information is available describing therapeutic alternatives. Cytotoxicity related to immunosuppressant regimens limits continuous treatment and may necessitate the use of alternative agents. CONCLUSIONS: This case describes the use of IVIG in an adolescent patient presenting with WG as a generalized, active disease with neurologic complications. IVIG may be useful in generalized, active WG complicated by intolerance to cyclophosphamide and seizures, but further study is necessary to define its role.- - - - - - - - - - ranking = 2keywords = organ (Clic here for more details about this article) |
3/101. 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.- - - - - - - - - - ranking = 0.85265133691067keywords = nerve (Clic here for more details about this article) |
4/101. otitis media as a sign of Wegener's granulomatosis in childhood.Wegener's granulomatosis (WG) is a rare disease among paediatric patients. Chronic otitis media with or without facial nerve dysfunction is a known manifestation of the disease among adults. A case of a 15-year-old boy with WG, whose initial symptoms were acute otitis media and facial nerve paralysis, is presented. The otorhinolaryngological manifestations, as well as diagnostic and current treatment modalities in paediatric patients with WG, are discussed.- - - - - - - - - - ranking = 1.7053026738213keywords = nerve (Clic here for more details about this article) |
5/101. P-ANCA-positive Wegener's granulomatosis presenting with hypertrophic pachymeningitis and multiple cranial neuropathies: case report and review of literature.An autopsy case of hypertrophic pachymeningitis and multiple cranial neuropathies is reported. A 53-year-old woman with paraplegia and various neurological signs which developed over a 2 year period was diagnosed as having an epidural mass with thickened dura mater extending from the lower cervical to the thoracic spinal cord. In addition, bilateral episcleritis, blephaloptosis, and blindness of the right eye with various cranial nerve deficits were found to be caused by the mass lesions involving the paranasal sinuses, orbit, and the cavernous sinus. Perinuclear antineutrophil cytoplasmic antibody (p-ANCA) was positive, but cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) was negative by enzyme-linked immunosorbent assay. The partially removed epidural mass with hypertrophied dura mater and biopsy of the paranasal lesions showed chronic granulomatous inflammation with vasculitis. The remaining lesions resolved with steroid therapy with remarkable neurological improvement. The positive p-ANCA test, paranasal involvement, the report of a similar histopathological case and a review of the literature on granulomatous pachymeningitis suggest the presence of p-ANCA-positive Wegener's granulomatosis with central nervous system involvement characterized by hypertrophic pachymeningitis and/or multiple cranial neuropathies.- - - - - - - - - - ranking = 0.85265133691067keywords = nerve (Clic here for more details about this article) |
6/101. An unusual case of Wegener's granulomatosis.A 35-year-old Malay man underwent treatment for uveitis of the right eye in 1992 but developed marked visual impairment in the affected eye after he failed to attend follow-up. Two years later, he complained of difficulty swallowing and was found to have left sided X and XI cranial nerve palsies. Chest radiograph showed a cavitating lesion in the lower zone of the right lung field. inflammation and perforation of the nasal septum was found on examination of his upper respiratory tract. Punch biopsies taken from that area showed chronic inflammatory change and necrotizing vasculities. The patient was diagnosed as having Wegener's granulomatosis and made a very good recovery with immunosuppressive therapy.- - - - - - - - - - ranking = 0.85265133691067keywords = nerve (Clic here for more details about this article) |
7/101. Wegener's granulomatosis--a diagnostic challenge.Wegener's granulomatosis is a systemic vasculitis characterized by necrotizing granulomatous lesions in the upper and lower respiratory tracts, glomerulonephritis and vasculitis involving other organs. Limited forms have been described in which some features of the disease may be absent. Four patients with this disease are being reported with special emphasis on differences in presentation, the ensuing diagnostic problems, and individual outcome. In three, the disease began as a limited form with upper respiratory tract and eye involvement, while in one patient, onset was systemic including affection of the lower respiratory tract. The mean delay from first symptoms to diagnosis was 20 months--much longer for the three limited forms than for the one with systemic onset, in whom the condition was recognized after 2 months following initial misdiagnosis of respiratory and urinary tract infections. One patient developed endocarditis and required aortic valve replacement. Immunofluorescence revealed antineutrophil cytoplasmic antibodies in all, three showing a cytoplasmic pattern and antibodies to proteinase 3, and the fourth a perinuclear pattern and antibodies to myeloperoxidase. Upper respiratory tract biopsies were not specific. kidney biopsies were performed in all the patients and were crucial for definitive diagnosis and treatment of the disease, which was successful in three patients.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
8/101. An unusual case of multiple cranial nerve palsies in Wegener's granulomatosis.We describe an unusual case of Wegener's granulomatosis, which initially caused fulminant palsies affecting cranial nerves II, V, VI, VII, and VIII during a brief episode of the disease. The patient was successfully treated with immunosuppressive therapy. Wegener's granulomatosis should be suspected when multiple cranial nerves are initially affected.- - - - - - - - - - ranking = 5.115908021464keywords = nerve (Clic here for more details about this article) |
9/101. Wegener's granulomatosis presenting as mediastinal soft tissue mass invading the tracheal wall.Wegener's granulomatosis (WG) is a clinicopathologic entity of unknown cause characterised by a necrotising granulomatous vasculitis affecting multiple organs, especially the upper and lower respiratory tracts, lung and kidney. The lung is the most frequently, and sometimes the only involved organ. Single or multiple nodules, with or without cavitation, are the most common pulmonary manifestations in WG, but mediastinal involvement is atypical. The sole tracheal involvement is rare and hilar and mediastinal involvement has been thought not to be part of the clinical feature. We experienced a rare case of WG presenting as paratracheal mediastinal lesions with tracheal wall invasion, which responded dramatically to corticosteroid treatment. We present this case with a review of the literature.- - - - - - - - - - ranking = 2keywords = organ (Clic here for more details about this article) |
10/101. Wegener's granulomatosis responding to antituberculous drugs.We present a case of Wegener's granulomatosis (WG) that responded to antituberculous drugs. A 44-year-old woman with multiple nodules on chest radiograph received a diagnosis of pulmonary tuberculosis because open-lung biopsy specimens showed caseous granulomas. Her chest shadows underwent repeated resolution after the start of antituberculous treatment, and relapse after the cessation of the drugs. Antineutrophil cytoplasmic antibody was positive (14 enzyme-linked immunosorbent assay units), and the second lung biopsy specimens showed necrotizing granulomas and vasculitis without pathogenic organisms. Thus, the patient received a diagnosis of WG and was successfully treated with trimethoprim/sulfamethoxazole 10 years after her initial evaluation. Antituberculous drugs were effective in this case of WG.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
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