No abstract available.
Wegener Granulomatosis*

No abstract available.
Maxillary Sinus* ; Wegener Granulomatosis*

No abstract available.
Kidney Transplantation* ; Wegener Granulomatosis*

No abstract available.
Antibodies, Antineutrophil Cytoplasmic* ; Wegener Granulomatosis*

No abstract available.
Antibodies, Antineutrophil Cytoplasmic ; Fever* ; Ulcer ; Wegener Granulomatosis

Wegener's granulomatosis is a distinct form of necrotizing granulomatous vasculitis which usually affects the kidneys and the upper and lower respiratory tracts. Unusual manifestations have also been reported, and these include colitis, urethritis and diabetes insipidus. We describe a case of Wegener's granulomatosis which presented with rapidly progressive renal insufficiency, sudden deafness, red eye, facial palsy, and complicated by uncommon manifestations that were diffuse pulmonary hemorrhage and thrombotic thrombocytopenic purpura.
Aged ; Cyclosporine/therapeutic use ; Female ; Hemorrhage/complications* ; Human ; Lung Diseases/complications* ; Prednisolone/therapeutic use ; Purpura, Thrombotic Thrombocytopenic/complications* ; Wegener's Granulomatosis/drug therapy ; Wegener's Granulomatosis/diagnosis ; Wegener's Granulomatosis/complications*

We reviewed ten cases of Wegener's granulomatosis with special emphasis on the characteristics of the early stage of Wegener's granulomatosis. All patients presented with nonspecific symptoms and signs, so that Wegener's granulomatosis was not initially considered. However, half of the patients had clinical or radiologic disease in the nose/or paranasal sinuses as the primary presenting problems and showed neutrophil microabscess surrounded by palisading epithelioid cells and irregularly arranged giant cells in the nasal biopsy as the most characteristic feature. Five of ten patients were believed to have a protracted superficial phenomenon before involvement of other organs, specifically the lung or kidney. Four of ten patients showed nonreactivity to ANCA test at the time of presentation. Although the number of cases reviewed in this study was small, the rate of nonreactivity to ANCA was higher than those of the larger series. The importance of early diagnosis of Wegener's granulomatosis can not be overemphasized in view of the fact that cases unrecognized clinicopathologically finally progress to full-blown systemic form of Wegener's granulomatosis with poor prognosis. The diagnosis of Wegener's granulomatosis should be based on a thorough and meticulous examination of its characteristic histologic changes in biopsied tissue particularly extravascular foci.
Adolescence ; Adult ; Antibodies, Antineutrophil Cytoplasmic/blood ; Female ; Human ; Male ; Middle Age ; Retrospective Studies ; Wegener's Granulomatosis/pathology* ; Wegener's Granulomatosis/drug therapy ; Wegener's Granulomatosis/diagnosis

In Wegener's granulomatosis, the classical clinical picture is composed of a triad of symptoms: sinusitis-rhinitis, pulmonary lesion and glomerulonephritis with granulomatous lesions and necrotizing vasculitis of involved organs. But localization in retroperitoneal tissue is unusual. In this case, hydronephrosis and ureteral obstruction is the sole presenting symptoms of the disease. At exploratory laparotomy, the right ureter was found to be obstructed by dense, extrinsic fibroinflammatory reaction. There was a necrotizing granulomatous vasculitis in the muscle layer of the ureter.
Glomerulonephritis ; Hydronephrosis ; Laparotomy ; Ureter* ; Ureteral Obstruction* ; Vasculitis ; Wegener Granulomatosis*

Wegener's granulomatosis was first described by Wegener in 1936 as a vasculitis and necrotizing granuloma of the upper or lower respiratory tract. It is largely classified into a generalized necrotizing granuloma and a focal necrotizing granuloma. As a localized form of the disease, the respiratory tract is known to be involved frequently; however, the paranasal sinus is a less frequent site of involvement than other organs. In Korea, there has been no reports of Wegener's granulomatosis in the last ten years, and reports of localized form is extremely rare. We are reporting here a case of Wegener's granulomatosis in the maxillary sinus with literature review.
Granuloma ; Korea ; Maxillary Sinus* ; Respiratory System ; Vasculitis ; Wegener Granulomatosis*

Cryptococcosis, a disseminated illness due to Cryptococcus neoformans in most case, is an opportunistic systemic fungal infection that frequently infects the immunosuppressed host. Although it can cause disease in immunocompetent as well as immunocompromised hosts, the later group, particulary those with cell-mediated immune defects, are the greatest risk for severe disseminated disease. Cryptococcal tenosynovitis is extremely rare, with only 2 cases previously documented in the English literature. In this paper we report a case of cryptococcal tenosynovitis with multiple lung nodules in the setting of cryptococcemia in a patient with Wegener's granulomatosis who has been treated with steroid, oral cyclophosphamide for one year.
Cryptococcosis ; Cryptococcus neoformans ; Cyclophosphamide ; Humans ; Immunocompromised Host ; Lung ; Tenosynovitis* ; Wegener Granulomatosis*