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

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*

Wegener's granulomatosis (WG)is a multisystemic inflammatory disease characterized by necrotizing granulomatous inflammation and vasculitis of unknown etiology which classically affects the upper airway,lung,and kidney.Subglottic stenosis (SGS)is rare and occurs independently of other features of active WG. SGS can be a life-threatening manifestation of disease,and prompt diagnosis is essential.Since the presenting symptoms of SGS can be nonspecific,SGS should be considered in the differential diagnosis of any patient with WG who has increasing dyspnea,voice change,or cough.Although both surgical and medical treatments have been utilized,the optimal therapeutic approach to SGS in patients with WG has not been determined.So far,a case of WG with SGS has not been reported in Korea.Recently,we experienced a case of 59-year-old woman with SGS in WG.She responded to prednisolone and methotrexate therapy and did not require surgical intervention.So we report this case with a review of literatures.
Constriction, Pathologic* ; Diagnosis ; Diagnosis, Differential ; Female ; Humans ; Inflammation ; Methotrexate ; Middle Aged ; Prednisolone ; Vasculitis ; Wegener Granulomatosis*

A case of Wegener's Granulomatosis of 35 year old Korean male having 38 months duration with typical clinical symptoms and coular manifestations had been treated and observed in our clinic form April to November 21, 1958. The diagnosis was confirmed by the histopathological studies on biopsy specimen and by postmortem examination. The ultimate cause of the death in this case was thought to be massive cerebral hemorrhages as a result of systemic involvement of necrotizing angitis which is one of the characteristic features of this disease. Uremia and hypertension were not observed in this case to the end of the course. There is no report dealing with Wegener's Granulomatosis in Korean literatures to the best of our knowledge at this moment.
Adult ; Autopsy ; Biopsy ; Cerebral Hemorrhage ; Diagnosis ; Humans ; Hypertension ; Male ; Uremia ; Wegener Granulomatosis*

Wegener`s granulomatosis(WG) is uncommon disease characterized by necrotizing granulomatous lesions in the both upper and lower respiratory tracts, generalized focal necrotizing vasculitis, and focal necrotizing glomerulonephritis. Other organs are also involved frequently. Ocular complications may occur secondary to contiguous granulomatous sinusitis or as a result of focal vasculitis and are encountered in 40-50% of cases of generalized form. We experienced a case of WG developed progressive paranasal sinusitis and rhinitis combined with orbital and ocular involvement. The diagnosis was confirmed by clinical findings and histopathologic features of tissue biopsy. The histopathology from periorbital area had typical findings of WG with necrotizing vasculitis and granuloma formation.
Biopsy ; Diagnosis ; Glomerulonephritis ; Granuloma ; Orbit* ; Respiratory System ; Rhinitis ; Sinusitis ; Vasculitis ; Wegener Granulomatosis*

Bronchocentric granulomatosis(BCG), first defined in 1973, consists of granulomatous replacement of bronchial mucous membrane, often with heavy eosinophilic reaction within and about the involved bronchi. Etiologic factors are from hypersensitivity reaction for aspergillus, most often from idiopathic form, and in others from being associated with mycobacterium, ecchinococcus, rheumatoid disease, ankylosing spodylitis, and glomerulonephritis. Diagnosis is responsible only for pathologic findings and, in many cases, is confirmed in postoperative findings with misleading for tumor, tuberculosis, infectious or Wegener's granulomatosis. We report a case of bronchocentric granulomatosis associated with aspergillus.
Aspergillus* ; Bronchi ; Diagnosis ; Eosinophils ; Glomerulonephritis ; Hypersensitivity ; Mucous Membrane ; Mycobacterium ; Tuberculosis ; Wegener Granulomatosis

Wegener's granulomatosis is an uncommon idiopathic, multisystem disease characterized by necotizing granuloma and vasculitis affecting small to medium sized vessels. It is classified into a classic form and a limited(localized) form based on the anatomic site of involvement by Carrington and Liebow in 1966. Recently, it has been made possible for the diagnosis of this disease to be carried out in an earlier stage by measurement of the ANCA(Antineutrophil cytoplasmic antibodies) and the result of treatment has improved since cyclophosphamide therapy. Wegener's granulomatosis in children has been extremely rare. The authors have observed 1 case of limited form of granulomatosis in a 12 year-old female child and report this with a literature review.
Antibodies, Antineutrophil Cytoplasmic ; Child* ; Cyclophosphamide ; Cytoplasm ; Diagnosis ; Female ; Granuloma ; Humans ; Methotrexate ; Vasculitis ; Wegener Granulomatosis*

Wegener's granulomatosis is characterized by necrotizing vasculitis and granuloma formation in both upper and lower respiratory tracts and in kidney. Other organs are also involved frequently. Ocular manifestations are Ehown in about 50% of patients with it. We experienced a case of Wegener's granulomatcsis developed prcgressive paranasal sinusitis and rhinitis combined with orbital and ocular involvement shown proptosis of both eyes, necrotic change of cornea and anterior portion of sclera in left eye and so forth. The diagnosis was confirmed by clinical findings and histopathologic features of tissue biopsy. The histopathology from periccular area of left eye had the typical findings of Wegener's granulomatosis with necrotizing vasculitis and granuloma formation. We had treated with oral cyclophosphamide(30~120mg/day) and corticosteroid(betamethaEcne 3mg 1M) and performed the enucleation of left eye due to widespread necrosis. The general condition and ocular findings were moderately improved with medical treatment but the eventual visual acuity was zero in right eye.
Biopsy ; Cornea ; Diagnosis ; Exophthalmos ; Granuloma ; Humans ; Kidney ; Necrosis ; Orbit ; Respiratory System ; Rhinitis ; Sclera ; Sinusitis ; Vasculitis ; Visual Acuity ; Wegener Granulomatosis*

Wegener's granulomatosis is characterized by necrotizing vasculitis and granuloma formation in both upper and lower respiratory tracts and in kidney. Other organs are also involved frequently. Ocular manifestations are Ehown in about 50% of patients with it. We experienced a case of Wegener's granulomatcsis developed prcgressive paranasal sinusitis and rhinitis combined with orbital and ocular involvement shown proptosis of both eyes, necrotic change of cornea and anterior portion of sclera in left eye and so forth. The diagnosis was confirmed by clinical findings and histopathologic features of tissue biopsy. The histopathology from periccular area of left eye had the typical findings of Wegener's granulomatosis with necrotizing vasculitis and granuloma formation. We had treated with oral cyclophosphamide(30~120mg/day) and corticosteroid(betamethaEcne 3mg 1M) and performed the enucleation of left eye due to widespread necrosis. The general condition and ocular findings were moderately improved with medical treatment but the eventual visual acuity was zero in right eye.
Biopsy ; Cornea ; Diagnosis ; Exophthalmos ; Granuloma ; Humans ; Kidney ; Necrosis ; Orbit ; Respiratory System ; Rhinitis ; Sclera ; Sinusitis ; Vasculitis ; Visual Acuity ; Wegener Granulomatosis*

Wegener's granulomatosis is a necrotizing and granulomatous vasculitis which involves upper and lower respiratory tract, kidney and skin. The patient who have the protracted Wegener's granulomatosis live a long peroid without major organ imvolvement, In mild cases, the progression is slow and may not need systemic cyclophosphamide treatment. Some cases, however, demonstrate renal involvement and may result in renal failure and death if adequate treatment is not provided. We experienced a case of protracted Wegener's granulomatosis that was a new concept and has not been reported !n Korea. The diagnosis was confirmed by clinical finding and histopathologic features of tiasue biopsy. This case represents a protracted Wegener's granulomatosis with paranasal sinusitis, rhinitis and skin purpura and progress to a gener alized form with kidney involvement after 6years. Treatment with oral cyclophosphamide, steroid and sulfamethoxazole-trimethoprime result improvement of skin, nasal symptom and labratory parameters.
Biopsy ; Cyclophosphamide ; Diagnosis ; Humans ; Kidney ; Korea ; Purpura ; Renal Insufficiency ; Respiratory System ; Rhinitis ; Sinusitis ; Skin ; Vasculitis ; Wegener Granulomatosis