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

Granulomatosis with polyangiitis (GPA) is an idiopathic vasculitis of medium and small arteries, characterized by necrotizing granulomatous inflammation. GPA typically affects upper and lower respiratory tract with coexisting glomerulonephritis. This disease is generally characterized by antineutrophil cytoplasm antibodies (ANCA), nevertheless, there are rare cases with negative ANCA. GPA affects people at any age, with predominance of the sixth and seventh decade of life. In 80%-95% of the patients the first symptoms of GPA are otorhinolaryngological manifestations of head and neck including nose/sinuses, ears, eyes, larynx/trachea, oral cavity, and salivary glands. Diagnosis of GPA is based on Criteria of the American College of Rheumatology. In clinical practice diagnosis, the presence of distinctive ANCA antibodies and biopsy of affected organ are crucial. GPA must be differentiated from neoplastic, infectious or inflammatory ulcerative lesions of the head and neck. The standard treatment procedure is divided into two essential phases, induction and maintenance. The induction phase is based on combination of systemic corticosteroid and immunosuppressant therapy, whereas the maintenance phase comprises corticosteroids and azathioprine/methotrexate supplementation. Surgical treatment ought to be considered for patients who are not responding to pharmacotherapy.
Adrenal Cortex Hormones ; Antibodies ; Antibodies, Antineutrophil Cytoplasmic ; Arteries ; Biopsy ; Cytoplasm ; Diagnosis ; Drug Therapy ; Ear ; Glomerulonephritis ; Head ; Humans ; Inflammation ; Mouth ; Neck ; Otolaryngology ; Respiratory System ; Rheumatology ; Salivary Glands ; Ulcer ; Vasculitis ; Wegener Granulomatosis

Wegener's granulomatosis (WG) is a disease characterized by a granulomatous necrotizing vasculitis of small vessels. Although any organ systems can be involved, gastrointestinal involvement in WG is notably uncommon. We present a case of 67-year-old man who was admitted with abdominal pain and diarrhea lasting for 3 weeks. Colonoscopy and abdominal CT scan revealed vasculitis and multiple mesenteric lymphadenopathy. Jejunum and mesenteric lymph nodes biopsies confirmed limited form of WG. The present case indicates that WG might involve only gastrointestinal tract and the histological confirmation is important for diagnosis.
Aged ; Anti-Inflammatory Agents/therapeutic use ; Colonoscopy ; Diagnosis, Differential ; Gastroenteritis/complications/*diagnosis ; Humans ; Lymphatic Diseases/complications/*diagnosis/pathology ; Male ; Prednisolone/therapeutic use ; Tomography, X-Ray Computed ; Wegener Granulomatosis/complications/*diagnosis/drug therapy

Wegener's granulomatosis (WG) is a disease characterized by a granulomatous necrotizing vasculitis of small vessels. Although any organ systems can be involved, gastrointestinal involvement in WG is notably uncommon. We present a case of 67-year-old man who was admitted with abdominal pain and diarrhea lasting for 3 weeks. Colonoscopy and abdominal CT scan revealed vasculitis and multiple mesenteric lymphadenopathy. Jejunum and mesenteric lymph nodes biopsies confirmed limited form of WG. The present case indicates that WG might involve only gastrointestinal tract and the histological confirmation is important for diagnosis.
Aged ; Anti-Inflammatory Agents/therapeutic use ; Colonoscopy ; Diagnosis, Differential ; Gastroenteritis/complications/*diagnosis ; Humans ; Lymphatic Diseases/complications/*diagnosis/pathology ; Male ; Prednisolone/therapeutic use ; Tomography, X-Ray Computed ; Wegener Granulomatosis/complications/*diagnosis/drug therapy

Wegener's granulomatosis (WG) is a systemic vasculitis affecting small and medium-sized vessels with granulomatous formation. Though it is known for respiratory tract and kidney involvement, neurologic manifestation has been also reported. Herein we report a patient who suffered pansinusitis with multiple lower cranial nerve palsies but reached remission by immunosuppressant after the diagnosis of WG. A 54-yr-old female visited with headache, hearing difficulty, and progressive bulbar symptoms. She experienced endoscopic sinus surgeries due to refractory sinusitis. Neurologic examination revealed multiple lower cranial nerve palsies. Vasculitic markers showed no abnormality. Nasal biopsy revealed granulomatous inflammation and vasculitis involving small vessels. Given cyclophosphamide and prednisolone, her symptoms were prominently improved. WG should be considered in the patient with multiple cranial nerve palsies, especially those with paranasal sinus disease. Because WG can be lethal if delayed in treatment, prompt immunosuppressant is warranted after the diagnostic tissue biopsy.
Anti-Inflammatory Agents/therapeutic use ; Antibodies, Antineutrophil Cytoplasmic ; Antineoplastic Agents, Alkylating/therapeutic use ; Cranial Nerve Diseases/*diagnosis/drug therapy/radionuclide imaging ; Cyclophosphamide/therapeutic use ; Diagnosis, Differential ; Female ; Humans ; Middle Aged ; Prednisolone/therapeutic use ; Sinusitis/surgery ; Vasculitis ; Wegener Granulomatosis/*diagnosis/drug therapy/radionuclide imaging