Cases reported "Wegener Granulomatosis"

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1/37. Orbital wegener granulomatosis without systemic findings.

    PURPOSE: To describe a case of orbital wegener granulomatosis without systemic disease. METHOD: Case report. RESULTS: A 69-year-old patient with bilateral inflammatory lacrimal gland masses underwent multiple biopsies that showed a nonspecific lymphoplasmacytic infiltrate consistent with orbital pseudotumor. After unsuccessful treatment with systemic corticosteroids and radiation, severe orbital disease rapidly progressed and the patient underwent unilateral enucleation. The enucleated specimen showed multifocal vasculitis, tissue necrosis, and granulomas consistent with wegener granulomatosis (WG). Elevated antineutrophil cytoplasmic antibody titers supported the diagnosis of WG. The patient did not have any extraocular signs of WG and continues to be disease-free systemically. CONCLUSION: The authors believe this is the first report of bilateral lacrimal gland masses presenting as a localized form of WG in the total absence of systemic disease.
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2/37. Granulomatous hypophysitis due to Wegener's granulomatosis.

    We describe the MR image findings in a case of granulomatous hypophysitis due to Wegner's granulomatosis. A high index suspicion of hypophysitis based on imaging findings allowed successful medical management and helped avoid surgery. The MR imaging features included a thickened stalk, a diffusely and uniformly enlarged gland, a normal size or minimally enlarged sella, and enhancement of the optic chiasm.
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3/37. diabetes insipidus and anterior pituitary insufficiency as presenting features of Wegener's granulomatosis.

    Central diabetes insipidus (DI) is a rare complication of Wegener's granulomatosis (WG), which usually presents after pulmonary or kidney involvement. Anterior pituitary dysfunction secondary to WG has been extremely rare, documented in only three cases. We report a case of a 47-year-old postmenopausal woman who was diagnosed with hypopituitarism in November 1999 and started on vasopressin, thyroxine, and hydrocortisone. She sought treatment at the Mayo Clinic in February 2000 with a purpuric rash, fever, cough, shortness of breath, and blood in the sputum. Computed tomography of the chest showed a 6-cm irregular mass in the right lower lobe, and a biopsy of the mass showed marked reactive atypia and necrosis. Positive C-antineutrophil cytoplasmic antibodies (ANCA) and skin biopsy of a purpuric lesion showing leukocytoclastic vasculitis confirmed the diagnosis of WG. Hormonal studies showed low gonadotropins, thyroid-stimulating hormone (TSH), and prolactin. magnetic resonance imaging (MRI) of the head showed cystic enlargement of the pituitary gland that did not enhance with gadolinium. Two months into the treatment with cyclophosphamide and prednisone, she had persistent pituitary dysfunction, despite the normal appearance of the pituitary gland on repeat MRI. We conclude that WG should be included in the differential diagnosis of DI and anterior pituitary dysfunction in the proper clinical setting. early diagnosis and treatment may be crucial in preventing pituitary gland destruction and long-term endocrine sequelae. We suggest screening for anterior pituitary failure in the presence of the WG-associated DI.
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4/37. Salivary gland involvement as an initial presentation of Wegener's disease. A case report.

    The case is presented of a 47-year-old woman with an 8-week history of persistent right ear discomfort and a 2-week history of unilateral parotid swelling, as well as peripheral paresis of the facial nerve. The case points to the difficulties encountered when Wegener's granulomatosis presents in an unusual and varied way which mimics a malignant tumour of the parotid gland. To our knowledge, the association of parotid gland involvement and facial palsy in Wegener's granulomatosis has not previously been described in the literature.
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5/37. sublingual gland involvement in systemic Wegener's granulomatosis: a case report.

    Wegener's granulomatosis is a systemic vasculitis characterized by necrosis, granulomatosis and inflammation. The usual targets are extra-oral, although salivary gland involvement has been recognized, usually confined to parotid and submandibular glands. A case report is presented of sublingual gland presentation, confirmed on biopsy, which proceeded to systemic involvement. Some evidence suggests that salivary gland involvement, if recognized, may lead to an early diagnosis and the potential for earlier therapeutic intervention.
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6/37. Dacryoadenitis as the earliest presenting manifestation of systemic Wegener's granulomatosis.

    PURPOSE: To report a case presenting with dacryoadenitis as the earliest manifestation of systemic Wegener's granulomatosis (WG). DESIGN: Observational case report. methods: A 41-year-old woman initially presented symptoms of bilateral dacryoadenitis. She subsequently developed upper and lower respiratory tract involvement, scleritis and keratitis. RESULTS: Cytoplasmic antineutrophil antibody (c-ANCA) titer was positive. The lacrimal gland and lung biopsies were consistent with WG. The patient responded well to cyclophosphamide and prednisolone. CONCLUSIONS: Dacryoadenitis maybe the earliestpresenting manifestation of WG andprompt immunosuppressive chemotherapy may control it preventing the limited disease from progressing to a complete form and reducing its morbidity and mortality.
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7/37. Bilateral parotid and submandibular gland enlargement: rare features of Wegener's granulomatosis.

    Wegener's granulomatosis is a potentially fatal disease of unknown origin affecting mainly the upper and lower respiratory tracts and kidneys. Prompt recognition of the more unusual presentations of the disease is necessary to ensure early treatment. We present a case of a 46-year-old female with bilateral submandibular and parotid gland enlargement. Parotid or submandibular salivary gland enlargement is a rare presenting feature of Wegener's granulomatosis. Common to 80 per cent of these cases is nasal involvement, while ear pathology or lung lesions may occur in the remaining cases. The diagnosis is both clinical and pathological, biopsy of suspicious tissue, serum c-ANCA levels and a chest X-ray are valuable investigations. Treatment with immunosuppressive therapy is essential, and usually ensures a long-term remission.
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8/37. Wegener's granulomatosis with parotid gland involvement and pneumothorax.

    OBJECTIVE: Wegener's granulomatosis is a systemic vasculitis characterized by necrotizing granulomatous lesions mostly involving the upper and lower respiratory tract. The disease rarely causes parotid gland involvement and pneumothorax. We report a case of Wegener's granulomatosis involving parotid gland, and complicated with a pneumothorax. CLINICAL PRESENTATION: A 45-year-old man admitted with a 3-week history of painful left parotid gland enlargement and hemoptysis. On physical examination a painful and hard mass was detected on the left pre-auricular area. Cervical CT revealed a 2 x 1.5 cm hypodense lesion mimicking an abscess on the left parotid gland. Chest radiograph and thorax CT demonstrated nodular and cavitating opacities on the right and left upper zones. There were numerous erythrocytes in urine sediment. The drained pus material from the parotid abscess demonstrated only gram-positive cocci (staphylococcus aureus). Two weeks treatment with teicoplanin resulted in no improvement. Meanwhile, parotid gland biopsy revealed necrotizing granulomatous inflammation. There was a sixfold increase in serum cANCA levels. With the diagnosis of Wegener's granulomatosis, cyclophosphamide and prednisolone were initiated. However, 1 month later, pneumothorax developed as a complication of rupture of a cavitary lesion. CONCLUSION: parotid gland swelling may be the initial presenting symptom of Wegener's granulomatosis. It can be confused with infectious or malignant diseases of the gland, and the lung involvement may be complicated with pneumothorax.
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9/37. Wegener's granulomatosis presenting with otologic and neurologic symptoms: clinical and pathological correlations.

    A case of Wegener's granulomatosis in a 59-year-old woman is reported. The disease first involved the parotid gland, the brain stem and the spinal cord, then running a rapidly progressive course as systemic pathology. Anti-neutrophil cytoplasmic antibody (cANCA) levels raised as renal failure set in. Renal biopsy confirmed the diagnosis of Wegener's granulomatosis and an appropriate therapy was adopted.
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10/37. Wegener's granulomatosis of the prostate gland.

    Wegener's granulomatosis (WG) is a systemic granulomatous vasculitis affecting medium and small arteries, venules, and arterioles. The upper and lower respiratory tract and kidney are primarily involved. patients with classic WG essentially present with upper airway and pulmonary involvement. Renal disease is common. Involvement of other organ systems is also relatively frequent, most often heart, joints, muscles, eyes, skin, and central and/or peripheral nervous system. We present a patient in whom WG was diagnosed primarily because of prostate involvement. This seems to be a rare manifestation.
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