Cases reported "Wegener Granulomatosis"

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1/8. Relapse of Wegener's granulomatosis. Concerning a case after 20 years of remission.

    Immunosuppressive drugs have transformed the prognosis of systemic Wegener's granulomatosis. Nowadays, the main residual problem is illness relapses, for which management is largely undefined. We describe the case of a patient, aged 47 in 1977. The diagnosis of Wegener's granulomatosis was made when faced with polyarthralgias, cutaneous vasculitis, rhinitis, dyspnea, hemoptysis and global decline of her physical condition. The treatment associated high-dose corticotherapy and intramuscular cyclophosphamide for 1 year. This treatment led to a complete remission. Twenty years later, the patient was hospitalized for reoccurrence of rhinitis, dyspnea and right knee effusion associated with biological inflammatory syndrome, renal insufficiency and antibodies against polymorphonuclear neutrophil cytoplasm, type c-ANCA. Chest CT-scan disclosed parenchymal infiltrates. Wegener relapse was diagnosed and the combination of three methylprednisolone perfusions followed by oral prednisone (1 mg/kg/d) and a monthly bolus of cyclophosphamide led to a new remission. Nevertheless, 4 months after beginning the treatment the patient died from an infectious complication (pneumocystis carinii and aspergillosis). Relapses of Wegener's granulomatosis are frequent and difficult to predict. Moreover, some cases occur very early. The remarkable efficiency of cyclophosphamide to induce remission is however shaded by the high rate of relapse. Other drugs are studied to identify more efficient therapy, able to both induce remission and prevent relapses, but reliable data are still missing to determine the best therapeutic regimen.
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2/8. Recurrent Wegener's granulomatosis with subglottic granulation.

    While following the course of patients with Wegener's granulomatosis (WG) in which it had been possible to achieve a remission, we encountered a patient in whom we observed granuloma formation in the subglottis. A tracheotomy was performed because of the risk of respiratory distress, and after that there was a recrudescence of the patient's WG. During the course of the above, the patient remained cANCA (anti-neutrophil cytoplasmis antibody with cytoplasmic pattern)-negative. When these facts were considered, it appeared that when the lesions are localized and progressing gradually, cANCA positivity does not develop. While cANCA test has gradually become a powerful tool for the diagnosis of WG, a comprehensive physical examination to identify localized lesions, together with biopsy and histopathological examination, should allow the diagnosis of WG.
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3/8. Nontraumatic, nonneoplastic subglottic stenosis.

    During a 20-year period, 12 patients with nontraumatic, nonneoplastic subglottic stenosis were seen at the Mayo Clinic. The etiologic factors were relapsing polychondritis, amyloidosis, sarcoidosis, and Wegener's granulomatosis. Because of the diverse initial presentation of the disease, the clinician should consider that the stenosis is a manifestation of a systemic disorder and carry out an otolaryngologic and physical examination with the appropriate roentgenograms and blood and urine tests. Treatment, if a systemic disease is proved, consists of appropriate medication. Surgery may be necessary, depending on the nature of the lesion. Small strictures may not need to be treated.
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4/8. 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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5/8. Wegener's granulomatosis: parotid involvement and associated pancreatitis with C.T. findings.

    Parotid enlargement is an uncommon physical sign and even less commonly the reason for referral for computerised tomography (CT) as the aetiology is usually self-evident. In cases referred for CT examination Wegener's granulomatosis should be considered, particularly if the pathology appears bilateral. This diagnosis may be confirmed by biopsy and/or supported by Anti-Neutrophil Cytoplasmic Antibody (ANCA) assay. A case of Wegener's granulomatosis with parotid gland involvement and temporally related pancreatitis with pseudocyst formation is reported. This is the second reported case of possible pancreatic involvement in Wegeners granulomatosis and approximately the tenth reported case of salivary gland involvement.
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6/8. Wegener's granulomatosis diagnosed by testicular biopsy.

    Urogenital involvement, other than the kidneys, is extremely rare in Wegener's granulomatosis (WG) and occurs in less than 1% of the cases. When encountered it is confined to prostate, bladder, urethra, cervix, and vagina. Granulomatous infiltration of the testis from WG has not been cited in the medical literature. We report a case of WG in a Hispanic male who presented with sensorineural hearing loss and hemoptysis. He had a pulmonary lesion and a painless right testicular mass, which was found to have necrotizing granulomas on excisional biopsy. This may be the first reported case of WG diagnosed by testicular biopsy as testicular involvement is rare in WG. We believe that the actual incidence of testicular involvement in WG may be higher as genital examination may be ignored during routine physical examinations.
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7/8. Computed tomography of diffuse tracheal stenosis in wegener granulomatosis.

    A patient with wegener granulomatosis presented with diffuse tracheal narrowing and was evaluated using CT and cine-CT. The CT findings included severe laryngeal and tracheal airway narrowing, due to abnormal soft tissue within the laryngeal cartilages and tracheal rings, and enlarged abnormally calcified tracheal cartilages. Computed tomography precisely confirmed the site, level, and submucosal extent of tracheal narrowing, not obtainable on physical or bronchoscopic examination, and assisted in choosing a site for tracheostomy. wegener granulomatosis should be considered in patients with diffuse tracheal narrowing even when the typical histology is not present.
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8/8. lymphomatoid granulomatosis--a condition with affinities to Wegener's granulomatosis and lymphoma.

    A case of lymphomatoid granulomatosis of the lung is described in which the presenting features were a skin eruption and peripheral neuropathy. The onset of the pulmonary symptoms of breathlessness and productive cough was delayed nine months but, when apparent, the extent of the radiological changes contrasted with the mildness of the symptoms and the triviality of the physical signs. biopsy of the affected lung revealed a mixed lymphocytic, plasma cell, and histiocytic infiltrate following a perivascular distribution. This combination of clinical and pathological findings is in every detail that of lymphomatoid granulomatosis as recently identified by Liebow et al. (1972). Additional, previously undescribed, and unexplained findings in this case were persistent hypercalciuria and the presence in three axillary lymph nodes of subcapsular groups of cells resembling those of a benign naevus. This is the first case described in the British literature, and it is important that more cases be reported in order that the prevalence, prognosis, and aetiology of the condition should be further established.
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