| aspergillus fumigatus is present in the environment worldwide and it is only able to infect debilitated or immunodepressed subjects. Nosocomial outbreaks of A. fumigatus infection have been associated with hospital reconstruction. spores are released into the environment and are inhaled by immunodepressed patients housed in nearby Medical Units. Specific clinical syndromes are allergic bronchopulmonary aspergillosis and invasive pulmonary aspergillosis with characteristic radiological features. Invasive A. fumigatus infection is commonly fatal, even if promptly diagnosed and treated. Three consecutive cases of A. fumigatus infection occurred in debilitated patients housed in our Renal Unit while building renovation near the Unit was being performed. Two of these patients died and pulmonary and diffuse aspergillosis was found on postmortem examination. The third patient, highly suspected to be infected with Aspergillus, was aggressively and successfully treated with liposomal amphotericin b. Our experience suggests that fungal infections have gained increasing prominence in clinical medicine and they must be considered in chronic debilitated patients including dialysis patients, and that liposomal amphotericin b represents an important advance in the treatment of aspergillosis. ( info) |
692/784. sulfasalazine pulmonary toxicity in ulcerative colitis mimicking clinical features of Wegener's granulomatosis. The centrally accentuated antineutrophil cytoplasmic antibody test (c-ANCA) is widely regarded as a sensitive and specific marker for Wegener's granulomatosis (WG). There are increasing reports, however, of false-positive c-ANCAs, usually in the setting of other vasculidities. We report a case of a 27-year-old man with ulcerative colitis who developed pulmonary symptoms, peripheral nodular lung infiltrates, and an elevated c-ANCA suggesting WG. Chest CT and open lung biopsy specimens were consistent with WG. The symptoms and pulmonary infiltrates resolved after discontinuation of sulfasalazine therapy. The c-ANCA remained elevated due to the occurrence of false-positive values in ulcerative colitis. We conclude sulfasalazine toxicity can mimic clinical aspects of WG and that c-ANCA testing should be interpreted with caution in patients with ulcerative colitis. ( info) |
693/784. Subretinal tumour in a patient with a limited form of Wegener's granulomatosis. In 1939 F. Wegener published a report concerning a peculiar febrile syndrome with necrotizing inflammation of the upper respiratory tract, focal glomerulonephritis and systemic angiitis (Wegener 1939). Clinically Wegener's granulomatosis can be divided into a limited and a generalized form. We describe a patient presenting with unilateral ocular symptoms suspicious of a neoplasm, one episode of angina pectoris and general malaise without any clinical signs of upper respiratory tract involvement. Before Wegener's granulomatosis was diagnosed by a positive titre of antineutrophil cytoplasmic antibodies and open lung biopsy, immunoscintigraphy for malignant melanoma showed a false positive result. Systemic treatment with corticosteroids and cyclophosphamide resulted in an improvement of the ocular symptoms within weeks. ( info) |
694/784. Wegener's granulomatosis presenting as major salivary gland enlargement. Salivary gland involvement is rare in Wegener's granulomatosis. We report the first case of widespread major salivary gland enlargement as part of the presentation of this disease. A review of the few reported cases in the literature suggests that salivary gland involvement may be associated with a limited form of the disease and an improved prognosis. The anti-neutrophil cytoplasmic antibody (c-ANCA) assay remains the gold standard of diagnosis but care should be exercised in the interpretation of results. This patient responded well to current immunosuppressive therapy. ( info) |
695/784. early diagnosis of Wegener's granulomatosis presenting with facial nerve palsy. Wegener's granulomatosis is a multi-system disease characterized by granuloma formation and a necrotizing vasculitis. It classically presents with involvement of the upper and lower respiratory and renal systems. However locoregional disease is common and may include otological manifestations. facial nerve palsy has been reported during the course of the disease process but it is extremely rare for it to be the presenting feature. Previously reported cases have involved a protracted diagnostic process including exploratory tympanotomy, mastoidectomy and facial nerve decompression. We report a case of Wegener's granulomatosis which presented with a facial nerve palsy. An early diagnosis was achieved by measurement of the erythrocyte sedimentation rate (ESR), followed by serological assay of cytoplasmic anti-neutrophil cytoplasmic antibody (cANCA). Histological examination of nasal mucosal biopsies confirmed granuloma formation consistent with a diagnosis of Wegener's granulomatosis. This allowed early treatment with immunosuppressive therapy and avoided unnecessary and potentially hazardous middle ear surgery. ( info) |
696/784. Wegener's granulomatosis with multiple cranial nerve involvements as the initial clinical manifestations. Wegener's granulomatosis (WG) is a disease of unknown origin characterized by necrotizing granulomas of both the upper and lower respiratory tracts and glomerulonephritis. A 62-year-old woman presenting unusual neurological manifestations is reported. The patient suffered from palsies of multiple cranial nerves without manifestation of respiratory tracts in the initial clinical course. Seven years after the onset, pulmonary consolidations appeared on the chest X-ray study. A diagnosis was made by a needle biopsy of the lung. Palsies of cranial nerves suspected to be due to meningeal involvement of WG. During the entire clinical course, no finding of glomerulonephritis was observed. ( info) |
697/784. Relapsing Wegener's granulomatosis: successful treatment with cyclosporin-A. We describe the case of a 32-year-old splenectomised man with severe Wegener's granulomatosis which was refractory to conventional treatment with oral cyclophosphamide and prednisolone. Remission was temporarily induced only with plasma exchange or i.v. immunoglobulin. Because of frequent relapses of the disease and cyclophosphamide side effects, he was started on treatment with cyclosporin-A and a long lasting remission was achieved. ( info) |
698/784. Wegener's granulomatosis overlapped with takayasu arteritis. A 27-year-old woman presented with destructive scleritis of the left eye with subcutaneous haemorrhage and swelling of the lower eyelid. She had experienced recurrent nasal bleeding for the last six years, and chronic sinusitis resulting in the destruction of the nasal septum and left maxillary sinus. Nasal mucosal biopsies demonstrated granuloma formation with no evidence of vasculitis. The level of serum cytoplasmic anti-neutrophil cytoplasm antibody (c-ANCA) was significantly increased. Furthermore, multiple sites of complete occlusions were detected in the left subclavian and common carotid arteries by subtraction angiography and MRI. We describe a case of Wegener's granulomatosis overlapped with takayasu arteritis, which, to our knowledge, has not been previously reported. ( info) |
699/784. Wegener's granulomatosis: a case report and literature review. Wegener's granulomatosis may present with a variety of findings and be difficult to diagnose. We report a case of 27-year-old man presenting with chronic cough and hemoptysis who was diagnosed by open lung biopsy to have Wegener's granulomatosis. Based on this case, we reviewed the clinical features, pathophysiology, diagnosis, treatment, and prognosis of Wegener's granulomatosis. ( info) |
| wegener granulomatosis traditionally has been treated with glucocorticoids and cyclophosphamide. Both the disease and its treatments are associated with significant morbidity and mortality rates. There has been an effort to find effective but less toxic alternative treatments. We describe three children with wegener granulomatosis who responded well to treatment with glucocorticoids and methotrexate, similar to a regimen used in adults. ( info) |