1,721,216 research outputs found
Diffuse alveolar hemorrhage in Wegener′s granulomatosis
No full textDiffuse alveolar hemorrhage is a life-threatening though rare manifestation of Wegener′s granulomatosis (WG). An active diagnostic workup, intensive observation, and aggressive immunosuppressive treatment are cornerstones of the management. The treatment modalities available for such complications are pulse cyclophosphamide therapy with steroids. We report here a case of WG with diffuse alveolar hemorrhage as the first manifestation of the disease in life that responded to steroids and cyclophosphamide
A Case of Wegener`s Granulomatosis with Thin Glomerular Basement Membrane Disease
Full text linkWegener`s granulomatosis is a necrotizing, granulomatous vasculitis that involves multiple organs including the upper and lower respiratory tract and the kidney. The kidney initially exhibits focal necrotizing glomerulonephritis, which progresses to crescentic glomerulonephritis in Wegener`s granulomatosis. We experienced a case of Wegener`s granulmatosis which was associated with a thin glomerular basement membrane disease. The patient suffered from nasal stuffiness, recurrent serous otitis media, and tinnitus. Despite antibiotic therapy and ventral tube insertion, symptoms did not improve and hearing difficulty was aggravated. Ulcerative, necrotizing granulomatous inflammations with multinucleated giant cells were seen on nasal biopsy. She had recurrent microscopic hematuria and the renal biopsy findings by light and immunofluorescent microscopy did not reveal any abnormalities but diffuse thinning of the glomerular basement membrane (226nm) was observed by electronmicroscopy. With the above clinical findings and biopsy results, we diagnosed Wegener`s granulmatosis with thin glomerular basement membrane disease. Thin glomerular basement membrane disease, also called benign recurrent hematuria, is characterized by diffuse thinning of the glomerular basement membrane and hematuria. Weekly low-dose methotrexate together with prednisone was used as treatment regimen because nonglomerular microscopic hematuria may be the first sign of cyclophosphamide-induced renal toxicity. With the above combination therapy, she felt well-being sense and her hearing difficulty was also much improved. She has been treated as an outpatient with glucocorticoid.ope
A Case of Wegener`s Granulomatosis with Pulmonary Hemorrhage and a Hemorrhagic Mass in the Brain
No full textWegener`s granulomatosis is a necrotizing, granulomatous vasculitis that involves multiple organs including the upper and lower respiratory tract and the kidney. The anitineutrophilic cytoplasmic antibody is a serologic marker with high sensitivity and specificity, which is useful in diagnosis of the disease and is considered to have a significant role in its pathogenesis.
Wegener`s granulomatosis had been a fatal disease until combination therapy with cyclophosphamide and corticosteroids was introduced, which have improved the clinical course and the survival significantly. Wide spectrum of neurologic involvements such as peripheral and cranial neuritis, myelopathy, and cerebritis in Wegener`s granulomatosis has been reported, but manifestation of mass-like formation in the brain has rarely been reported.
We experienced a case of Wegener`s granulomatosis with pulmonaty hemorrhage, renal failure, and a hemorrhagic mass in the brain. Its diagnosis was based on clinical findings, positive result of ANCA test, and the thoracoscopic lung biopsy. The hemorrhagic mass of the brain was found by magnetic resonance imaging and it is considered to be the result of granulomstous inflammation and vasculitis. With the combination therapy the cerebral lesion was slightly improved but the patient expired due to irreversible renal failure and progressive pulmonary hemorrhage.ope
Wegener′s granulomatosis disease mimicking pulmonary tuberculosis
No full textWegener′s granulomatosis (WG) is an uncommon autoimmune disease with multi-system involvement that manifests as vasculitis, granulomatosis, and necrosis. While its standard form involves the upper and lower respiratory tracts and kidneys, it may essentially involve any organ. We present a case in a young female patient presenting with symptoms of cough with expectoration, fever, dyspnea and chest discomfort, having cavitating lesion in right upper lobe and nodule in left upper lobe with bilateral paranasal sinus involvement on computed tomography. Allergic rhino-sinusitis with pulmonary tuberculosis was suspected, and patient was investigated further. Upon further investigation, renal involvement was detected, and serology revealed cytoplasmic antineutrophil cytoplasmic antibody. WG was suspected and was proven by histopathology of nasal tissue which revealed necrotizing granulomas
Wegener′s granulomatosis presenting as spontaneous pneumothorax in young adult
No full textPulmonary involvement in Wegener′s granulomatosis (WG) usually starts with nonspecific symptoms such as cough, dyspnea, hemoptysis, and pleuritis. Spontaneous pneumothorax as initial presentation is extremely rare. Although its real incidence is unknown, according to different classic series, it ranges between 3 and 5% of the cases. In this case, a 28-year-old male presented with complaints of epistaxis and breathlessness, which was diagnosed as WG with pneumothorax on the basis of chest X-ray and computed tomography (CT) chest and pathological confirmation by high level of serum cytoplasmic antineutrophil cytoplasmic antibody (ANCA)
Chronic meningitis with multiple cranial neuropathies: A rare initial presentation of Wegener′s granulomatosis
No full textWegener′s granulomatosis (WG) is a systemic necrotizing vasculitis that affects the small blood vessels. It mainly affects the upper and lower respiratory tract and kidneys. Central nervous system (CNS) involvement is rare, and has been reported only in about 8% of cases during the course of illness. Initial presentation with neurologic affection, particularly chronic hypertrophic meningitis is very unusual. We report the case of a 34 year old male who presented with chronic hypertrophic meningitis and multiple cranial nerve involvement as the initial manifestation, without respiratory and renal symptoms. This case highlights the difficulties in diagnosing a rare disease with rarer presentation, and at the same time illustrates that Wegener′s granulomatosis should be considered in the differential diagnosis of chronic meningitis
Wegener`s granulomatosis and mucoromycosis: A case study and review of literature
No full textMucormycosis is a fatal invasive infection which mostly involves diabetic or immunosuppressed patients. Early diagnosis, improving immunosuppression, systemic antifungal therapy, and surgical debridement are necessary for successful treatment. In this case study, we represent a known case of Wegener′s granulomatosis (WG), with concomitant sinusal mucormycosis mimicking vasculitic disease relapse, which was successfully treated with surgical debridement, amphotericine, and intravenous immunoglobuline
Wegener´s granulomatosis in a young patient preceded by localized cutaneous manifestations
No full textWegener´s granulomatosis (WG) is a rare, systemic vasculitis involving multiple organs. The clinical presentation is highly diverse, and there is considerable risk of mortality if diagnosis and treatment are delayed. We present a case illustrating that patients with WG may initially present with localized cutaneous symptoms and signs
Wegener′s granulomatosis of urinary tract presenting as bladder outlet obstruction
No full textWe report a rare case of Wegener′s granulomatosis involving the prostate gland in a 45-year-old male who presented with acute urinary retention. Treatment was initiated with oral cyclophosphamide and steroids. The prostate size regressed in four weeks and patient voided well after removal of catheter
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