Manifestation Of Wegener's Granulomatosis Research Articles

Thoracic manifestation of Wegener's granulomatosis: Computed tomography findings and analysis of misdiagnosis.

The aim of the present study was to investigate the computed tomography (CT) manifestations of Wegener granulomatosis (WG) in the chest and potential reasons for misdiagnosis. Conventional CT scans and clinical data of 45 patients with WG were retrospectively analyzed. Patients typically presented with multiple system involvement, primarily in the upper and lower respiratory tract. The incidence of thoracic involvement was 75.56% (34/45). Radiographic features were varied between cases in the present study, with the most common features being numerous cavitary nodules and masses in the lungs. Cavitations were usually irregular, with uneven wall thickness, partial centrality, fuzzy inner edges and piecemeal necrosis. These results indicate that WG typically has multiple system involvement, with the chest being most prominent. Multiple variable-sized cavitary nodules with irregular edges and piecemeal necrosis were the most notable features revealed using CT scanning; however, in order to give a definitive diagnosis, biopsies should be performed.

Офтальмологические проявления гранулематоза Вегенера (клиническое наблюдение)

Офтальмологические проявления гранулематоза Вегенера (клиническое наблюдение)

Aortitis with aneurysm formation as a rare complication of Wegener's granulomatosis

Wegener's granulomatosis (WG) is a systemic vasculitis of medium-sized and small blood vessels. Aortic involvement in WG is very uncommon. We present a 43-year-old patient with an aortitis with aneurysm formation as a manifestation of WG. The patient was operated on and an aortoiliac Dacron inlay graft was inserted. Postoperatively, he recovered uneventfully. Abdominal pain occurring during a WG flare may result from vasculitis of large abdominal arteries with or without aneurysmatic changes for which surgical treatment and immunosuppressive agents are indicated to prevent a possible rupture.

Renal & Ocular Targets for Therapy in Wegeners Granulomatosis

Wegener's granulomatosis (WG) is a multisystem small-vessel vasculitis which is characterised by granulomatous inflammation. Respiratory tract involvement is most commonly seen, affecting up to 85% of patients, closely followed by the renal system in up to 75% of patients; ocular involvement in WG is estimated to occur in 50-60% of patients. The purpose of this review is to provide an overview of the renal and ocular manifestations of WG and discuss the rationale behind the therapeutic approach. In particular, we will focus on how understanding the disease processes in both of these organs has led to more targeted therapy. The mechanism of action of the various immunosuppressive medications in both systemic and ocular inflammation and the evidence available for their use will also be discussed.

Wegener's granulomatosis and posterior ischemic optic neuropathy: Atypical associated conditions

We report the atypical course of two patients with posterior ischemic optic neuropathy (PION) and Wegener's granulomatosis (WG). The first case suggests that the optic nerve could be the primary and unique localization of WG. The second case suggests retrobulbar neuritis as another manifestation of WG, secondary to the autoimmune diathesis underlying this disease.

Pulmonary Manifestations of Wegener Granulomatosis: CT Findings in 57 Patients and a Review of the Literature

Pulmonary Manifestations of Wegener Granulomatosis: CT Findings in 57 Patients and a Review of the Literature

Wegener's granulomatosis: A challenging disease for otorhinolaryngologists

ConclusionsDiagnosis of Wegener's granulomatosis (WG) can be delayed because of its aspecific presenting symptoms. Detection of serum circulating antineutrophil cytoplasm antibodies (c-ANCAs), in combination with histology, permits one to identify WG at an early stage and to implement stage-adapted therapy. c-ANCA levels may also help to evaluate the response to medical therapy. Recently, the quality of life of WG patients has been improved by administering cotrimoxazole in order to prevent infections and recurrent diseases during the remission period.ObjectiveWG is of special significance to the otorhinolaryngologist because it is often initially limited to the upper respiratory tract before becoming systemic. The aim of this paper was to describe a series of WG patients and underline the difficulties involved in diagnosing and treating this challenging disease.Material and methodsThis was a prospective study in 23 consecutive patients with head and neck manifestations of WG (17 systemic, 6 limited). Diagnosis was performed by means of both c-ANCAs detection using indirect immunofluorescence and histology in biopsy specimens. Treatment consisted of daily cyclophosphamide (CYC; 2 mg/kg/day) and glucocorticoids (prednisone; 1 mg/kg/day). If an improvement or toxic events occurred, CYC was discontinued and methotrexate was started. If, during remission of the disease, low serum c-ANCAs levels were detected, CYC was suspended and cotrimoxazole (1 g/day) was introduced.ResultsSerum c-ANCAs detection was positive for all patients. Biopsy was diagnostic from the beginning in 19/23 cases. The six patients with limited WG did not show a progression to systemic disease. Only 3 patients with a diagnosis of delayed systemic WG died, whereas 19/23 patients were alive with good control of relapses.

ANCA in ocular inflammatory disorders.

400 patients presenting with active inflammation of the conjunctiva, sclera, orbita, uvea or retina were cANCA negative during active disease, however, pANCA were found in 15%. Another group of 100 patients suffering from Wegener's granulomatosis (WG) showed in 72 cases one or multiple ocular inflammatory symptoms. They were frequently cANCA-positive. Contrary to some other reports dealing with ocular inflammation, we find a high specificity of cANCA for Wegener's granulomatosis and, therefore, recommend cANCA-testing in all ocular inflammatory disorders, which may be difficult to differentiate from a manifestation of WG.