Eosinophilia in Wegener's granulomatosis

Significant eosinophilia is a prominent feature in Churg-Strauss syndrome but has only rarely been described in Wegener's granulomatosis (WG). We describe two Wegener's granulomatosis patients with > 30% eosinophilia on their initial presentations. Other etiologies that could account for their eosinophilia were excluded. Both patients had pulmonary alveolar hemorrhage, sinusitis, arthritis, high-titer cytoplasmic antineutrophil cytoplasmic antibodies (cANCA), and proteinase-3 antibodies, but no evidence of renal disease. Herein we discuss eosinophilia, the differential diagnosis of pulmonary infiltrates and eosinophilia, the role of cANCA in vasculitis and autoimmune disease, compare Wegener's granulomatosis and Churg-Strauss syndrome, and review possible pathogenic mechanisms.     

Etoposide in Wegener's granulomatosis

SIR, Further to the letter of Papo et al. [1], we would liketo report on five more patients with Wegener's granulomatosis(WG) who have been treated with etoposide. The indications wereas follows: (1) induction of remission in severe cyclophosphamide-and steroid-resistant disease (patients 1 and 2); (2) inductionof remission in patients with severe disease who were intolerantof cyclophosphamide (patients 3, 4 and 5). Patient 1 is a 28-yr-old lady who presented to our unit witha severe flare of scleritis and sinusitis whilst taking azathioprine.WG had been diagnosed 8 yr earlier, with an average prednisolonedose during     

Wegener's granulomatosis in pregnancy

This report describes a case of severe limited Wegener's granulomatosis (WG) presenting in the third trimester of pregnancy with pansinusitis and necrotizing pneumonitis. The patient was treated successfully with a combination of corticosteroids and cyclo-phosphamide (CYC). The outcomes in the mother and the newborn were excellent. In a review of the English-language literature, we found 10 similar cases of WG with 13 pregnancies. WG occurring during pregnancy may have a more aggressive course and may require more aggressive treatment compared with WG occurring at other times. The treatment options for WG in pregnancy are discussed.     

Treatment of Wegener's granulomatosis

Treatment of Wegener's granulomatosis, often a life-threatening disease, has greatly improved, considering that before corticosteroids and immunosuppressives were available, the average survival time of patients amounted to no more than 5 months. The management of Wegener's granulomatosis can be divided in two stages: induction of remission and maintenance of remission. The standard regimen for the induction of remission consists of cyclophosphamide, 2 mg/kg/day orally, in combination with prednisone, 1 mg/Kg/day orally, with a gradual tapering once remission has been obtained. To lower the overall cumulative dose, monthly intravenous pulses of cyclophosphamide have been evaluated. Other alternative treatments as high doses of corticosteroids, methotrexate, or plasmapheresis have been proposed, together as prophylaxis with trimethoprim-sulfamethoxazole. To minimize toxicity, for maintenance therapy other drugs are also used such as methotrexate, azathioprine, cyclosporine. Frequent therapeutic changes are needed due to the great variability of the disease; while important aspects are the recognition and treatment of relapse, and include not only the management of resistant disease, but also some particular aspects such as disease in chronic dialysis, renal transplant, pregnancy. Other cytotoxic drugs like leflunomide or mycofenolic mofetil appear to be promising, while new efforts to identify more effective and less toxic therapies include biologic products, such as high-dose immunoglobulin, TNF antagonists and other monoclonal antibodies. Many different kind of clinical trials are going on to better evaluate the real efficacy and safety of these treatments in Wegener's granulomatosis.     

Therapeutics of Wegener's granulomatosis

The treatment of Wegener's granulomatosis, one of the most common forms of systemic vasculitis, has changed substantially over the past two decades. The principal aims of therapy are to control the disease swiftly, to limit the extent and severity of permanent organ damage, and to minimize the short-term and long-term morbidities that often result from therapy. This review provides an overview of the treatment regimens that are currently available for inducing and maintaining remission in patients with Wegener's granulomatosis, and also discusses newer agents that might have a role in the management of this disease in the future. Severe toxicity associated with the available agents and, therefore, there is keen interest in the development of alternative treatment strategies for this disease.     

Wegener's granulomatosis in the elderly

Wegener's granulomatosis is a systemic necrotizing granulomatous vasculitis in which the average age at onset is in the forties. The disease has an excellent response to treatment with cyclophosphamide. We proposed that the tendency to accept multisystem disease in elderly patients (greater than or equal to 60 years old) and a reluctance to administer aggressive therapy would be reflected in the diagnosis and treatment of Wegener's granulomatosis in the elderly, and that as a consequence, the delay in diagnosis and implementation of therapy could result in a needlessly higher morbidity and mortality rate for this population of patients.