Wegener Granulomatosis: A Case Report and Update
Autor: | Shahnila Latif, M. M. Ahmed, Maureen Heldmann, Seth Mark Berney, E. Mubashir, Samina Hayat |
---|---|
Rok vydání: | 2006 |
Předmět: |
Male
medicine.medical_specialty Systemic disease Cyclophosphamide Drug Administration Schedule Antibodies Antineutrophil Cytoplasmic Pregnancy Recurrence Prednisone medicine Humans Glucocorticoids Tumor Necrosis Factor-alpha Vascular disease Mononeuritis Multiplex business.industry Granulomatosis with Polyangiitis Plasmapheresis General Medicine Middle Aged medicine.disease Dermatology Surgery Pregnancy Complications Kidney Failure Chronic Female Pulmonary hemorrhage Headaches medicine.symptom Vasculitis business Immunosuppressive Agents medicine.drug |
Zdroj: | Southern Medical Journal. 99:977-988 |
ISSN: | 0038-4348 |
Popis: | Wegener granulomatosis (WG) is a systemic disease of unknown etiology characterized by necrotizing granulomatous inflammation, tissue necrosis, and variable degrees of vasculitis in small and medium-sized blood vessels. The classic clinical pattern is a triad involving the upper airways, lungs and kidneys. Ninety percent of patients present with symptoms involving the upper and/or lower airways, and 80% will eventually develop renal disease. WG should be suspected in any patient with progressive or unresponsive sinus disease, glomerulonephritis, pulmonary hemorrhage, mononeuritis multiplex or unexplained multisystem disease. Before the routine use of glucocorticoids and cyclophosphamide, the one year mortality was 82%. However in 1973, Fauci and Wolf discovered that daily prednisone and cyclophosphamide induced complete remission in 75% of patients. The continued use of prednisone and cyclophosphamide for 1 year past remission leads to marked improvement in more than 90% of patients; however, is also associated with serious toxicities. Depending on the disease severity, current treatments employ induction with short-term cyclophosphamide followed by less toxic agents such as methotrexate to maintain disease remission. Although it is a rare disorder, it is pertinent to internists because it is a multisystem disease that presents in a variety of ways. We describe a 63-year-old white male with WG who presented with progressively worsening headaches, bilateral eye redness, epistaxis, hemoptysis and an unintentional 20 pound weight loss, and review the current treatment recommendations. |
Databáze: | OpenAIRE |
Externí odkaz: |