title: Wegener granulomatosis: a case report and update.
abstract: 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.
authors.affiliation: Center of Excellence for Arthritis and Rheumatology, Section of Rheumatology, Department of Medicine, Louisiana State University Health Sciences Center, Shreveport, LA, USA.
authors.forename: Eisha
authors.initials: E
authors.lastname: Mubashir
authors.forename: M Mubashir
authors.initials: MM
authors.lastname: Ahmed
authors.forename: Samina
authors.initials: S
authors.lastname: Hayat
authors.forename: Shahnila
authors.initials: S
authors.lastname: Latif
authors.forename: Maureen
authors.initials: M
authors.lastname: Heldmann
authors.forename: Seth Mark
authors.initials: SM
authors.lastname: Berney
mesh_terms: D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D003520:Cyclophosphamide / Q000627:therapeutic use; D004334:Drug Administration Schedule; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D010956:Plasmapheresis; D011241:Prednisone / Q000627:therapeutic use; D011247:Pregnancy; D011248:Pregnancy Complications; D012008:Recurrence; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D014409:Tumor Necrosis Factor-alpha; D003520:Cyclophosphamide; D011241:Prednisone