Inflammation of small and medium blood vessels characterizes Wegener's granulomatosis, which is one type of autoimmune vasculitis. Dr. Julia U. Holle of the University Hospital in Luebeck, Germany, and her collaborators reported that improvements in treatment over the last four decades have produced better outcomes for patients with this condition.
Increased awareness of Wegener's granulomatosis is one factor identified by Holle as significant for the improved prognosis. Earlier diagnosis and therapy contribute to reduction in relapse rates, lower cumulative doses of cyclophosphamide, and less mortality. The standardized mortality ratio for patients diagnosed between 1966 and 1993 was 2.1, which means that these patients had more than twice as many deaths as age-matched individuals in the general population. Patients diagnosed between 1999 and 2002 had a standardized mortality ratio of 1.03, which means that they had only a 3 percent increased risk of death.
The respiratory system and kidneys are the most common sites of damage. Holle reported that 10 to 45 percent of patients also experience damage to the central or peripheral nervous system, and these cases in particular require early diagnosis and treatment. She cited rituximab and infliximab as treatment options.
Dr. James M. Kelley wrote a clinical vignette of the typical Wegener's granulomatosis patient. Caucasians aged 45 to 65 are at highest risk. Some studies report no gender bias, but others report more male than female cases, so Kelley chose a man in his late 50's as the composite patient. He had joint pain, sinus problems with nosebleeds, and hearing loss, which all started about 18 months before his first visit to a doctor. He thought he just had allergies, since his eyes also turned red easily. The symptoms lasted several months, then improved, and then got worse again. His doctor noticed lesions on his elbow immediately. Further examination showed oral lesions as well. Routine lab work showed evidence of kidney damage. A chest X-ray and tests for autoimmune factors confirmed the diagnosis of Wegener's.
Rheumatologists are the specialists most familiar with Wegener's granulomatosis, because of its autoimmune mechanism.
References:
1. Holle JU et al, “Improved outcome in 445 patients with Wegener's granulomatosis in a German vasculitis center over four decades”, Arthritis Rheum. 2011 Jan; 63(1): 257-66.
2. Holle JU et al, “Neurological involvement in Wegener's granulomatosis”, Curr Opin Rheumatol. 2011 Jan; 23(1): 7-11.
3. Kelley JM et al, “Wegener's granulomatosis: A model of auto-antibodies in mucosal autoimmunity”, Clin Immunol. 2010 Feb; 134(2): 104.
Linda Fugate is a scientist and writer in Austin, Texas. She has a Ph.D. in Physics and an M.S. in Macromolecular Science and Engineering. Her background includes academic and industrial research in materials science. She currently writes song lyrics and health articles.