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Wegener’s granulomatosis is a form of vasculitis that affects the lungs, kidneys and other organs. Due to its end-organ damage, it can be a serious disease that requires long-term immune suppression.[1] It is named after Dr. Friedrich Wegener, who described the disease in 1936.[2]
Wegener’s granulomatosis is part of a larger group of vasculitic syndromes, all of which feature the presence of an abnormal type of circulating antibody termed ANCAs (antineutrophil cytoplasmic antibodies) and affect small and medium-size blood vessels. Apart from Wegener’s, this category includes Churg-Strauss syndrome and microscopic polyangiitis.[1] Although Wegener’s granulomatosis affects small and medium-sized vessels,[3] it is formally classified as one of the small vessel vasculitides in the Chapel Hill system.[4][5]
Initial signs are protean, and diagnosis can be severely delayed due to the nonspecific nature of the symptoms. Rhinitis is generally the first sign in most patients.[1]
Wegener’s granulomatosis is usually only suspected when a patient has had unexplained symptoms for a long period of time. Determination of ANCAs can aid in the diagnosis, but positivity is not conclusive and negative ANCAs are not sufficient to reject the diagnosis. Cytoplasmic staining ANCAs that react with the enzyme proteinase 3 (cANCA) in neutrophils (a type of white blood cell) are associated with Wegener’s.[1]
If the patient has renal failure or cutaneous vasculitis, these are the most logical organs to obtain a biopsy from. Rarely, thoracoscopic lung biopsy is required. On histopathological examination, a biopsy will show leukocytoclastic vasculitis with necrotic changes and granulomatous inflammation (clumps of typically arranged white blood cells) on microscopy. These granulomas are the main reason for the appellation of “Wegener’s granulomatosis,” although it is not an essential feature. Unfortunately, many biopsies can be nonspecific and 50% provide too little information for the diagnosis of Wegener’s.[1]
Differential diagnosis (alternative possible diagnoses) can be extensive. ANCAs can be positive after the use of certain drugs and other forms of vasculitis can present with very similar symptoms. The saddle-nose deformity may also be seen in relapsing polychondritis, cocaine abuse and in congenital syphilis.
In 1990, the American College of Rheumatology accepted classification criteria for Wegener’s. These criteria were not intended for diagnosis, but for inclusion in randomised controlled trials. Two or more positive criteria have a sensitivity of 88.2% and a specificity of 92.0% of describing Wegener’s.[6]
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