Abstract
Granulomatosis with Polyangiitis (GPA), formerly known as Wegener’s Granulomatosis, is a multisystem disease that predominantly involves the respiratory tract and the kidneys. The pathological hallmarks are necrotizing granulomatous lesions and vasculitis of small and/or medium vessels. It is difficult to diagnose because its presentation is similar to many common diseases such as upper respiratory tract infections, pneumonia, and asthma. Understanding the signs and symptoms, laboratory findings, and biopsy results are key to making the diagnosis of Granulomatosis with Polyangiitis. A heightened degree of clinical suspicion for this rare disease will often lead to the correct diagnosis.
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References
Leavitt RY, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum. 1990;33(8):1101–7.
Savige J, et al. Antineutrophil cytoplasmic antibodies and associated diseases: a review of the clinical and laboratory features. Kidney Int. 2000;57(3):846–62.
Jennings C, Jones N, Dugar J, et al. Wegener’s granulomatosis—a review of diagnosis and treatment in 53 subjects. Rhinology. 1998;36:189–91.
Hoffman GS, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992;116(6):488.
Lohrmann C, et al. Pulmonary manifestations of Wegener granulomatosis: CT findings in 57 patients and a review of the literature. Eur J Radiol. 2005;53(3):471–7.
Jeannette C, Falk R, Andrassy K, et al. Nomenclature of systemic vasculitides. Arthritis Rheum. 1994;37(2):187–92.
Rao JK, Weinberger M. The role of antineutrophil cytoplasmic antibody (c-ANCA). Ann Intern Med. 1995;123(12):925–932.
Matthay R, bromberg S, Putman C. Pulmonary renal syndromes—a review. Yale J Biol Med. 1980;53(6):497–523.
Reinhold-Keller E, et al. An interdisciplinary approach to the care of patients with Wegener’s granulomatosis: long-term outcome in 155 patients. Arthritis Rheum. 2000;43(5):1021–32.
Groot K, Dwomoa A, Savage O. The value of pulse cychophosphamide in ANCA-associated vasculitis: meta analysis and critical review. Nephrol Dial Transplant. 2001;16(1):2018–27.
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Shenoy, K.V., Sloane, J., D’Alonzo, G. (2013). Wegener’s Granulomatosis. In: Mahmoudi, M. (eds) Challenging Cases in Rheumatology and Diseases of the Immune System. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-5088-7_11
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DOI: https://doi.org/10.1007/978-1-4614-5088-7_11
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