Granulomatosis with Polyangiitis (GPA) / Wegner's Granulomatosis: A Summary of Clinical Issues
Abstract
Vasculitis can mimic many diseases; often, there are delays in diagnosis and often ends up in life/organ threatening complications without proper diagnosis and timely treatment. Patients can present with various combinations of constitutional symptoms, vasculitic symptoms, and organ involvements. ANCA tests also have many false positive and false negative results, which adds to the complexity. In addition, Anti-neutrophilic Cytoplasmic Antibody (ANCA) associated Vasculitis (AAV) have different pathological and clinical manifestations from other Vasculitis. There are four types of AAV, including Granulomatosis with Polyangiitis (GPA, previously known as Wegner's Granulomatosis), Microscopic Polyangiitis (MPA), Eosinophilic Granulomatosis with Polyangiitis (E-GPA, previously called as Churg Strauss Vasculitis), and Renal Limited / Organ limited ANCA associated Vasculitis
How to Cite This Article
Hussain A, Javaid MM, Avais J. Granulomatosis with Polyangiitis (GPA) / Wegner's Granulomatosis: A Summary of Clinical Issues. J Pak Soc Intern Med. 2021;2(4):284–292. doi:10.70302/jpsim.v2i4.2164
Conflict of Interest
All authors declare no competing interests.
Funding
No specific funding was received for this research.
Funder information follows the Crossref Funder Registry standard.
References
- Comarmond C, Cacoub P. Granulomatosis with Polyangiitis. Clinical aspects of treatment. Autoimmun Rev. 2014;13(10):1121-5.
- McKinney EF, Willcocks LC, Broecker V, Smith KG. The immunopathology of ANCA-associated Vasculitis. Semin Immunopathol. 2014;36(4):461-78.
- Cartin-Ceba, Peikert T, Specks U. Pathogenesis of ANCA Associated Vasculitis. Curr Rheumatol Rep. 2002;14(4):481-93.
- Lutalo P, Cruz D. Diagnosis and classification of granulomatosis with Polyangiitis (aka Wegner's granulomatosis). J Autoimmun. 2014;48-49(1);94-8.
- Schonermarck U, Lamprecht P, Csernok E, Gross WL. Prevalence and spectrum of rheumatic disease associated with proteinase 3-ANCA and myeloperoxidase-ANCA. Rheumatol. 2001;40(2);178-84
- De Geeter F, Gykiere P. F-FDG PET imaging of GPA-Wegner's syndrome. Hell J Nucl Med. 2016;19(1):53-6.
- Nelson DR, Johnson GB, Cartin-Ceba R, Specks U. Characterization of F-18 FDG PET/CT in GPA. Sarcoidosis Vasc Diffuse Lung Dis. 2016;32(4):342-52.
- Natsuki E, Watts RA, Scott DG. Epidemiology of ANCA Associated Vasculitis. Rheum Dis Clin North Am. 2010;36(5);447-61.
- Yi ES, Colby TV. Wegner's granulomatosis. Semin Daign Pathol. 2001;18(1):34-6.
- Pakalniski MG, Berg AD, Policeni BA, Gentry LR, Sato Y, Moritani T, et al. The many faces of GPA. A review of the head and neck imaging manifestations. AJR. 2015;205(6): W619-29.
- Grindler D, Cannady S, Batra PS. Computed tomography findings in sino-nasal Wegner's granulomatosis. Ann J Rhinol Allergy. 2009;23(5):497-501.
- Laudien M, Lamprecht P, Hedderich J, Holle J, Ambrosch P. Olfactory dysfunction in Wegner’s granulomatosis. Rhinology. 2009;47(3):254-9.
- Wang JC, Leader BA, Crane RA, Koch BL, Smith MM, Ishman SL. GPA presenting as facial palsy in a teenager. Int J Pediatr Otolaryngol. 2018;107(2);160-3.
- Muller K, Lin JH. Orbital granulomatosis with Polyangiitis (Wegner;'s granulomatosis): clinical and pathologic findings. Arch Pathol Lab Med. 2014;138(10);1110-4.
- Schmidt J, Pulido JS, Matteson EL. Ocular manifestations of systemic disease. ANCA associatd vasculitis. Curr Opin Ophthalmol. 2011;22(5):489-90.
- Takazawa T, Ikeda K, Nagaoka T, Hirayama T, Yamamoto T, Yanagihashi M, Tochikubo T, Iwasaki Y. Wegner's granulomatosis associated with optic peri-neuritis. Orbit. 2014;33(1):13-6.
- Perry S, rootman J, White VA. The clinical and pathologic constellation of Wegner's granulomatosis of the orbit. Ophthalmol. 1997;104(4):683-94.
- Tarabishy AB, Schulte M, Papaliodis GN, Hoffman GS. Wegner's granulomatosis; clinical manifestations, differential diagnosis, and management of the ocular and systemic disease. Surv Opthalmol. 2010;55(5):429-44.
- Polychronopoulos VS, Prakash UB, Golbin JM, Edell ES, Specks U. Airway involvement in Wegner's granulomatosis. Rheum Dis Clin North Am. 2007;33(6);755-75.
- Castaner E, Alguersuari A, Gallardo X, Andreu M, Pallardo Y, Mata JM, et al. When to suspect pulmonary vasculitis. Radiologic and clinical clues. Radiographics. 2010;30(1): 33-53.
- Holle JU, Gross WL. Neurological involvement in Wegner's granulomatosis. Curr Opin Rheumatol. 2011;23(1):7-11.
- De Parisot A, Puechal X, Langrand C, Raverot G, Gil H, Perard L, et al. Pituitary involvement in GPA: REPORT OF nine patients and review of the literature. Medicine. 2015:94(16): e748.
- Kensi SP, Wiley EL, Dutra JC, Mellott AL, Barr WG, Altman KW. Skull base Wegner's granulomatosis results in multiple cranial neuropathies. Am J Otolaryngol 2005;26:146-9.
- Ozaki T, Maeshima K, Kiyonaga Y, Torigoe M, Imada C, Hamasaki H, et al. Large-vessel involvement in GPA: successfully treated with RTX. A case report and literature review. Mod Rheumatol.2017;27(6):699-704.
- Kemna MJ, Bucerius J, Drent M, Voo S, Veeman M, Van Paassen P, et al. Aortic 18F-FDG uptake in patients suffering from GPA. Eur J Nucl Med Mol Imaging. 2015;42(9);1423-9.
- Galaska R, Kulawiak-Galaska D, Czuszyriska Z, Masiak A, Zdroiewski Z, Gruchala M. A rare case of complex cardiac involvement in GPA. Pol Arch Intern Med. 2017;127(1):63-5.
- McGeoch L, Carette S, Cuthbertson D, Hoffman GS, Khalidi N, Koening CL, et al. Cardiac involvement in GPA. J Rheumatol. 2015;42(9);1209-12.
- Masiak A, Zdrojewski L, Zdrojewski Z, Bullo-Piontecka B, Rutkowski B, Gastrointestinal tract involvement in GPA. Prz Gastroenterol. 2016;11(4):270-5.
- John H Stone. Wegener's Granulomatosis Etanercept Trial (WGET) Research Group. Limited versus severe Wegener's granulomatosis: baseline patient data on patients in the Wegener's granulomatosis etanercept trial. Arthritis Rheum. 2003;48(8):2299-309.
- Yates M, Watts RA, Bajema IM, Cid MC, Crestani B, Hauser T, et al. EULAR/EDTA recommendations for managing ANCA associated vasculitis. Ann Rheum Dis 2016;75(9);1583-94.
- Stegeman CA, Tervaert JW, Suiter WJ, Manson WL, de Jong PE, Kallenberg CG. Association of chronic nasal carriage of staphylococcus aureus and higher relapse rate in Wegner's granulomatosis. Ann Intern Med. 1994;120(1): 12-17.
- Lally L, Spiera R. Current therapies for ANCA associated Vasculitis. Annu Rev Med. 2015;66(3):227-40.
- Holle JU, Dubrau C, Herlyn K, Heller M, Ambrosch P, Noelle B, Reinhold-Keller E, Gross WL. Rituximab for refractory GPA (Wegner's granulomatosis). Comparison of efficacy in granulomatosis versus vasculitic manifestations. Ann Rheum Dis. 2017;71(4):327-33.
- Pagnoux C, Hogan SL, Chin H, Jennette JC, Falk RJ, Guillevin L, Nachman PH. Predictors of treatment resistance and relapse in ANCA associated Vasculitis: comparison of two independent cohorts. Arthritis Rheum. 2008;58(9):2908-18.
- Shi L. ANCA associated vasculitis: Prevalence, treatment, and outcomes. Rheumatol Int. 2017;37(11):1779-88.
- Unizony S, Villarreal M, Miloslavsky E, Lu N, Merkel P, Spiera R, et al. Clinical outcomes of treatment of ANCA associated Vasculitis based on ANCA type. Ann Rheum Dis. 2016;75(6):1166-9
- Phillip R, Luqmani R. Mortality in systemic Vasculitis: a systematic review. Clin Exp Rheumatol. September-October. 2008;26(1): S94-104.
- Rood IM, Bavinck A, Lipska-Ziętkiewicz BS, Lugtenberg D, Schaefer F, Deegens JK, Wetzels JF. Later Response to Corticosteroids in Adults with Primary FSGS Is Associated with Favorable Outcomes. Kidney International Reports. 2021 Oct 29.In press.
Reference list indexed for Google Scholar and ResearchGate. DOI links resolve via doi.org.