Overview
Lymphomatoid granulomatosis (LG) is a rare Epstein-Barr virus (EBV)-associated B-cell lymphoproliferative disorder characterized by angiocentric and angiodestructive lymphoproliferation, often involving the central nervous system, lungs, and skin 1.Diagnosis
Management
Special Populations
Key Recommendations
References
Showing 100 most recent of 673 indexed papers.
1 Rysgaard CD, Stone MS. Lymphomatoid granulomatosis presenting with cutaneous involvement: a case report and review of the literature. Journal of cutaneous pathology 2015. link 2 Arslan S, Işik AÜ, Mungan S, Ural A, Sari RA. Wegener's granulomatosis with an atypical presentation as acute tonsillitis. ORL; journal for oto-rhino-laryngology and its related specialties 2014. link 3 Takazawa T, Ikeda K, Nagaoka T, Hirayama T, Yamamoto T, Yanagihashi M et al.. Wegener granulomatosis-associated optic perineuritis. Orbit (Amsterdam, Netherlands) 2014. link 4 Mota A, Sahebghadam Lotfi A, Jamshidi AR, Najavand S. Alpha 1-antitrypsin activity is markedly decreased in Wegener's granulomatosis. Rheumatology international 2014. link 5 Dinić MZ, Sekulović LK, Zolotarevski L, Zecević RD. Fulminant Wegener's granulomatosis: a case report. Vojnosanitetski pregled 2013. link 6 Ito K, Minamimoto R, Yamashita H, Yoshida S, Morooka M, Okasaki M et al.. Evaluation of Wegener's granulomatosis using 18F-fluorodeoxyglucose positron emission tomography/computed tomography. Annals of nuclear medicine 2013. link 7 Gómez-Torres A, Tirado Zamora I, Abrante Jiménez A, Esteban Ortega F. Wegener's granulomatosis causing bilateral facial paralysis and deafness. Acta otorrinolaringologica espanola 2013. link 8 Relle M, Cash H, Schommers N, Reifenberg K, Galle PR, Schwarting A. PR3 antibodies do not induce renal pathology in a novel PR3-humanized mouse model for Wegener's granulomatosis. Rheumatology international 2013. link 9 Pape E, Béné J, Buchdahl AL, Gautier S, Hatron PY, Lambert M. Desmopressin-related myocardial infarction in a patient with Wegener's granulomatosis: a case report and review of the literature. Journal des maladies vasculaires 2013. link 10 Huchzermeyer C, Mardin C, Holbach L, Zwerina J, Schett G, Rech J. Successful remission induction with a combination therapy of rituximab, cyclophosphamide, and steroids in a patient with refractory optic neuritis in Wegener's granulomatosis. Clinical rheumatology 2013. link 11 Singh R, Rosen S. Tumor of the heart in a young woman; a rare manifestation of Wegener granulomatosis. Human pathology 2012. link 12 Yamazaki H, Fujiwara K, Shinohara S, Kikuchi M, Kanazawa Y, Kurihara R et al.. Reversible cochlear disorders with normal vestibular functions in three cases with Wegener's granulomatosis. Auris, nasus, larynx 2012. link 13 Akbulut S. Multiple ileal perforations in a patient with Wegener's granulomatosis: a case report and literature review. Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract 2012. link 14 Lin CH, Tsai SH, Chen HC, Chen SJ. Heart blockage in a patient with cavitary lung lesions. The American journal of emergency medicine 2012. link 15 Thiel G, Shakeel M, Ah-See K. Wegener's granulomatosis presenting as meningitis. The Journal of laryngology and otology 2012. link 16 Ceri M, Ortabozkoyun L, Unverdi S, Kirac M, Duranay M. Massive intracerebral hemorrhage associated with Wegener granulomatosis. Rheumatology international 2012. link 17 Sharma A, Deshmukh S, Shaikh A, Dabholkar J. Wegener's granulomatosis mimicking skull base osteomyelitis. The Journal of laryngology and otology 2012. link 18 Shen J, Gill J, Shangguan M, Sampaio MS, Bunnapradist S. Outcomes of renal transplantation in recipients with Wegener's granulomatosis. Clinical transplantation 2011. link 19 Ishii T, Katada Y, Saeki Y. Spontaneous perirenal hematoma due to Wegener's granulomatosis after initiation of immunosuppressant. Modern rheumatology 2011. link 20 Pagnoux C, Stubbe M, Lifermann F, Decaux O, Pavic M, Bérezné A et al.. Wegener's granulomatosis strictly and persistently localized to one organ is rare: assessment of 16 patients from the French Vasculitis Study Group database. The Journal of rheumatology 2011. link 21 Barlas NB, Hassan HH, Al Badr FB, Bilal A. Structural and functional involvement of pituitary gland in Wegener's granulomatosis. Clinical neuroradiology 2011. link 22 Martinez Del Pero M, Walsh M, Luqmani R, Flossmann O, Mukhtyar C, Jani P et al.. Long-term damage to the ENT system in Wegener's granulomatosis. European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery 2011. link 23 Bennett HS, Reilly PG. Restylane--a temporary alternative for saddle nose deformity in nasal Wegener's granulomatosis--how we do it. The British journal of oral & maxillofacial surgery 2011. link 24 Szpirt WM, Heaf JG, Petersen J. Plasma exchange for induction and cyclosporine A for maintenance of remission in Wegener's granulomatosis--a clinical randomized controlled trial. Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association 2011. link 25 Vogt PM, Gohritz A, Haubitz M, Steiert A. Reconstruction of nasal deformity in Wegener's granulomatosis: contraindication or benefit?. Aesthetic plastic surgery 2011. link 26 Luqmani R, Suppiah R, Edwards CJ, Phillip R, Maskell J, Culliford D et al.. Mortality in Wegener's granulomatosis: a bimodal pattern. Rheumatology (Oxford, England) 2011. link 27 Hughes M, Burgess J, Parekh N. Wegener's granulomatosis complicated by pulmonary embolism: a case report and review of the literature. Modern rheumatology 2011. link 28 Vordenbäumen S, Timm D, Bleck E, Richter J, Fischer-Betz R, Chehab G et al.. Altered serum levels of human neutrophil peptides (HNP) and human beta-defensin 2 (hBD2) in Wegener's granulomatosis. Rheumatology international 2011. link 29 Barreto P, Pagnoux C, Luca L, Aouizerate J, Ortigueira I, Cohen P et al.. Dorsal prevertebral lesions in Wegener granulomatosis: report on four cases. Joint bone spine 2011. link 30 Thurner L, Müller A, Cérutti M, Martin T, Pasquali JL, Gross WL et al.. Wegener's granuloma harbors B lymphocytes with specificities against a proinflammatory transmembrane protein and a tetraspanin. Journal of autoimmunity 2011. link 31 Qian Q, Cornell L, Chandan V, Hartman R, Caples S. Hemorrhagic colitis as a presenting feature of Wegener granulomatosis. Journal of gastrointestinal and liver diseases : JGLD 2010. link 32 Church AC, Goldsmith K, Sivasothy P. Aspiration and development of subglottic stenosis in patients with Wegener's granulomatosis. The Journal of laryngology and otology 2010. link 33 Cordiner D, Evans CA, Morrice J. HSP or not HSP... that is the question. BMJ case reports 2010. link 34 Mukhopadhyay S, Hensley RG, Tazelaar HD. Cardiac involvement in Wegener granulomatosis diagnosed at autopsy. Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology 2010. link 35 Hattar K, Oppermann S, Ankele C, Weissmann N, Schermuly RT, Bohle RM et al.. c-ANCA-induced neutrophil-mediated lung injury: a model of acute Wegener's granulomatosis. The European respiratory journal 2010. link 36 Esposito S, Corona F, Defilippi A, Petaccia A, Chidini G, Dell'Era L et al.. Wegener's granulomatosis presenting with life-threatening lung hemorrhage in a 7-year-old child. Rheumatology international 2010. link 37 Koening CL, Gota CE, Langford CA, Hoffman GS, Natowicz MR. Serum chitotriosidase activity and Wegener's granulomatosis. Clinical biochemistry 2010. link 38 Grisaru S, Yuen GW, Miettunen PM, Hamiwka LA. Incidence of Wegener's granulomatosis in children. The Journal of rheumatology 2010. link 39 Hayes D, Iocono JA, Bennett JS, Lachman DC, Ballard HO. Epistaxis due to Wegener's granulomatosis in a pediatric patient. American journal of otolaryngology 2010. link 40 Flossmann O, Jayne DR. Long-term treatment of relapsing Wegener's granulomatosis with 15-deoxyspergualin. Rheumatology (Oxford, England) 2010. link 41 Ueda N, Inoue Y, Himeji D, Shimao Y, Oryoji K, Mitoma H et al.. Wegener's granulomatosis detected initially by integrated 18F-fluorodeoxyglucose positron emission tomography/computed tomography. Modern rheumatology 2010. link 42 Göktas O, Cao Van H, Fleiner F, Lacroix JS, Landis BN. Chemosensory function in Wegener's granulomatosis: a preliminary report. European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery 2010. link 43 Knight A, Sandin S, Askling J. Occupational risk factors for Wegener's granulomatosis: a case-control study. Annals of the rheumatic diseases 2010. link 44 Primo VC, Marusic S, Franklin CC, Goldmann WH, Achaval CG, Smith RN et al.. Anti-PR3 immune responses induce segmental and necrotizing glomerulonephritis. Clinical and experimental immunology 2010. link 45 Holvast A, de Haan A, van Assen S, Stegeman CA, Huitema MG, Huckriede A et al.. Cell-mediated immune responses to influenza vaccination in Wegener's granulomatosis. Annals of the rheumatic diseases 2010. link 46 Kelley JM, Edberg JC, Kimberly RP. Wegener's granulomatosis: a model of auto-antibodies in mucosal autoimmunity. Clinical immunology (Orlando, Fla.) 2010. link 47 Sharma A, Kumar S, Wanchu A, Lal V, Singh R, Gupta V et al.. Successful treatment of hypertrophic pachymeningitis in refractory Wegener's granulomatosis with rituximab. Clinical rheumatology 2010. link 48 Frigui M, Ben Hmida M, Kechaou M, Jlidi R, Bahloul Z. Wegener's granulomatosis presenting as multiple bilateral renal masses: case report and literature review. Rheumatology international 2009. link 49 Charlier C, Henegar C, Launay O, Pagnoux C, Berezné A, Bienvenu B et al.. Risk factors for major infections in Wegener granulomatosis: analysis of 113 patients. Annals of the rheumatic diseases 2009. link 50 Ullrich S, Gustke H, Lamprecht P, Gross WL, Schumacher U, Ambrosch P et al.. Severe impaired respiratory ciliary function in Wegener granulomatosis. Annals of the rheumatic diseases 2009. link 51 Baaj AA, Vale FL, Carter JD, Rojiani AM. Granulomatosis with CNS involvement: a neuroimaging clinicopathologic correlation. Journal of neuroimaging : official journal of the American Society of Neuroimaging 2009. link 52 Wilde B, Dolff S, Cai X, Specker C, Becker J, Tötsch M et al.. CD4+CD25+ T-cell populations expressing CD134 and GITR are associated with disease activity in patients with Wegener's granulomatosis. Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association 2009. link 53 Zubairi AB, Liaquat HB, Husain SJ, Fatima K. Wegener's granulomatosis: a diagnostic challenge. JPMA. The Journal of the Pakistan Medical Association 2009. link 54 Flossmann O, Baslund B, Bruchfeld A, Tervaert JW, Hall C, Heinzel P et al.. Deoxyspergualin in relapsing and refractory Wegener's granulomatosis. Annals of the rheumatic diseases 2009. link 55 Zycinska K, Wardyn KA, Zielonka TM, Krupa R, Lukas W. Co-trimoxazole and prevention of relapses of PR3-ANCA positive vasculitis with pulmonary involvement. European journal of medical research 2009. link 56 Novick D, Elbirt D, Dinarello CA, Rubinstein M, Sthoeger ZM. Interleukin-18 binding protein in the sera of patients with Wegener's granulomatosis. Journal of clinical immunology 2009. link 57 White JB, Shah RK. Wegener's granulomatosis of the pediatric airway: a case demonstrating a conservative management approach. American journal of otolaryngology 2009. link 58 Ooka S, Maeda A, Ito H, Omata M, Yamada H, Ozaki S. Treatment of refractory retrobulbar granuloma with rituximab in a patient with ANCA-negative Wegener's granulomatosis: a case report. Modern rheumatology 2009. link 59 Holvast A, Stegeman CA, Benne CA, Huckriede A, Wilschut JC, Palache AM et al.. Wegener's granulomatosis patients show an adequate antibody response to influenza vaccination. Annals of the rheumatic diseases 2009. link 60 Tsuzuki K, Fukazawa K, Takebayashi H, Hashimoto K, Sakagami M. Difficulty of diagnosing Wegener's granulomatosis in the head and neck region. Auris, nasus, larynx 2009. link 61 Voswinkel J, Assmann G, Held G, Pitann S, Gross WL, Holl-Ulrich K et al.. Single cell analysis of B lymphocytes from Wegener's granulomatosis: B cell receptors display affinity maturation within the granulomatous lesions. Clinical and experimental immunology 2008. link 62 Zycinska K, Wardyn KA, Zycinski Z, Smolarczyk R. Correlation between Helicobacter pylori infection and pulmonary Wegener's granulomacytosis activity. Journal of physiology and pharmacology : an official journal of the Polish Physiological Society 2008. link 63 Faurschou M, Sorensen IJ, Mellemkjaer L, Loft AG, Thomsen BS, Tvede N et al.. Malignancies in Wegener's granulomatosis: incidence and relation to cyclophosphamide therapy in a cohort of 293 patients. The Journal of rheumatology 2008. link 64 Sezer I, Kocabas H, Melikoglu MA, Budak BS, Ozbudak IH, Butun B. Spontaneous pneumothorax in Wegener's granulomatosis: a case report. Modern rheumatology 2008. link 65 Frantz MC, Frank H, von Weyhern C, Kiefer J. Unspecific parotitis can be the first indication of a developing Wegener's granulomatosis. European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery 2008. link 66 Heckmann M, Holle JU, Arning L, Knaup S, Hellmich B, Nothnagel M et al.. The Wegener's granulomatosis quantitative trait locus on chromosome 6p21.3 as characterised by tagSNP genotyping. Annals of the rheumatic diseases 2008. link 67 Boudny C, Nievergelt H, Braathen LR, Simon D. Wegener's granulomatosis presenting as pyoderma gangrenosum. Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG 2008. link 68 Ghinoi A, Pipitone N, Cavazza A, Boiardi L, Salvarani C. Wegener granulomatosis with spleen infarction: case report and review of the literature. Seminars in arthritis and rheumatism 2008. link 69 Nouraei SA, Obholzer R, Ind PW, Salama AD, Pusey CD, Porter F et al.. Results of endoscopic surgery and intralesional steroid therapy for airway compromise due to tracheobronchial Wegener's granulomatosis. Thorax 2008. link 70 Aouba A, Pagnoux C, Bienvenu B, Mahr A, Guillevin L. Analysis of Wegener's granulomatosis responses to rituximab: current evidence and therapeutic prospects. Clinical reviews in allergy & immunology 2008. link 71 Zycinska K, Wardyn KA, Zycinski Z, Zielonka TM. Association between clinical activity and high-resolution tomography findings in pulmonary Wegener's granulomacytosis. Journal of physiology and pharmacology : an official journal of the Polish Physiological Society 2008. link 72 Arlet JB, Le Thi Huong D, Marinho A, Cluzel P, Wechsler B, Piette JC. Arterial aneurysms in Wegener's granulomatosis: case report and literature review. Seminars in arthritis and rheumatism 2008. link 73 Schokkenbroek AA, Franssen CF, Dikkers FG. Dilatation tracheoscopy for laryngeal and tracheal stenosis in patients with Wegener's granulomatosis. European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery 2008. link 74 Zycinska K, Wardyn KA, Zielonka TM, Demkow U, Traburzynski MS. Chronic crusting, nasal carriage of Staphylococcus aureus and relapse rate in pulmonary Wegener's granulomatosis. Journal of physiology and pharmacology : an official journal of the Polish Physiological Society 2008. link 75 Danda D, Mathew AJ, Mathew J. Wegener's granulomatosis: a rare presentation. Clinical rheumatology 2008. link 76 Levi M, Kodsi SR, Rubin SE, Lyons C, Golden R, Olitsky SE et al.. Ocular involvement as the initial manifestation of Wegener's granulomatosis in children. Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus 2008. link 77 Zycinska K, Wardyn KA, Zielonka TM, Tyszko P, Straburzynski M. Procalcitonin as an indicator of systemic response to infection in active pulmonary Wegener's granulomacytosis. Journal of physiology and pharmacology : an official journal of the Polish Physiological Society 2008. link 78 Ahmed M, Niffenegger JH, Jakobiec FA, Ben-Arie-Weintrob Y, Gion N, Androudi S et al.. Diagnosis of limited ophthalmic Wegener granulomatosis: distinctive pathologic features with ANCA test confirmation. International ophthalmology 2008. link 79 Korkmaz B, Kuhl A, Bayat B, Santoso S, Jenne DE. A hydrophobic patch on proteinase 3, the target of autoantibodies in Wegener granulomatosis, mediates membrane binding via NB1 receptors. The Journal of biological chemistry 2008. link 80 Koukoura O, Mantas N, Linardakis H, Hajiioannou J, Sifakis S. Successful term pregnancy in a patient with Wegener's granulomatosis: case report and literature review. Fertility and sterility 2008. link 81 Sebastian JK, Voetsch B, Stone JH, Romay-Penabad Z, Lo GH, Allen NB et al.. The frequency of anticardiolipin antibodies and genetic mutations associated with hypercoagulability among patients with Wegener's granulomatosis with and without history of a thrombotic event. The Journal of rheumatology 2007. link 82 Peshin R, O'Gradaigh D. Mononeuritis multiplex as a presenting feature of Wegener granulomatosis: a case report. Clinical rheumatology 2007. link 83 Schuerwegh AJ, Verhelst J, Slabbynck H, Kockx MM, Coolen D. Wegener's granulomatosis presenting as a thyroid mass. Clinical rheumatology 2007. link 84 Armani M, Spinazzi M, Andrigo C, Fassina A, Mantovan M, Tavolato B. Severe dysphagia in lower cranial nerve involvement as the initial symptom of Wegener's granulomatosis. Journal of the neurological sciences 2007. link 85 Finkielman JD, Merkel PA, Schroeder D, Hoffman GS, Spiera R, St Clair EW et al.. Antiproteinase 3 antineutrophil cytoplasmic antibodies and disease activity in Wegener granulomatosis. Annals of internal medicine 2007. link 86 Thiryayi W, Donaldson MH, Border D, Tyagi A. An enhancing pituitary lesion in a young woman: a diagnostic dilemma. Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia 2007. link 87 Zycinska K, Wardyn KA, Zielonka TM, Otto M. The role ANCA and anti-GBM antibodies in pulmonary-renal syndrome due to Wegener's granulomatosis. Journal of physiology and pharmacology : an official journal of the Polish Physiological Society 2007. link 88 Ye S, Yang CD. How could we make a diagnosis of Wegener's granulomatosis?. Clinical rheumatology 2007. link 89 Kantari C, Pederzoli-Ribeil M, Amir-Moazami O, Gausson-Dorey V, Moura IC, Lecomte MC et al.. Proteinase 3, the Wegener autoantigen, is externalized during neutrophil apoptosis: evidence for a functional association with phospholipid scramblase 1 and interference with macrophage phagocytosis. Blood 2007. link 90 Wang DC, Wei JW, Liu JH, Hu YG. The upper thoracic spinal cord compression as the initial manifestation of Wegener's granulomatosis: a case report. European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society 2007. link 91 Zycinska K, Wardyn KA, Tyszko P, Otto M. Analysis of early death based on the prediction model in Wegener's granulomatosis with pulmonary and renal involvement. Journal of physiology and pharmacology : an official journal of the Polish Physiological Society 2007. link 92 Srouji IA, Andrews P, Edwards C, Lund VJ. Patterns of presentation and diagnosis of patients with Wegener's granulomatosis: ENT aspects. The Journal of laryngology and otology 2007. link 93 Zycinska K, Romanowska M, Nowak I, Rybicka K, Wardyn KA, Brydak LB. Antibody response to inactivated subunit influenza vaccine in patients with Wegener's granulomatosis. Journal of physiology and pharmacology : an official journal of the Polish Physiological Society 2007. link 94 de Silva DJ, Cole C, Luthert P, Olver JM. Masked orbital abscess in Wegener's granulomatosis. Eye (London, England) 2007. link 95 Levine D, Akikusa J, Manson D, Silverman E, Schneider R. Chest CT findings in pediatric Wegener's granulomatosis. Pediatric radiology 2007. link 96 Reddy RS, Biyyani S, Pauskar P, Fahmy NM, King JF. Extensive gastrointestinal tract and thyroid involvement with Wegeners granulomatosis. Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology 2007. link 97 Chen M, Yu F, Wang SX, Zou WZ, Zhang Y, Zhao MH et al.. Renal histology in Chinese patients with anti-myeloperoxidase autoantibody-positive Wegener's granulomatosis. Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association 2007. link 98 Keogh KA, Ytterberg SR, Fervenza FC, Carlson KA, Schroeder DR, Specks U. Rituximab for refractory Wegener's granulomatosis: report of a prospective, open-label pilot trial. American journal of respiratory and critical care medicine 2006. link 99 Voswinkel J, Mueller A, Kraemer JA, Lamprecht P, Herlyn K, Holl-Ulrich K et al.. B lymphocyte maturation in Wegener's granulomatosis: a comparative analysis of VH genes from endonasal lesions. Annals of the rheumatic diseases 2006. link 100 Ozbalkan Z, Kiraz S, Ozturk MA, Ertenli AI, Apras S, Calguneri M. Wegener's granulomatosis: clinical and laboratory results of a university hospital study of 20 patients from Turkey. Clinical rheumatology 2006. link