Rare Manifestation of Granulomatosis With Polyangiitis

Introduction: Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is a multisystem disorder that mainly involves airways and kidneys. Oral GPA may present as ulcerations or strawberry gingivitis. Early diagnosis and intervention are crucial for overall prognosis.Case Presentation: An unusual case of oral GPA manifested by chronic localized inflammation, leading to failure of dental implants in an elderly woman, is presented. Diagnosis was based on antineutrophil cytoplasmic testing and histopathologic evidence of necrotizing vasculitis. Disease remission was achieved with cyclophosphamide and steroids.Conclusion: Familiarity with oral manifestations of GPA will help dentists and oral surgeons diagnose and treat early or incipient GPA without delay.

[1]  P. Merkel,et al.  Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis. , 2011, Arthritis and rheumatism.

[2]  P. Merkel,et al.  Rituximab versus cyclophosphamide for ANCA-associated vasculitis. , 2010, The New England journal of medicine.

[3]  P. van Paassen,et al.  Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. , 2010, The New England journal of medicine.

[4]  P. Kiely,et al.  Cocaine‐induced midline destructive lesions mimicking ENT‐limited Wegener's granulomatosis , 2008, Scandinavian journal of rheumatology.

[5]  N. Jones,et al.  Lessons learnt in the management of Wegener's Granulomatosis: long-term follow-up of 60 patients. , 2007, Rhinology.

[6]  W. Ammouri,et al.  Lingual necrosis as the presenting sign of Wegener's granulomatosis , 2006, Clinical and experimental dermatology.

[7]  E. Marck,et al.  Pitfalls with anti-neutrophil cytoplasmic antibodies (ANCA) , 1989, Clinical Rheumatology.

[8]  Augustine S. Lee,et al.  Antineutrophil cytoplasmic antibodies reacting with human neutrophil elastase as a diagnostic marker for cocaine-induced midline destructive lesions but not autoimmune vasculitis. , 2004, Arthritis and rheumatism.

[9]  L. Suresh,et al.  Intraosseous sarcoidosis of the jaws mimicking aggressive periodontitis: a case report and literature review. , 2004, Journal of periodontology.

[10]  S. Muller,et al.  Aggressive destructive midfacial lesion from cocaine abuse. , 2002, Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics.

[11]  J. Greenspan,et al.  The epidemiology of the oral lesions of HIV infection in the developed world. , 2002, Oral diseases.

[12]  S. Crean,et al.  Sublingual gland involvement in systemic Wegener's granulomatosis: a case report. , 2002, International journal of oral and maxillofacial surgery.

[13]  J. V. von Lindern,et al.  Salivary gland involvement as an initial presentation of Wegener's disease. A case report. , 2000, International journal of oral and maxillofacial surgery.

[14]  D. Ettlin,et al.  Diagnostic patterns and delays in pemphigus vulgaris: experience with 99 patients. , 2000, Archives of dermatology.

[15]  M. Hayduk,et al.  "Strawberry" gingival hyperplasia: a pathognomonic mucocutaneous finding in Wegener granulomatosis. , 2000, Archives of dermatology.

[16]  L. Leventhal,et al.  Positive c-ANCA in a Patient with Lymphoma and Without Vasculitis. , 1997, Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases.

[17]  J. Piette,et al.  Wegener's granulomatosis. Dermatological manifestations in 75 cases with clinicopathologic correlation. , 1994, Archives of dermatology.

[18]  Guy Hoffman,et al.  Wegener Granulomatosis: An Analysis of 158 Patients , 1992, Annals of Internal Medicine.

[19]  D A Bloch,et al.  The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. , 2010, Arthritis and rheumatism.

[20]  M. Keogan,et al.  Anti-neutrophil cytoplasm antibodies in patients with monoclonal gammopathies. , 1990, Journal of clinical & laboratory immunology.

[21]  K. Andrassy,et al.  "False-positive" anti-neutrophil cytoplasmic antibodies in HIV infection , 1990, The Lancet.

[22]  E. Truelove,et al.  Oral presentation of Wegener granulomatosis. , 1990, Journal of the American Dental Association.

[23]  U. Alon,et al.  Anti-neutrophil cytoplasmatic antibodies in drug-induced Wegener-like disease. , 1990, Pediatric nephrology.

[24]  P. Tugwell,et al.  The American College of Rheumatology 1990 Criteria for the Classification of Fibromyalgia. Report of the Multicenter Criteria Committee. , 1990, Arthritis and rheumatism.

[25]  B. Mains Wegener's granulomatosis: false positive anti-neutrophil cytoplasmic antibody test , 1989, The Journal of Laryngology & Otology.

[26]  H. Takahashi,et al.  Primary extranodal non-Hodgkin's malignant lymphoma of the oral region: analysis of 11 autopsy cases. , 1987, Journal of oral pathology.

[27]  T. H. The,et al.  AUTOANTIBODIES AGAINST NEUTROPHILS AND MONOCYTES: TOOL FOR DIAGNOSIS AND MARKER OF DISEASE ACTIVITY IN WEGENER'S GRANULOMATOSIS , 1985, The Lancet.

[28]  B. Haynes,et al.  Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. , 1983, Annals of internal medicine.

[29]  M. Edwards,et al.  Wegener's granulomatosis: a case with primary mucocutaneous lesions. , 1978, Oral surgery, oral medicine, and oral pathology.

[30]  R. Deremee,et al.  Wegener's granulomatosis. Anatomic correlates, a proposed classification. , 1976, Mayo Clinic proceedings.

[31]  E W WALTON,et al.  Giant-cell Granuloma of the Respiratory Tract (Wegener's Granulomatosis) , 1958, British medical journal.

[32]  J. L. Fahey,et al.  Wegener's granulomatosis. , 1954, The American journal of medicine.