Digestive Diseases and Sciences

, Volume 63, Issue 10, pp 2810–2812 | Cite as

Extensive Gastrointestinal Manifestations as the Main Relapsing Disease in Granulomatosis with Polyangiitis

  • Omar Vergara-Fernández
  • Fredy Chablé-Montero
  • Natllely Ruiz
  • David Mitre-Reyes
  • Eduardo Cerda-Contreras
  • Andrea Hernández
  • Erika Marroquín-Fabián
  • Luis Felipe Flores-SuárezEmail author
Case Report

Granulomatosis with polyangiitis (GPA-Wegener’s) is a small vessel vasculitis associated with the presence of antineutrophil cytoplasm autoantibodies. Gastrointestinal involvement ranges from 5 to 20% of cases. We describe a case in whom relapsing disease with widespread gastrointestinal manifestations including pharyngeal, esophageal, small and large intestine, and anus was present for the first time.

A 43-years-old male, with a 10-year history of proteinase-3 (PR3-ANCA) positive GPA which presented initially with general (fever, malaise), articular, otorhinolaryngological, ocular (scleritis), and pulmonary manifestations (nodular disease, biopsy-proven), was treated with glucocorticoids (GC) and methotrexate (MTX) as remission induction therapy, followed by MTX as maintenance treatment for 5 years. After remission was achieved at month 6, gradual tapering of MTX during the following 4.5 years allowed for its withdrawal in 2010. He already had a history of a hemorrhoidal disease,...


Granulomatosis with polyangiitis ANCA Vasculitis Gastrointestinal Anorectal 



We thank Debora Bork, MA, for reviewing the article’s grammatical structure, and Mr. Jorge Velázquez for image editing.


  1. 1.
    Morchón-Simon D, Martín-Escudero JC. Hemorrhagic colitis as the onset of Wegener´s granulomatosis. Int J Colorectal Dis. 2011;26:259–260.CrossRefPubMedGoogle Scholar
  2. 2.
    Aymard B, Bigard NA, Thompson H, Schmutz JL, Finbet JF, Borrelly J. Perianal ulcer: an unusual presentation of Wegener´s Granulomatosis. Report of a case. Dis Colon Rectum. 1990;33:427–430.CrossRefPubMedGoogle Scholar
  3. 3.
    Latus J, Koetter I, Fritz P, et al. Gastrointestinal involvement in granulomatosis with polyangiitis and microscopic polyangiitis: histological features and outcome. Int J Rheum Dis. 2014;17:412–419.CrossRefPubMedGoogle Scholar
  4. 4.
    Eriksson P, Segelmark M, Hallböök O. Frequency, diagnosis, treatment, and outcome of gastrointestinal disease in granulomatosis with polyangiitis and microscopic polyangiitis. J Rheumatol. 2018;45:529–537.CrossRefPubMedGoogle Scholar
  5. 5.
    Torres J, Mehandru S, Colombel JF, Peyrin-Biroulet L. Crohn’s disease. Lancet. 2017;389:1741–1755. Scholar
  6. 6.
    Humbert S, Guilpain P, Puéchal X, et al. Inflammatory bowel disease in anti-neutrophil cytoplasmic antibody-associated vasculitides: 11 retrospective cases from the French Vasculitis Study Group. Rheumatology. 2015;54:1970–1975.CrossRefPubMedGoogle Scholar
  7. 7.
    Bossuyt X, Cohen Tervaert JW, Arimura Y, et al. Position paper: Revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis. Nat Rev Rheumatol. 2017;13:683–692.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Omar Vergara-Fernández
    • 1
  • Fredy Chablé-Montero
    • 2
  • Natllely Ruiz
    • 3
  • David Mitre-Reyes
    • 1
  • Eduardo Cerda-Contreras
    • 4
  • Andrea Hernández
    • 5
  • Erika Marroquín-Fabián
    • 3
  • Luis Felipe Flores-Suárez
    • 3
    • 6
    Email author
  1. 1.Colorectal SurgeryMédica Sur HospitalMexico CityMexico
  2. 2.Department of Pathologic AnatomyMédica Sur HospitalMexico CityMexico
  3. 3.Primary Systemic Vasculitides ClinicInstituto Nacional de Enfermedades RespiratoriasMexico CityMexico
  4. 4.Gastroenterology and Endoscopy ServicesMédica Sur HospitalMexico CityMexico
  5. 5.Internal Medicine ResidentMédica Sur HospitalMexico CityMexico
  6. 6.RheumatologyMédica Sur HospitalMexico CityMexico

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