Granulomatosis with polyangiitis history and symptoms

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2]Associate Editor(s)-in-Chief: Krzysztof Wierzbicki M.D. [3]Cafer Zorkun, M.D., Ph.D. [4]Amandeep Singh M.D.[5]


The symptomatology depends on the system involved. The disease involve ear nose and throat and cause symptoms of Sinusitis, nasal crusting, otitis media, Otorrhea, epistaxis. Patients with history of lung involvement presents with Hoarseness, Cough, Dyspnea, Stridor, Hemoptysis. Renal involvement presents with cloudy urine with hematuria, edema.

History and Symptoms

Obtaining a complete history is an important aspect in making a clinical diagnosis of Granulomatosis with polyangiitis. As it can help differentiate between the ANCA associated vasculitis and other possible causes that may mimic the disease.

The initial symptomsthat are present in patients with Granulomatosis with polyangiitis are non-specific in nature. The following are history and symptoms of Granulomatosis with polyangiitis:[1][2][3][4]

Constitutional Symptoms

Ear, nose and throat involvement:

Pulmonary involvement:

Renal involvement:

Cutaneous involvement:

Ophthalmic involvement:

Nervous system involvement:

A diagnosis of Granulomatosis with polyangiitis can be made when three out of the six criteria are established. They are:[5]

  1. a histopathology that shows granuloma
  2. the upper respiratory tract is involved
  3. there is a stenosis that is present in larynx, trachea, and the bronchioles
  4. the pulmonary system is involved
  5. the presence of anti-neutrophil cytoplasmic antibodies
  6. the presence of glomerulonephritis


  1. WALTON EW (1958). "Giant-cell granuloma of the respiratory tract (Wegener's granulomatosis)". Br Med J. 2 (5091): 265–70. PMC 2026251. PMID 13560836.
  2. Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP; et al. (1990). "The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis". Arthritis Rheum. 33 (8): 1101–7. PMID 2202308.
  3. Fauci AS, Haynes BF, Katz P, Wolff SM (1983). "Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years". Ann Intern Med. 98 (1): 76–85. PMID 6336643.
  4. Pagnoux C (2016). "Updates in ANCA-associated vasculitis". Eur J Rheumatol. 3 (3): 122–133. doi:10.5152/eurjrheum.2015.0043. PMC 5058451. PMID 27733943.
  5. Noone D, Hebert D, Licht C (2016). "Pathogenesis and treatment of ANCA-associated vasculitis-a role for complement". Pediatr Nephrol.   ( ):  . doi:10.1007/s00467-016-3475-5. PMID 27596099.

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