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]

Overview

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. There are many similarities that are present between ANCA associated vacuities and Granulomatosis with polyangiitis. A history and clinical symptoms can help assess the disease.

History

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. They include:

  • Eosinophilic granulomatosis with polyangiitis
  • Microscopic polyangiitis
  • Goodpasture syndrome
  • Henoch-Schonlein purpura
  • Lupus nephritis
  • Membranous nephropathy
  • Drug induced

Symptoms

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

Constitutional Symptoms

  • malaise
  • pyrexia
  • weight loss
  • arthralgia
  • cough
  • dyspnea
  • purpura
  • abnormal urinary sediment

Ear, nose and throat involvement:

  • nasal crusting
  • sinusitis
  • otitis media
  • otorrhea
  • epistaxis
  • ulcers of the oral cavity or nasal
  • conductive and or sensorineural hearing loss
  • saddle nose deformity
  • masses present in the upper airway
  • cranial nerve entrapment

Pulmonary involvement:

  • hoarseness
  • cough
  • dyspnea
  • stridor
  • hemoptysis
  • pulmonary fibrosis
  • pulmonary arterial hypertension
  • parenchymal lung nodules

Renal involvement:

  • glomerulonephritis
  • hematuria
  • rise in serum creatinine
  • edema
  • hypertension
  • red cell cast

Cutaneous involvement:

  • leukocytoclastic angiitis
  • livedo reticularis

Ophthalmic involvement:

  • conjunctivitis
  • ulceration of the cornea
  • episcleritis

Nervous system involvement:

  • mononeuritis multiplex
  • sensory neuropathy
  • sensorineural hearing loss
  • pachymeningitis


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

References

  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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