Granulomatosis with polyangiitis history and symptoms

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]

Overview

Obtaining a complete history is an important aspect in making a clinical diagnosis of Granulomatosis with polyangiitis. As it can help aid in differentiating between the ANCA associated vacuities 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 physical can help assess the disease.

Symptoms

Obtaining a complete history is an important aspect in making a clinical diagnosis of Granulomatosis with polyangiitis. As it can help aid in differentiating between the ANCA associated vacuities 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 physical can assess the disease. The initial common signs that are present in patients with Granulomatosis with polyangiitis are non-specific in nature. The most common complaints are constitutional symptoms such as:[1]

  • 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

A diagnosis of Granulomatosis with polyangiitis can be made when three out of six criteria are established:[2]

  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

Initial signs are protean, and diagnosis can be severely delayed due to the non-specific nature of the symptoms. The rhinitis is generally the first sign in most patients.

References

  1. 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.
  2. 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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