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]
- a histopathology that shows granuloma
- the upper respiratory tract is involved
- there is a stenosis that is present in larynx, trachea, and the bronchioles
- the pulmonary system is involved
- the presence of anti-neutrophil cytoplasmic antibodies
- 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.
- Upper airway, eye and ear disease:
- Nose: pain, stuffiness, nosebleeds, rhinitis, crusting, saddle-nose deformity due to aperforated septum
- Ears: conductive hearing loss due to Eustachian tube dysfunction, sensorineural hearing loss (unclear mechanism)
- Eyes: pseudotumours, scleritis, conjunctivitis, uveitis, episcleritis
- Airways:
- Trachea: subglottal stenosis
- Lungs: pulmonary nodules (referred to as "coin lesions"), infiltrates (often interpreted aspneumonia), cavitary lesions, pulmonary hemorrhage causing hemoptysis), and rarely bronchial stenosis.
- Kidney: rapidly progressive segmental necrotising glomerulonephritis (75%), leading tochronic renal failure
- Arthritis: Pain or swelling (60%), often initially diagnosed as rheumatoid arthritis
- Skin: nodules on the elbow, purpura, various others (see cutaneous vasculitis)
- Nervous system: occasionally sensory neuropathy (10%) and rarely mononeuritis multiplex
- Heart, gastrointestinal tract, brain other organs: rarely affected.
References
- ↑ 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.
- ↑ 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.