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{{Wegener's granulomatosis}}
{{Wegener's granulomatosis}}


{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}
{{CMG}}{{APM}}{{AE}}{{KW}}{{CZ}}{{ADS}}


==Overview==
==Overview==
Initial signs are protean, and diagnosis can be severely delayed due to the non-specific nature of the [[symptom]]s. The rhinitis is generally the first sign in most patients.
The symptomatology depends on the system involved. The disease involve ear nose and throat and cause symptoms of [[Sinusitis]], nasal crusting[[Otitis media|, otitis media]], [[Otorrhea]][[Epistaxis|, epistaxis]]. Patients with history of lung involvement presents with [[Hoarseness]], [[Cough]], [[Dyspnea]], [[Stridor]], [[Hemoptysis]]. Renal involvement presents with cloudy urine with [[hematuria]], [[edema]].
* [[Respiratory tract|Upper airway]], [[eye]] and [[ear]] disease:
** Nose: pain, stuffiness, [[nosebleed]]s, [[rhinitis]], crusting, ''saddle-nose'' deformity due to a[[perforated septum]]
** Ears: conductive [[hearing loss]] due to [[Eustachian tube]] dysfunction, sensorineural hearing loss (unclear mechanism)
** Eyes: pseudotumours, [[scleritis]], [[conjunctivitis]], [[uveitis]], episcleritis
* Airways:
** [[Vertebrate trachea|Trachea]]: subglottal [[stenosis]]
** [[Lung]]s: pulmonary nodules (referred to as "coin lesions"), infiltrates (often interpreted as[[pneumonia]]), cavitary lesions, [[pulmonary hemorrhage]] causing [[hemoptysis]]), and rarely bronchial stenosis.
* [[Kidney]]: rapidly progressive segmental necrotising [[glomerulonephritis]] (75%), leading to[[chronic 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]]
* [[The Heart in Wegener's Granulomatosis|Heart]], [[gastrointestinal tract]], [[brain]] other organs: rarely affected.


==Symptoms==
==History and Symptoms==
The first symptoms of Wegener's granulomatosis are often vague and frequently include upper respiratory tract symptoms, joint pains, weakness, and tiredness.
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.  


===Upper respiratory tract===
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:<ref name="pmid13560836">{{cite journal| author=WALTON EW| title=Giant-cell granuloma of the respiratory tract (Wegener's granulomatosis). | journal=Br Med J | year= 1958 | volume= 2 | issue= 5091 | pages= 265-70 | pmid=13560836 | doi= | pmc=2026251 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=13560836  }}</ref><ref name="pmid2202308">{{cite journal| author=Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP et al.| title=The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. | journal=Arthritis Rheum | year= 1990 | volume= 33 | issue= 8 | pages= 1101-7 | pmid=2202308 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2202308  }} </ref><ref name="pmid6336643">{{cite journal| author=Fauci AS, Haynes BF, Katz P, Wolff SM| title=Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. | journal=Ann Intern Med | year= 1983 | volume= 98 | issue= 1 | pages= 76-85 | pmid=6336643 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6336643  }} </ref><ref name="pmid27733943">{{cite journal| author=Pagnoux C| title=Updates in ANCA-associated vasculitis. | journal=Eur J Rheumatol | year= 2016 | volume= 3 | issue= 3 | pages= 122-133 | pmid=27733943 | doi=10.5152/eurjrheum.2015.0043 | pmc=5058451 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27733943  }} </ref>
The most common sign of Wegener's granulomatosis is involvement of the upper respiratory tract, which occurs in nearly all patients. Symptoms include sinus pain, discolored or bloody fluid from the nose, and, occasionally, nasal ulcers. A common sign of the disease is almost constant rhinorrhea ("runny nose") or other cold symptoms that do not respond to usual treatment or that become increasingly worse.


Rhinorrhea can result from nasal inflammation or sinus drainage and can cause pain. A hole may develop in the cartilage of the nose, which may lead to collapse (called saddle-nose deformity). The eustachian tubes, which are important for normal ear function, may become blocked, causing chronic ear problems and hearing loss. Bacterial infection can cause Wegener's-related sinusitis (inflammation of the sinuses) with congestion and chronic sinus pain.
'''Constitutional Symptoms'''
* [[Malaise]]
* [[Pyrexia]]
* [[Weight loss]]
* [[Arthralgia]]
* [[Cough]]
* [[Dyspnea]]
* [[Purpura]]


===Lungs===
'''Ear, nose and throat involvement:'''
The lungs are affected in most people with Wegener's granulomatosis, although no symptoms may be present. If symptoms are present, they include cough, hemoptysis (coughing up blood), shortness of breath, and chest discomfort.
* [[Sinusitis]]
* Nasal crusting
* [[Otitis media]]
* [[Otorrhea]]
* [[Epistaxis]]
* Ulcers of the oral cavity or nasal
* [[Conductive hearing loss|Conductive]] and or [[sensorineural hearing loss]]
* [[Saddle nose]] deformity
* Masses present in the upper airway
* Cranial nerve entrapment


===Kidneys===
'''Pulmonary involvement:'''
Kidney involvement, which occurs in more than three-fourths of people with this disorder, usually does not cause symptoms. If detected by blood and urine tests, a doctor can start proper treatment, preventing long-term damage to the kidneys.
* [[Hoarseness]]
* [[Cough]]
* [[Dyspnea]]
* [[Stridor]]
* [[Hemoptysis]]
* [[Pulmonary fibrosis]]
* [[Pulmonary arterial hypertension]]
* Parenchymal lung nodules


===Musculoskeletal system===
'''Renal involvement:'''
Pain in the muscles and joints or, occasionally, joint swelling affects two-thirds of people with Wegener's granulomatosis. Although joint pain can be very uncomfortable, it does not lead to permanent joint damage or deformities.
* [[Glomerulonephritis]]
* [[Hematuria]]
* Rise in [[serum creatinine]]
* [[Edema]]
* [[Hypertension]]


===Eyes===
'''Cutaneous involvement:'''
Wegener's granulomatosis can affect the eyes in several ways. People may develop;
* Leukocytoclastic angiitis
* [[Livedo reticularis]]


* Conjunctivitis (inflammation of the conjunctiva, the inner lining of the eyelid)
'''Ophthalmic involvement:'''
* Scleritis (inflammation of the scleral layer, the white part of the eyeball)
* [[Conjunctivitis]]
* Episcleritis (inflammation of the episcleral layer, the outer surface of the sclera)
* Ulceration of the [[cornea]]
* Mass lesion behind the eye globe
* [[Episcleritis]]


Symptoms in the eye include redness, burning, or pain. Double vision or a decrease in vision are serious symptoms requiring immediate medical attention.
'''Nervous system involvement:'''
* [[Mononeuritis multiplex]]
* [[Sensory neuropathy]]
* [[Sensorineural hearing loss]]
* [[Pachymeningitis interna|Pachymeningitis]]


===Skin lesions===
Nearly half of people with Wegener's granulomatosis develop skin lesions. These often have the appearance of small red or purple raised areas or blister-like lesions, ulcers, or nodules that may or may not be painful.


===Other symptoms===
A diagnosis of Granulomatosis with polyangiitis can be made when three out of the six criteria are established. They are:<ref name="pmid27596099">{{cite journal| author=Noone D, Hebert D, Licht C| title=Pathogenesis and treatment of ANCA-associated vasculitis-a role for complement. | journal=Pediatr Nephrol | year= 2016 | volume=  | issue=  | pages=  | pmid=27596099 | doi=10.1007/s00467-016-3475-5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27596099  }}</ref>
Some people experience narrowing of the trachea. The symptoms can include voice change, hoarseness, shortness of breath, or cough.
# a histopathology that shows granuloma
 
# the upper respiratory tract is involved
The nervous system and heart occasionally may be affected. Fever and night sweats may occur. Fever also may signal an infection, often of the upper respiratory tract.
# 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


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}


[[Category:Arthritis]]
[[Category:Arthritis]]

Latest revision as of 17:23, 11 April 2018

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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]

Overview

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

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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