Granulomatosis with polyangiitis history and symptoms: Difference between revisions

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


{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}
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==Overview==
==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.
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]].


== History ==
==History and 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.
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 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:<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>
* malaise
* pyrexia
* weight loss
* arthralgia
* cough
* dyspnea
* purpura
* abnormal urinary sediment


Ear, nose and throat involvement:
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>
* 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:
'''Constitutional Symptoms'''
* hoarseness
* [[Malaise]]
* cough
* [[Pyrexia]]
* dyspnea
* [[Weight loss]]
* stridor
* [[Arthralgia]]
* hemoptysis
* [[Cough]]
* pulmonary fibrosis
* [[Dyspnea]]
* pulmonary arterial hypertension
* [[Purpura]]
* parenchymal lung nodules


Renal involvement:
'''Ear, nose and throat involvement:'''
* glomerulonephritis
* [[Sinusitis]]
* hematuria
* Nasal crusting
* rise in serum creatinine
* [[Otitis media]]
* edema
* [[Otorrhea]]
* hypertension
* [[Epistaxis]]
* red cell cast
* 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


Cutaneous involvement:
'''Pulmonary involvement:'''
* leukocytoclastic angiitis
* [[Hoarseness]]
* livedo reticularis
* [[Cough]]
* [[Dyspnea]]
* [[Stridor]]
* [[Hemoptysis]]
* [[Pulmonary fibrosis]]
* [[Pulmonary arterial hypertension]]
* Parenchymal lung nodules


Ophthalmic involvement:
'''Renal involvement:'''
* conjunctivitis
* [[Glomerulonephritis]]
*  
* [[Hematuria]]
* Rise in [[serum creatinine]]
* [[Edema]]
* [[Hypertension]]


A diagnosis of Granulomatosis with polyangiitis can be made when three out of six criteria are established:<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>
'''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 interna|Pachymeningitis]]
 
 
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>
# a histopathology that shows granuloma
# a histopathology that shows granuloma
# the upper respiratory tract is involved
# the upper respiratory tract is involved
Line 64: Line 73:
# the presence of anti-neutrophil cytoplasmic antibodies
# the presence of anti-neutrophil cytoplasmic antibodies
# the presence of glomerulonephritis
# the presence of glomerulonephritis
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.
* [[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==
The first symptoms of Wegener's granulomatosis are often vague and frequently include upper respiratory tract symptoms, joint pains, weakness, and tiredness.
===Upper respiratory tract===
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.
===Lungs===
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.
===Kidneys===
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.
===Musculoskeletal system===
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.
===Eyes===
Wegener's granulomatosis can affect the eyes in several ways. People may develop;
* Conjunctivitis (inflammation of the conjunctiva, the inner lining of the eyelid)
* Scleritis (inflammation of the scleral layer, the white part of the eyeball)
* Episcleritis (inflammation of the episcleral layer, the outer surface of the sclera)
* Mass lesion behind the eye globe
Symptoms in the eye include redness, burning, or pain. Double vision or a decrease in vision are serious symptoms requiring immediate medical attention.
===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===
Some people experience narrowing of the trachea. The symptoms can include voice change, hoarseness, shortness of breath, or cough.
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.


==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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