Wegeners granulomatosis history and symptoms: Difference between revisions

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{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}
{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}


==Signs and symptoms==
==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.
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:
* [[Respiratory tract|Upper airway]], [[eye]] and [[ear]] disease:
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* [[The Heart in Wegener's Granulomatosis|Heart]], [[gastrointestinal tract]], [[brain]] other organs: rarely affected.
* [[The Heart in Wegener's Granulomatosis|Heart]], [[gastrointestinal tract]], [[brain]] other organs: rarely affected.


===Symptoms in Detail===
==Symptoms==
The first symptoms of Wegener's granulomatosis are often vague and frequently include upper respiratory tract symptoms, joint pains, weakness, and tiredness.
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====
===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.
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.
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====
===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.
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====
===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.
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====
===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.
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====
===Eyes===
Wegener's granulomatosis can affect the eyes in several ways. People may develop;
Wegener's granulomatosis can affect the eyes in several ways. People may develop;


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Symptoms in the eye include redness, burning, or pain. Double vision or a decrease in vision are serious symptoms requiring immediate medical attention.
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====
===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.
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====
===Other symptoms===
Some people experience narrowing of the trachea. The symptoms can include voice change, hoarseness, shortness of breath, or cough.
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.
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.
==Diagnosis==
Vasculitis such as Wegener's granulomatosis is usually only suspected when a patient has had unexplained symptoms for a longer period of time. Determination of [[antineutrophil cytoplasmic antibody|ANCA]]s can aid in the diagnosis, but positivity is not conclusive and negative ANCAs are not sufficient to reject the diagnosis. Cytoplasmic staining ANCAs that react with the enzyme[[proteinase 3]] (cANCA) in [[neutrophil granulocyte|neutrophils]] (a type of [[white blood cell]]) are associated with Wegener's.
If the patient has [[chronic renal failure|renal failure]] or cutaneous vasculitis, these are the most logical organs to obtain a [[biopsy]] from. Rarely, [[thoracoscopy|thoracoscopic]] lung biopsy is required. On [[histopathology|histopathological]] examination, a biopsy will show''leukocytoclastic vasculitis'' with [[necrosis|necrotic]] changes and [[granuloma]]tous[[inflammation]] (clumps of typically arranged white blood cells) on [[light microscopy|microscopy]]. The latter is the main reason for the appellation of "Wegener's granulomatosis", although it is not an essential feature. Unfortunately, many biopsies can be non-specific and 50% provide too little information for the diagnosis of Wegener's.
==Diagnostic Criteria==
In 1990, the American College of Rheumatology accepted classification criteria for Wegener's. They were not intended for diagnosis, but for inclusion in [[randomized controlled trial]]s. Two or more positive criteria have a sensitivity of 88.2% and a specificity of 92.0% of describing Wegener's.<ref name=Leavitt>Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. ''Arthritis Rheum'' 1990;33:1101-7. PMID 2202308.</ref>
* Nasal or oral inflammation:
** painful or painless oral ulcers ''or''
** purulent or bloody nasal discharge
* Lungs: abnormal chest X-ray with:
** nodules,
** infiltrates ''or''
** cavities
* Kidneys: urinary sediment with:
** microhematuria''or''
** red cell [[urinary casts|casts]]
* Biopsy: granulomatous inflammation
** within the arterial wall ''or''
** in the perivascular area
According to the Chapel Hill Consensus Conference (CHCC) on the nomenclature of systemic vasculitis (1992), establishing the diagnosis of Wegener's granulomatosis demands:<ref name=Jenette>Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG,''et al''. Nomenclature of systemic vasculitides. Proposal of an international consensus conference.''Arthritis Rheum'' 1994;37:187-92. PMID 8129773.</ref>
* a granulomatous inflammation involving the respiratory tract, and
* a [[vasculitis]] of small- to medium-sized vessels.
Several investigators have compared the ACR and Chapel Hill criteria.<ref name=Bruce>Bruce IN, Bell AL. A comparison of two nomenclature systems for primary systemic vasculitis. ''Br J Rheumatol''1997;36:453-8. PMID 9159539.</ref>\


==References==
==References==
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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

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.

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

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