Wegener's granulomatosis history and symptoms


 * Associate Editor-In-Chief:

Signs and symptoms
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.

Symptoms in Detail
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.

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 ANCAs 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 enzymeproteinase 3 (cANCA) in neutrophils (a type of white blood cell) are associated with Wegener's.

If the patient has renal failure or cutaneous vasculitis, these are the most logical organs to obtain a biopsy from. Rarely, thoracoscopic lung biopsy is required. On histopathological examination, a biopsy will showleukocytoclastic vasculitis with necrotic changes and granulomatousinflammation (clumps of typically arranged white blood cells) on 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 trials. Two or more positive criteria have a sensitivity of 88.2% and a specificity of 92.0% of describing Wegener's.
 * 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:
 * microhematuriaor
 * red cell 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:
 * 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. \