Uveitis with Wegner's Granulomatosis

Introduction to Uveitis associated with Wegner's granulomatosis Wegener granulomatosis (Wegner's granulomatosis) is a kind of idiopathic systemic necrotizing vasculitis, mainly involving the upper respiratory tract, often accompanied by kidney damage, and 28% to 58% of patients have multiple eye damage. basic knowledge The proportion of illness: 0.001% Susceptible people: no special people Mode of infection: non-infectious Complications: retinal vasculitis recurrent polychondritis associated with uveitis

Causes of uveitis associated with Wegner granulomatosis

Causes:

The cause is unclear. Some people think that the disease is caused by an allergic reaction. Some infectious factors such as parvovirus B19 and Staphylococcus aureus may be related to the disease. The respiratory tract may be the initial site of allergic reaction, but so far there is no Conclusive evidence confirms this view, and it has been suggested that the disease may be caused by the deposition of immune complexes and by the release of multiple active products.

Pathogenesis:

The pathogenesis of Wegner's granulomatosis is not fully understood. It is believed that type IV allergic reactions play an important role in its occurrence. Activated CD4 cells release cytokines such as -interferon and -tumor necrosis factor and cause inflammatory reactions. And tissue damage; there is evidence that anti-neutrophil cytoplasmic antibodies may enhance neutrophil oxidation, degranulate, enhance their adhesion to vascular endothelial cells, promote their release of IL-1 and promote monocyte release Mechanisms such as IL-8 enhance the inflammatory response; studies have shown that patients have abnormal expression of costimulatory molecules, and the above results indicate that there are multiple mechanisms involved in the development of Wegner granulomatosis.

Wegner granuloma associated with uveitis prevention

There is no effective preventive measure for this disease. Early detection and early diagnosis are the key to the prevention and treatment of this disease.

Wegner granulomatosis associated with uveitis complications Complications Retinal vasculitis recurrent polychondritis associated with uveitis

Wegener granulomatosis (Wegner's granulomatosis) is an idiopathic systemic necrotizing vasculitis, mainly involving the upper respiratory tract, often accompanied by kidney damage, 28% to 58% of patients with multiple eye damage, can be complicated by multiple diseases damage. Including retinal vasculitis, recurrent multiple chondritis associated with.

Wegner granulomatosis associated with uveal inflammation symptoms Common symptoms Joint pain Joint swelling Weight loss Proteinuria Hearing loss Anorexia Nasal nosebleeds Hematuria fever

1. The incidence of ocular involvement in Wegner's granulomatosis is 14% to 78%, but only 15% of patients at the onset of the disease have ocular involvement. In some patients, the eye lesion may be the initial manifestation of the disease. For unilateral involvement, occasionally both eyes are affected.

Any eye tissue can be affected, some are secondary, such as eyelid inflammation and granuloma secondary to paranasal sinusitis, and some are primary, such as scleritis, uveitis.

Uveitis is a relatively common ocular manifestation with an incidence of 5% to 38% (not including retinitis, retinitis vasculitis), which can be manifested as unilateral involvement or bilateral involvement. For anterior uveitis, intermediate uveitis, posterior uveitis, total uveitis, retinitis, retinal vasculitis, etc., uveitis is mostly non-specific inflammation, can be granulomatous or non-granulomatous, more Occurred after a period of disease, ocular lesions often appear after systemic disease, and can be used as the only clinical manifestation of the disease before a small number of patients can appear in systemic lesions.

2. Systemic manifestations of systemic manifestations of upper and lower respiratory tract granuloma, systemic vasculitis and necrotizing glomerulonephritis, but in some patients, at different stages of the disease, the above three major aspects of damage may not always be Come out, in addition to skin, nervous system, cardiovascular system and a variety of eye diseases, patients with non-specific systemic manifestations usually have fever, discomfort, weight loss, anorexia, joint pain, muscle pain and so on.

(1) Necrotizing granulomatous inflammation of the upper and lower respiratory tract: Respiratory involvement is the most common manifestation of this disease. More than 90% of patients have such changes. In the early stage of the disease, patients often complain of pain in the sinus area, purulent nasal discharge. , nasal congestion, nosebleed, hearing loss, etc., examination can be found in chronic rhinitis, sinusitis, nasal ulcers, serous otitis media, suppurative otitis media, mastoiditis, saddle-shaped nose, bilateral pulmonary parenchymal nodular infiltration, prone to lung Diffuse pulmonary hemorrhage can still occur in cavities, and pleural effusion occurs in about 20% of patients.

(2) glomerulonephritis: glomerulonephritis has a low incidence in the early stage of the disease, but it can be as high as 80% after 2 years of disease. The patient usually presents with proteinuria, hematuria, red blood cell cast, and finally progresses. For kidney failure.

(3) Skin damage: About 13% of patients have skin changes, the most common is purpura, which occurs in the lower limbs, trunk and upper limbs. Skin ulcers, blisters, papules, subcutaneous nodules and other diseases can also occur.

(4) joint pain and muscle pain: 2 kinds of performance is quite common, the incidence rate is as high as 70%, and patients may occasionally have joint swelling.

(5) nervous system damage: 21% to 54% of patients with nervous system involvement, manifested as peripheral neuropathy (such as multiple mononeuritis), cranial neuropathy, ophthalmoplegia, epilepsy, encephalitis, stroke.

(6) Others: a small number of patients may have pericarditis, myocarditis, arteritis, parotid gland, breast, urethra, intestines, etc. may also be affected.

The diagnosis of this disease is mainly based on necrotizing granulomatous vasculitis of the upper and lower respiratory tract, focal necrotizing glomerulonephritis and systemic necrotizing vasculitis involving arteries and veins, and the appearance of ocular protrusion and necrotizing scleritis Patients with peripheral corneal ulcers and various types of uveitis should be asked in detail about the respiratory tract, kidney disease and other medical history. If mucosal ulcers, ankle ulcers and destructive sinusitis are found, it will be helpful for diagnosis.

Wegner granuloma associated with uveitis examination

At present, there is no definite laboratory examination for this disease. Some patients may have leukocytosis, normal erythrocyte and erythrocyte pigmentation anemia, thrombocytosis, and erythrocyte sedimentation rate. PR3-anti-neutrophil cytoplasmic antibody determination during disease activity There is a sensitivity of up to 90%, but the sensitivity of this test is reduced to 40% during disease remission, and the specificity of this experiment is about 90%.

1. Chest X-ray examination found that lung infiltration and nodules are helpful for diagnosis, but to rule out other causes.

2. Biopsy is helpful for diagnosis. The most common pathological changes are tissue necrosis, granuloma formation and vasculitis, but it is difficult to see three changes in the same specimen, so when performing biopsy, One or two changes were found, and the diagnosis of Wegner's granulomatosis should not be easily ruled out.

Diagnosis and differentiation of ugitis associated with Wegner granulomatosis

The diagnostic criteria for this disease are mainly based on the triad of the disease, namely necrotizing granuloma of the upper and lower respiratory tract, systemic vasculitis and focal necrotizing glomerulonephritis. If the patient has three manifestations, It is a complete type, and if it lacks one or two kinds of performance, it is called an incomplete type.

Differential diagnosis

Wegner granulomatosis should be differentiated from systemic diseases that can cause vasculitis, respiratory inflammation, and glomerulonephritis. For uveitis and/or scleritis, it should be associated with sarcoma-like uveitis and recurrence. Identification of multiple chondritis and its associated uveitis.

1. Sarcoma-like disease can cause lung disease, uveitis and scleritis, but the lung lesions caused by it mainly manifest as hilar lymphadenopathy, in addition to causing many types of uveitis and scleritis, often Caused by conjunctival nodules, lacrimal gland enlargement, easy to cause lymphadenopathy, the level of angiotensin-converting enzyme is usually elevated in patients, according to these characteristics is generally not difficult to identify the two.

2. Recurrent polychondritis can also cause changes in nasal cartilage, saddle-shaped nasal deformity, uveitis and scleritis, but this disease can also cause changes in auricular cartilage, larynx, tracheal cartilage, generally no upper or lower The necrotizing granuloma of the respiratory tract, these characteristics contribute to the identification of the two.

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