Wegener's granulomatous scleritis

Introduction to Wegener's granulomatous scleritis Wegener's granulomatosis (WG) is a systemic disease of unknown etiology characterized by upper and lower respiratory granulomatous inflammation, necrotizing vasculitis and nephritis, which can be divided into complete, restrictive and highly restrictive 3 Types. basic knowledge Sickness ratio: 0.05% Susceptible people: no special people Mode of infection: non-infectious Complications: keratitis, anterior uveitis, glaucoma, cataract, optic disc edema

Wegener's granulomatous scleritis

Causes:

The cause is unknown and is considered to be an allergic reaction to an unknown inhaled antigen that causes respiratory granulomatous inflammation, vasculitis, elevated serum IgA and IgG, and systemic vascular and organ involvement, although no family, geography or occupation is found. Other factors are related to the disease, but studies have suggested that the detection rate of HLA-B8 antigen is high in the disease.

Pathogenesis:

Although the specific mechanism of granulomatous vasculitis is unclear, there is considerable evidence of immune disorders and antibody- and cell-mediated tissue damage, and 50% of patients have elevated circulating immune complexes (CIC). Rheumatoid factor (RF)-positive and elevated serum IgA and IgG levels are also common. In some renal biopsy specimens, IgG, complement components and fibrin deposition can be seen. For lung lesions, granulomatous lesions are mainly T. Lymphocytes and macrophages infiltrate, mainly in the inflammatory blood vessels and around the neutrophils, some studies suggest that the disease is neutrophil abnormalities, chemotaxis loss, the emergence of ANCA and leukocytes in the blood vessels It is an early change in the inflammatory process.

It is likely that more than one immune mechanism is involved in the onset of the disease, and an abnormal or excessive antibody response to an inhaled antigen can cause a CIC-stimulated macrophage-T lymphocyte granulomatous response in and around the blood vessel.

Wegener granulomatous scleritis prevention

Untreated WG can rapidly worsen death, especially in people with impaired renal function. When the patient develops kidney disease, the average survival rate is 5 months, the 1-year mortality rate is 82%, and the 2-year mortality rate is 90%. Treatment with systemic glucocorticoids and immunosuppressive agents can reduce the condition of 93% of patients. Therefore, early diagnosis and early treatment of Wegener's granulomatous scleritis is extremely important.

Wegener granulomatous scleritis complications Complications anterior keratitis uveitis glaucoma cataract optic disc edema

There may be chorioretinal ischemia and obstruction, dry conjunctival keratitis and anterior uveitis, keratitis, uveitis, glaucoma, cataracts and optic disc edema caused by scleritis, and about 50% of the eyes have decreased vision.

Wegener's granulomatous scleritis symptoms Common symptoms Corneal ulcer Hematuria Nasal mucosal ulcer Edge ulcerative keratitis Eyeball perforation Eyeball proteinuria Nasal septum perforation Retinal artery occlusion Saddle nose

Eye performance

29% to 58% of WG patients have abnormal ocular manifestations, which can be divided into adjacent and focal type 2, adjacent lesions include severe orbital pseudotumor, abscess, cellulitis and nasolacrimal duct obstruction, mainly by proximity Long-term granulomatous sinusitis spread, eyelid inflammation with eyeball protrusion is the most common ocular lesion in WG, focal lesions and upper respiratory tract disease are not related, mainly caused by focal vasculitis, the most common It is conjunctivitis, keratitis, scleritis, and scleral inflammation. Patients with WG uveitis may also have other focal eye diseases such as ischemic optic neuropathy and retinal artery occlusion, although most complete or restrictive WG The above abnormalities of the respiratory tract are more common, but the ocular manifestation may be the first symptom, which causes the patient's attention and seek medical treatment. In highly restrictive WG patients, the eye or eyelid lesion is the only clinical manifestation, and the ophthalmologist may be diagnosed with this type of WG. The first person.

(1) Scleritis: It is reported that the incidence of WG in patients with scleritis is 3.79%. Conversely, the incidence of scleritis in WG patients is 7% to 11%. The type of scleritis may be nodular, diffuse or necrotic. Necrotizing anterior scleritis and marginal ulcerative keratitis (Figures 1, 2) are the most severe ocular lesions of the WG, causing perforation of the eye, blindness, and even removal of the eyeball, and nodular polyarteritis scleritis (PAN) The corneal ulcers are very similar. The ulcers gradually progress toward the periphery and the center. The ulcer surface that erodes toward the center often forms the edge of the labial lip. The shape is very similar to the Mooren ulcer. The three types of WG can be found in the initial examination with scleritis, sclera. Inflammation usually occurs with systemic symptoms, but it is also the first manifestation of worsening systemic disease, but necrotizing anterior scleritis can occur after surgical trauma.

Of the 172 cases of scleritis observed by Foster et al, 14 were WG (8.13%), 2 of which were complete, 10 were restrictive, 2 were highly restrictive WG, and the mean age was 60 years (15~ 81 years old), more men than women (10:4), except for 1 case, all patients with scleritis is the first symptom, further examination to diagnose WG, 9 patients with upper and lower respiratory symptoms, scleritis and WG diagnosis The average time between the two is 8.5 months. Most of the scleritis is persistent or recurrent, often associated with acute exacerbation of systemic disease. 11 cases have necrotizing anterior scleritis, 4 of which occur after surgery and 2 are diffuse. Anterior scleritis, 1 case of nodular anterior scleritis, 7 cases (50%) of 14 cases of WG scleritis with marginal ulcerative keratitis, 6 cases of necrotizing anterior scleritis, 1 case of diffuse anterior scleritis Of the 14 patients, 6 (43%) had a similar incidence of anterior uveitis (42%) with 158 cases of scleritis without WG, indicating no correlation between anterior uveitis and WG. .

(2) Scleral outer inflammation: WG patients have a smaller rate of scleral inflammation than scleritis, and scleral inflammation may be the first symptom of WG.

2. Non-eye performance

The most common clinical manifestations of WG are upper and lower respiratory tract lesions, but it should not be forgotten that the disease can show a variety of symptoms and signs, should be reminded.

In the upper and lower respiratory tract abnormalities of the WG, pulmonary infiltration and sinusitis are the two most common clinical manifestations. Other common upper and lower respiratory tract abnormalities in WG include hemorrhagic rhinitis, nasal mucosal ulcers and otitis media caused by obstruction of the eustachian tube. Typical lower respiratory symptoms are cough, hemoptysis, shortness of breath and rare chest pain. Chest effusion and subglottic stenosis may also occur. Loss of nasal septum and nasal support may lead to characteristic saddle nose formation. Respiratory lesions often Concurrent with secondary infections such as Staphylococcus aureus.

Renal lesions often occur after respiratory lesions, either mild focal glomerulonephritis, or fulminant, diffuse, necrotizing glomerulonephritis, renal involvement with hyperplasia and crescentic changes Including hematuria, azotemia, proteinuria and foot edema, once renal disease occurs, can progress rapidly, the prognosis is generally poor.

Other systemic manifestations include joint pain and non-arthritis; skin lesions such as papules, small vesicles, cyanosis, ulcers and subcutaneous nodules; peripheral neuropathy, including multiple mononeuritis and cranial nerve palsy; cardiac involvement with acute pericardium Inflammatory and congestive heart failure, other rare manifestations of this disease are thyroiditis, parotid masses, nasal and tympanic granuloma and ulcerative breast masses.

Wegener's granulomatous scleritis

1. WG characteristic laboratory test results

Non-specific laboratory tests resulted in positive pigmented anemia, increased white blood cells and platelets, increased ESR, RF positive, elevated C-reactive protein (CRP) and serum immunoglobulin.

In 1985, anti-neutrophil cytoplasmic antibodies (ANCA) were found in sera of patients with WG, and their levels were associated with disease activity, and further studies confirmed this specificity, sensitivity of ANCA and type of WG, activity. And to the extent: the sensitivity of the restricted activity period is 67%, the sensitivity after remission is only 32%, and the sensitivity of the complete activity period is 96%. These findings suggest that ANCA negative can not rule out the diagnosis of WG, in the glomerulus In patients with nephritis, pulmonary-renal syndrome and vasculitis, the ANCA false positive was only 0.6%.

There are at least two methods for ANCA staining of neutrophils, one is classical granulocyte cytoplasmic staining (C-ANCA), which has a neutrophil serine protease (also known as pro-granulin). Specificity, all patients with C-ANCA positive performance meet the classic diagnostic criteria of WG, and the other is perinuclear staining (P-ANCA) for various microparticle breakdown enzymes such as myeloperoxidase, histone protease G, human leukocytes Elastase and lactate are specific, and P-ANCA is a specific marker of idiopathic necrotizing crescentic glomerulonephritis, which is often associated with subtle arthritis and occasionally associated with WG. The differential diagnosis of subtle arthritis and WG is that the former lacks granulomatous lesions, the latter has granulomatous lesion characteristics, WG idiopathic, necrotic, crescentic glomerulonephritis and subtle arthritis seem to be a disease Different manifestations, WG is mainly related to C-ANCA staining positive, and sometimes also shows P-ANCA positive, when suffering from eye diseases associated with systemic vasculitis, ANCA should be examined, ANCA measurement has a high degree of diagnosis of WG scleritis Specificity and sensitivity, 3 types Patients with WG (complete, restrictive, and highly restrictive) with ocular disease can have both C-ANCA and P-ANCA positive, and because the ANCA assay has high specificity and sensitivity to WG, it can be passed Early diagnosis and recurrence monitoring to improve the prognosis of WG, only ANCA should be cautious, and must be carefully examined in conjunction with other parts of the body before the diagnosis of WG can be made.

2. Bacterial culture and special staining

Granulomatous inflammation caused by acid-fast bacilli, fungi, etc. can be excluded for differential diagnosis.

3. Pathological examination

Microscopically, the tumor cells are loosely distributed, the nucleus is round or irregular, the deep staining or chromatin is spotted, and the cytoplasm is rare. The lesion may be accompanied by coagulative necrosis and inflammatory cell infiltration, and may invade the surrounding muscle tissue.

Chest X-ray: When a typical lower respiratory tract symptom occurs, it can show multiple inflamed nodular infiltration of bilateral lungs, easy to form cavities and atelectasis after infiltration and dissipation.

Diagnosis and differentiation of Wegener's granulomatous scleritis

WG diagnosis can be made based on clinical and pathological findings, changes in lower respiratory tract granuloma, glomerulonephritis, and vasculitis involving other organs. In 1990, the American College of Rheumatology listed the diagnostic criteria for WG. When two or more of the following four criteria appeared, the sensitivity and specificity of the WG were 88.2% and 92%, respectively: 1 abnormal urine sediment (cell tube type or 5 red blood cells per high power field), 2 Abnormal chest X-ray (nodules, cavities or mixed infiltration), 3 oral ulcers or nasal excretions, 4 vessel walls, granulomatous inflammation around the blood vessels or outside the blood vessels.

Regardless of whether there is ocular inflammation (nasal mucosa, sinus tissue, skin and lung) vasculitis and ANCA positive, as long as the characteristic pathological manifestations of necrotizing granulomatosis and the corresponding systemic clinical manifestations can diagnose WG, if ANCA is negative, Regardless of whether there is microvascular disease of the conjunctiva and/or scleral tissue, especially when the clinical features are atypical, the pathological manifestations are correlated with the diagnosis of complete WG.

When the corresponding eye disease occurs, even if there is no characteristic eye histopathological change, if the ANCA is positive, the height-restricted WG may be prompted. Conversely, if there is a characteristic eye pathological change without corresponding systemic clinical manifestations, ANCA is negative, then Support WG diagnostics.

When the cornea forms an ulcer, it can gradually progress to the periphery and the center. The eroded ulcer surface often forms the edge of the lip-shaped protrusion. The shape is very similar to the Mooren ulcer, but the Mooren ulcer is never accompanied by a scleral lesion and can be identified.

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