Allergic granulomatous vasculitis-associated scleritis
Introduction
Introduction to allergic granulomatous vasculitis-related scleritis Allergic granulomatous vasculitis (AGA), also known as Churg-Strauss syndrome, is a vascular disease similar to nodular polyarteritis (PAN) that affects any organ in the body. basic knowledge The proportion of sickness: 0.0001% - 0.0004% Susceptible people: no specific population Mode of infection: non-infectious complication:
Cause
Allergic granulomatous vasculitis-related causes of scleritis
(1) Causes of the disease
The cause is unknown.
(two) pathogenesis
The pathogenesis is related to the immune response. The circulating immune complex (CIC) can be measured in the blood. The deposition of immunoglobulin and complement can be measured in the vessel wall. Although CIC is the main mechanism causing granulomatous vasculitis, cellular immunity is also Can cause, granulomatous reaction is generally delayed type hypersensitivity reaction, but CIC itself can stimulate the formation of granuloma, resulting in granulomatous vasculitis, so the immune response of granulomatous vasculitis can be single, or More than one type of vasculitis is likely to be associated with many host factors, such as genetic factors, immune regulation mechanisms, and the integrity of the reticuloendothelial system. The reticuloendothelial system can clear complexes from the circulation, and some complexes cause blood vessels. Inflammation, but not other vascular involvement, may be related to the size and physicochemical properties of CIC, and may be related to other physical factors such as blood flow turbulence, intravascular hydrostatic pressure, and pre-existing vascular endothelial damage.
Prevention
Allergic granulomatous vasculitis-related scleritis prevention
Prior to the application of glucocorticoids, the disease was considered incurable, mainly due to congestive heart failure and myocardial infarction. Frequent asthma attacks and rapid progression of systemic vasculitis have a poor prognosis. The use of high-dose glucocorticoids, even with the addition of cyclic phosphate gel, has significantly improved the prognosis of this disease, and the 5-year survival rate has increased by more than 50% from 25%. For severe patients, intravenous injection of methylprednisolone 0.5~1g daily, after 3~5 days, changed to prednisone 40~60mg/d orally for 8 weeks, reduce the amount and maintain treatment. If the effect of hormone therapy alone is not good, cyclophosphoric acid or azathioprine can be added.
Complication
Allergic granulomatous vasculitis-related complications of scleritis Complication
Churg-Strauss syndrome is less common with polyarticular pain and arthritis, and central nervous system abnormalities such as seizures are rare.
Symptom
Allergic granulomatous vasculitis associated with scleritis symptoms Common symptoms Congestive nodules Heart failure granuloma Scleral outer inflammation
Eye performance
There are few ocular lesions in Churg-Strauss syndrome. Some patients may have necrotizing eosinophilic granulomatous lesions of the conjunctiva, cornea, uvea, retina and optic nerve, similar to other parts of the eye, occasionally scleritis or Scleral outer layer inflammation, sometimes eye abnormalities can be the first manifestation of the disease, further observation of these lesions can help early diagnosis and early treatment of Churg-Strauss syndrome, in Foster et al. Scleritis or sclera There are no cases of Churg-Strauss syndrome in the inflammation.
2. Non-eye performance
Allergic vasculitis is mainly caused by lung involvement, usually also the first manifestation, often with asthma or atopic lung disease, or both, with a history of pneumonia and bronchitis, after 8 years of asthma, when systemic symptoms occur Such as fever, discomfort and weight loss, it indicates that the onset of multiple system diseases such as skin, peripheral nerve, heart, gastrointestinal tract and kidney will occur. Other systemic clinical manifestations include:
1 skin damage, subcutaneous nodules, purpura and ulcers;
2 peripheral neuropathy, multiple mononeuritis;
3 heart involvement, congestive heart failure;
4 gastrointestinal involvement, stomach, small intestine, large intestine and other gastrointestinal infarction, ulcers and perforations;
5 eosinophilic granulomatous nephropathy, and involving the prostate and lower urethra.
The diagnosis of Churg-Strauss syndrome depends on the clinical manifestations and pathological findings, necrotizing small vasculitis, biochemical examination of eosinophilic vascular endovascular, external granuloma, and in line with multi-system clinical manifestations and laboratory findings Churg-Strauss syndrome can be diagnosed.
Examine
Examination of allergic granulomatous vasculitis-associated scleritis
1. Typical laboratory examination of Churg-Strauss syndrome: the result is an increase in eosinophil count, Churg and Strauss originally described patients with eosinophil counts of 5,000 to 20,000/mm3, with eosinophilic improvement with the disease Granulocytes can be reduced, sometimes elevated serum IgE, anemia and ESR increase.
2. Pathological examination: The affected lung, skin and peripheral nerves are the most common biopsy sites that are helpful for diagnosis.
Chest X-ray: can show easy dissipative or migratory pulmonary infiltration (Löffer syndrome), massive, nodular nodular nodular infiltration, or diffuse interstitial lung disease.
Diagnosis
Diagnosis and diagnosis of allergic granulomatous vasculitis related scleritis
The diagnosis of Churg-Strauss syndrome depends on clinical presentation and pathological findings. The necrotizing small vasculitis biopsy has eosinophilic vascular endovascular and extragranular granuloma, and can be diagnosed according to the multi-system clinical manifestations and laboratory findings in accordance with Churg-Strauss syndrome.
