What Is Churg-Strauss Syndrome?

Churg-Strauss syndrome-related scleritis is a disease in which patients can develop necrotizing eosinophilic granulomatous lesions of the conjunctiva, cornea, uvea, retina, and optic nerve.

Churg-Strauss syndrome-related scleritis

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Churg-Strauss syndrome-related scleritis is a disease in which patients can develop necrotizing eosinophilic granulomatous lesions of the conjunctiva, cornea, uvea, retina, and optic nerve.

Allergic granulomatous vasculitis (AGA), also known as Churg-Strauss syndrome, is a vascular disease similar to nodular polyarteritis (PAN), which can affect any organ of the body, but is similar to typical PAN The ratio has the following characteristics: More than 90% of patients invade the pulmonary blood vessels; Vasculitis affects small and medium arteries and veins; Intravascular and extravascular granuloma formation; Eosinophil infiltration; Asthma and peripheral blood Increased acid granulocytes; This disease is not related to hepatitis B.

Similar to other areas of the eye, scleritis or episcleritis can occasionally occur. A combination of prednisone and cyclophosphamide (CTX) can be used. Early diagnosis and early treatment are beneficial for timely recovery and can significantly improve the prognosis.

Churg-Strauss syndrome-related scleritis

allergic granulomatous angiitis associated scleritis

Churg-Strauss syndrome-related scleritis; allergic granulomatous vasculitis-related scleritis

Ophthalmology> Sclerosis> Non-infectious scleritis

H15.0

Allergic granulomatous vasculitis is a rare disease. The age of onset is from 15 to 70 years. Males are twice as many as females. There is no regional or ethnic difference.

The cause is unknown.

The pathogenesis is related to the immune response. Circulating immune complex (CIC) can be measured in the blood, and immunoglobulin and complement deposition can be measured in the vessel wall. Although CIC is the main mechanism causing granulomatous vasculitis, cellular immunity can also cause it. Granulomatous reactions are generally delayed-type hypersensitivity reactions, but CIC itself can stimulate the formation of granulomas, thereby producing granulomatous vasculitis. Therefore, the immune response to granulomatous vasculitis can be single or more than one. The occurrence of vasculitis is probably related to many host factors, such as genetic factors, immune regulatory mechanisms and the integrity of the reticuloendothelial system. The reticuloendothelial system removes complexes from the circulation. Vasculitis caused by some complexes rather than other vascular involvement may be related to the size and physical and chemical properties of CIC, but also to other physical factors such as turbulence of blood flow, hydrostatic pressure in blood vessels, and pre-existing vascular endothelial damage. related.

Churg-Strauss syndrome is less prone to polyarticular pain and arthritis. Central nervous system abnormalities such as seizures are rare.

Chest X-ray: can show dissipative or migratory lung infiltration (L? Ffer syndrome), massive, nodular nodular infiltration, or diffuse interstitial lung disease.

The diagnosis of Churg-Strauss syndrome depends on clinical manifestations and pathological findings. Necrotizing small vasculitis, eosinophilic necrotizing intravascular and extragranular granuloma in biopsy tissue, and consistent with multi-system clinical manifestations and laboratory results, Churg-Strauss syndrome can be confirmed.

A combination of prednisone and cyclophosphamide (CTX) was used. With prednisone 1mg / (kg · d), the dose was reduced after 2 months; CTX 2mg / (kg · d), the reduction was maintained after remission, and both were administered for at least 1 year. If the patient is intolerant to CTX, other immunosuppressants can be used including azathioprine, methotrexate (MTX), and cyclosporin A.

Early diagnosis and early treatment are beneficial for timely recovery and can significantly improve the prognosis.

Prednisone, cyclophosphamide, azathioprine, methotrexate

Circulating immune complex, eosinophil count