What Is Wegener's Granulomatosis?

Wegener's granulomatosis is a rare disease with necrotizing vasculitis and granulomatous inflammation as the main pathology. The cause is unknown, but it is believed that when the upper respiratory tract infection occurs, bacteria or viruses act to form antigens and cause allergic reactions to cause the disease. After 1970s, combined treatment with corticosteroids and immunosuppressive agents has achieved significant results.

Wegener's granulomatosis

Wegener's granulomatosis is a rare necrotizing

A rare disease with necrotizing vasculitis and granulomatous inflammation as the main pathology. It was named because German pathologist F. Wegner first reported it in 1936. The disease affects the upper and lower respiratory tract, kidneys and other organs. Occurs in adults. The condition is serious and can cause death if not treated. The basic pathological changes are necrotizing vasculitis (fibrinoid degeneration, necrosis, and multiple inflammatory cell infiltration in the vessel wall) and necrotizing granulomatosis (necrosis in the central area of the lesion with cell infiltration around it). The cause is unknown, but it is believed that when the upper respiratory tract infection occurs, bacteria or viruses act to form antigens and cause allergic reactions to cause the disease. In animal models, electron microscopy revealed that the basic lesions of the disease are necrotizing vasculitis and necrotizing granulomas caused by an immune complex.

The clinical symptoms are complicated. At the beginning, the upper respiratory tract symptoms are the main symptoms. Erosion, ulcers, and necrosis appear in the nose and pharynx, pus and blood or necrotic tissue flow out, granulomas appear in the maxillary sinus, which can damage the bone in the sinus wall. At the same time, purple scars and erythema can appear on the skin. , Blood blister, nodule, and ulcer necrosis. Oral mucosa also often has ulcers and necrosis. Ocular symptoms are also common, mainly manifested as exophthalmos and eyelid edema, conjunctivitis, keratitis caused by exophthalmos; granulomatous compression of the optic nipple can cause optic nipple edema and atrophy, leading to vision loss or loss. Following the symptoms of the upper respiratory tract, symptoms may also appear in the lungs, mainly cough, shortness of breath, and chest pain. X-rays show single or multiple nodular infiltrations, often bilateral, and cavity formation is not uncommon, often misdiagnosed as tuberculosis.

Nephritis often appears later in the course of the disease and is seen in more than 80% of patients. The severity is different. The milder is focal glomerulonephritis. There is a small amount of red blood cells and protein in the urine. If proliferative glomerulonephritis and interstitial glomerulonephritis occur, the symptoms are severe and the disease develops rapidly.

The main basis for the diagnosis of this disease is: upper respiratory symptoms. Nasal and sinus mucosal necrosis, X-rays show bone destruction in the sinuses. Pulmonary symptoms. X-rays showed flaky infiltrated nodules and voids. a variety of skin damage. Biopsy showed necrotizing vasculitis and necrotizing granulomatous changes. symptoms of renal inflammation.

The average survival time of this disease is only about six months before the application of corticosteroids and immunosuppressants. Since the treatment with corticosteroids has improved, the treatment is mainly cytotoxic drugs, such as cyclophosphamide, supplemented with corticosteroids, the efficacy is greatly improved. Experience has shown that the prognosis of this disease depends on whether the above treatment is obtained early, so early diagnosis and early treatment are the key to managing this disease.