Granulomatosis With Polyangiitis (Gpa)

(Wegener’s granulomatosis, Wegener’s granulomatosis)

The granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis) is characterized by necrotizing granulomatous inflammation, vasculitis of small and medium vessels and focal necrotizing glomerulonephritis, often with crescent formation. Typically, the upper and lower respiratory tract and the kidneys are affected, but any organ can be affected. Symptoms vary, depending on the affected organs and systems. Patients presenting with symptoms in the upper and lower respiratory tract (eg. As recurrent nasal discharge or epistaxis, cough), followed by hypertension and edema, or with symptoms that result from a multi-organ involvement. The diagnosis requires a biopsy evaluation generally. The treatment consists of corticosteroids plus immunosuppressants. Remission is usually possible, although recurrences are common.

The incidence of granulomatosis with polyangiitis (GPA) is 1: 25,000, whites are most often affected, but the disease occurs in all ethnic groups and at any age before. The average age of onset is 40 years.

The granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis) is characterized by necrotizing granulomatous inflammation, vasculitis of small and medium vessels and focal necrotizing glomerulonephritis, often with crescent formation. Typically, the upper and lower respiratory tract and the kidneys are affected, but any organ can be affected. Symptoms vary, depending on the affected organs and systems. Patients presenting with symptoms in the upper and lower respiratory tract (eg. As recurrent nasal discharge or epistaxis, cough), followed by hypertension and edema, or with symptoms that result from a multi-organ involvement. The diagnosis requires a biopsy evaluation generally. The treatment consists of corticosteroids plus immunosuppressants. Remission is usually possible, although recurrences are common. The incidence of granulomatosis with polyangiitis (GPA) is 1: 25,000, whites are most often affected, but the disease occurs in all ethnic groups and at any age before. The average age of onset is 40 years. The cause is unknown, immunological mechanisms play a role. Most patients with active generalized disease have anti-neutrophil cytoplasmic antibodies (ANCA). Pathophysiology Characteristically, granulomas form with histiocytic epithelioid cells and often with giant cells. Plasma cells, lymphocytes, neutrophils and eosinophils are present. The inflammation affects both the tissue and the blood vessels. The vasculitis may represent a small or large component in the disease. Micronecroses, usually with neutrophils (Microabscesses) occur early. The micronecroses develop into Makronekrosen. A central area of ??necrosis (so-called. Geographical necrosis) is surrounded by lymphocytes, plasma cells, macrophages and giant cells. A zone fibroblastic proliferation with picket shaped histiocytes may surround the area. On the nose there is a nonspecific chronic inflammation and necrosis. The lungs show most likely the entire spectrum of histopathological abnormalities, but the diagnostic criteria can be usually not identify small tissue samples as they are taken at a transbronchial biopsy. In the kidneys of the most common finding is a proliferative, progressive focal glomerulonephritis with necrosis and thrombosis of the individual loops or larger segments of the glomerulus. Vasculitic lesions and disseminated granuloma occur only occasionally. Symptoms and signs The disease may begin insidiously or acutely, to develop the full disease can take years. Some patients initially show symptoms in the upper and lower respiratory tract, at some point, the kidneys are affected later. In other patients, the disease is relatively acutely with systemic manifestations. Multiple organs and systems such. As the upper respiratory tract, the peripheral nervous system (leading to multiple mononeuropathy [mononeuritis multiplex]), the kidneys (leading to glomerulonephritis) and the lower respiratory tract (leading to hemorrhage, lung nodules, cavities or a combination) are affected simultaneously. Upper respiratory tract: It occurred sinus pain, bloody-serous or purulent discharge and nosebleeds on. The mucous membrane appears granulated (like cobblestones) and is fragile; Ulcers, thick dark crusts and septum are frequent. A nasal Chondritis with swelling, pain and collapse of the nose bridge (saddle nose) may occur. Patients experience recurrent sinusitis, which responded inadequately to multiple antibiotics applications and necessitated one or more sinus surgery prior to diagnosis. Secondary infections (eg., By Staphylococcus aureus) can develop. A subglottic stenosis may develop, leading to symptoms such as pain in the larynx hoarseness, shortness of breath, wheezing and stridor. Ears: otitis, sensorineural hearing loss, dizziness and Chondritis may occur. Middle ear, inner ear and mastoid are commonly affected. Eyes: The eyes appear red and swollen. Inflammation and obstruction of the nasolacrimal duct, conjunctivitis, scleritis, uveitis or retinal vasculitis may also occur. Inflammatory infiltrates in the retro-orbital space (orbital pseudotumor) may cause proptosis, compression of the optic nerve and blindness. An extension to the eye muscles leads to double vision. If serious eye symptoms develop, immediate evaluation and treatment is necessary to prevent permanent vision loss. Lower respiratory tract: Respiratory manifestations are common. An inflammation of the large bronchi and branches leading to localized wheezing, pneumonia and atelectasis postobstruktiver. Single or multiple pulmonary nodules with or without cavitation and parenchymal infiltrates sometimes cause symptoms such as chest pain, shortness of breath and productive cough. Dyspnea with bilateral infiltrates with or without hemoptysis may indicate alveolar hemorrhage and must be clarified immediately. Heart: Only rarely leads to coronary heart disease. Musculoskeletal: Patients presenting with muscle pain, joint pain or nonerosiver inflammatory arthritis. Skin: leukocytoclastic vasculitis, subcutaneous nodules, papules, livedo reticularis or pyoderma gangrenosum druckdolente may develop. Nervous system: Vasculitis leads to ischemic peripheral neuropathy, brain lesions or an extension of the lesions to adjacent regions. Lesions that have their origin in the paranasal sinuses or middle ear can be extended directly to the retropharyngeal region and the base of the skull, resulting in cranial neuropathy, exophthalmos, diabetes insipidus or meningitis. Kidney: It symptoms and complaints develop a glomerulonephritis. The urinary sediment may be abnormal, and serum creatinine may increase rapidly. It develop edema and hypertension. A rapidly progressive glomerulonephritis, which is life threatening, can develop. Venous System: A deep vein thrombosis can affect the lower extremities when the GPA is active. Other organs: Occasionally occurs an inflammatory mass in breast, kidney, prostate or other organs. Cutaneous vasculitis (Lower Extremities) © Springer Science + Business Media var model = {thumbnailUrl: ‘/-/media/manual/professional/images/391_leukocytoclastic_vasculitis_slide-9_springer_high_de.jpg?la=de&thn=0&mw=350’ imageUrl: ‘/ – /media/manual/professional/images/391_leukocytoclastic_vasculitis_slide-9_springer_high_de.jpg?la=de&thn=0 ‘, title:’ Cutaneous vasculitis (Lower extremities) ‘description:’ u003Ca id = “v37892955 ” class = “”anchor “” u003e u003c / a u003e u003cdiv class = “” para “” u003e u003cp u003eDiese figure shows multiple ecchymosis and purpura on the legs. u003c / p u003e u003c / div u003e ‘credits ‘© Springer Science + Business Media’

Health Life Media Team

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