Wegener's granulomatosis - Granulomatosis with polyangiitis - Symptoms and causes скачать в хорошем качестве

Wegener's granulomatosis - Granulomatosis with polyangiitis - Symptoms and causes

Granulomatosis with polyangiitis, previously known as Wegener's granulomatosis. Represents long term systemic disorder, which causes formation of granulomas and inflammation of blood vessels. Wegener’s granulomatosis affects small and medium size blood vessels. Signs and Symptoms: First symptom in most people is nose irritation and inflammation. Sinuses also involved. Nose associated signs and symptoms include: Crusting around the nose, stuffiness, nosebleeds, runny nose, and saddle-nose deformity due to a hole in the septum of the nose. Ears: conductive hearing loss due to auditory tube dysfunction, sensorineural hearing loss (unclear mechanism) Kidneys: rapidly progressive glomerulonephritis, leading to chronic kidney disease. Kidney involvement also common. Around 75 % of cases. Oral cavity: strawberry gingivitis, underlying bone destruction with loosening of teeth. Trachea: subglottal stenosis Lungs: pulmonary nodules (referred to as "coin lesions"), infiltrates (often interpreted as pneumonia), cavitary lesions, bleeding in the lungs causing a person to cough up blood, and rarely bronchial stenosis. Arthritis: Pain or swelling (60%), often initially diagnosed as rheumatoid arthritis. Skin: subcutaneous nodules (granulomas) on the elbow, purpura, various others (see cutaneous vasculitis) Nervous system: occasionally sensory neuropathy (10%) and rarely mononeuritis multiplex Heart, gastrointestinal tract, brain, other organs: rarely affected. Granulomatosis with polyangiitis can occur at any age. It most often affects people between the ages of 40 and 65. The cause of Wegener’s granulomatosis is unknown, although microbes, such as bacteria and viruses have some role. Genetics may have some influence but not Hight risk inheritance. Classic microscopically inflammation of blood vessels associated with poorly formed granulomas, necrosis, and many giant cells. Bacterial colonization with Staphylococcus aureus has been hypothesized as an initiating factor of the autoimmunity seen in people with GPA. Wegener’s granulomatosis is rare disease, occurs around 10 new cases per million in Europe. In Asians much rare. Diagnosis: Granulomatosis with polyangiitis is usually suspected only when a person has had unexplained symptoms for a long period of time. Determination of anti-neutrophil cytoplasmic antibodies (ANCAs) can aid in the diagnosis, but positivity is not conclusive and negative ANCAs are not sufficient to reject the diagnosis. More than 90% of people who have GPA test positive for ANCA. biopsy is obtained from the kidneys. On rare occasions, thoracoscopic lung biopsy is required. Granulomatosis with polyangiitis is part of a larger group of vasculitic syndromes called systemic vasculitides or necrotizing vasculopathies, all of which feature an autoimmune attack by an abnormal type of circulating antibody termed ANCAs (antineutrophil cytoplasmic antibodies) against small and medium-size blood vessels. Treatment: The standard treatment for severe GPA is to induce remission with immunosuppressants such as rituximab or cyclophosphamide in combination with high-dose corticosteroids. Plasmapheresis is sometimes recommended for very severe manifestations of GPA, such as diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. Oral and intravenous cyclophosphamide are both effective for induction of GPA remission. Oral cyclophosphamide at a dose of 2 mg/kg/day was the standard treatment for many years; this regimen resulted in complete remission in more than 75% of people with GPA but is associated with significant toxicities including infertility, inflammation and bleeding from the bladder, and bladder cancer. In contrast, administering pulsed doses of intravenous cyclophosphamide is equally effective for inducing remission, results in a lower cumulative dose, and decreases the incidence of abnormally low white blood cell counts by one-third. Without treatment prognosis is poor. 2-year survival under 10% and average survival five months. Death usually resulted from uremia or respiratory failure. With corticosteroids and cyclophosphamide, 5-year survival is over 80%. Long-term complications are common (86%), mainly chronic kidney failure, hearing loss, and deafness. By Tom Buur - Own work, CC BY-SA 4.0, https://commons.wikimedia.org/w/index... By Vw38 - Own work, CC BY-SA 4.0, https://commons.wikimedia.org/w/index... By Nephron - Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index... By https://wellcomeimages.org/indexplus/... https://wellcomeimages.org/indexplus/... Collection gallery (2018-04-03): https://wellcomecollection.org/works/... CC-BY-4.0, CC BY 4.0, https://commons.wikimedia.org/w/index...