Synonyms in a broader sense
Wegener’s disease, allergic angiitis and granulomatosis, Klinger-Wegener-Churg syndrome, Wegener’s granulomatosis, Wegener-Klinger-Churg giant cell granuloarteritis, rhinogenic granulomatosis
Definition
Wegner’s granulomatosis is a disease in which the small blood vessels become inflamed throughout the body (systemic vasculitis). This leads to the formation of tissue nodules (granulomas). Mostly the ears, airways, lungs and kidneys are affected. The blood vessels are altered necrotically and granulomatously.
EpidemiologyPopulation incidence
Wegner’s granulomatosis is relatively rare, occurring in about 5-7 people per 100,000. Women fall ill less frequently than men, the disease most frequently occurs around the age of 50, but cases have also been reported in which children and adolescents fall ill.
Symptoms
Wegner’s granulomatosis often begins with a persistent bloody cold, nosebleed and chronically congested nose. Sinusitis can also be associated with it. Inflammation of the middle ear (otitis media), earache, dizziness and even deafness can also occur both at the beginning and during the course of the disease.
In the course of the disease, hoarseness, dry cough, joint and muscle pain often occur. Inflammation of the renal vessels (glomerulonephritis) is also common in the course of the disease. In addition, there can be eye pain, inflammation of the eyes and even visual disturbances, as well as changes in the skin, such as bleeding, red spots and nodules.
An exact cause is not yet known. What is certain is that it is an inflammation of the vessels in the body (generalized vasculitis) caused by autoimmune antibodies. The associated antigen is not known, discussed are among other things inhaled allergens and an infestation with Staphylococcus aureus.
Diagnosis
Since the symptoms can vary greatly, the diagnosis of Wegener’s granulomatosis requires laboratory chemical, microscopic (histological) and clinical findings. A tissue sample (biopsy) can be taken, in which a microscopic examination reveals increased cells in a certain form (granulomas) and dying cells (necrosis). In the case of kidney involvement, a kidney biopsy can reveal typically altered kidney cells.
A blood test for certain autoantibodies (mostly c-ANCA, more rarely p-ANCA) is also required. These antibodies are directed against the body’s own white blood cells (leukocytes, especially neutrophil granulocytes). The abbreviation ANCA stands for Anti-Neutrophilic Cytoplasmic Antibody.
Inflammation markers (such as CRP) can also be elevated in the blood. Creatinine may also be elevated if Wegner’s granulomatosis has involved the kidney. An X-ray or CT scan often shows changes in the lungs (granulomas and scars).
