Causes and risk factors
The exact cause is unknown. It is caused due to an abnormal reaction of immune system to some inflammation. It affects blood vessels from ears, nose, sinuses, lungs and kidneys. Constriction of vessels lead to formation of harmful inflamed tissue masses called granulomas. It occurs usually between age 40 and 60 years.
Clinical presentation
At first, Wegener’s granulomatosis involves only respiratory tract. Eventually, the condition worsens rapidly, affecting blood vessels and the organs they supply. Signs and symptoms such as persistent coryza, sinusitis, otitis, epistaxis, redness or pain in eyes, is present. Pain in the chest, coughing, haemoptysis, shortness of breath is observed. Patient presents with loss of weight, malaise, pain and swelling in joints, fever, and heamaturia.
Investigation
Medical history by the patient and Clinical examination by the doctor helps in diagnosis. Blood tests to detect anti-neutrophil cytoplasmic autoantibodies [ANCA] which are present in most people suffering from this disease. Other blood tests include ESR, Complete blood count, Serum creatine level. Urine analysis is recommended. Chest X-ray is advised. Biopsy of the affected tissue confirms the diagnosis.
Treatment
Medications include drugs to suppress the immune system such as corticosteroids .To treat kidney damage; plasmapheresis or a kidney transplant may be advised.
