Wegener's granulomatosis, also known as granulomatosis with polyangiitis, is a rare and progressive disease that causes inflammation in blood vessels in different parts of the body, causing symptoms such as airway congestion, shortness of breath, skin lesions, nosebleeds, inflammations in ears, fever, malaise, loss of appetite or eye irritation.
As it is a disease caused by autoimmune changes, its treatment is done mainly with drugs to regulate the inume system, such as corticosteroids and immunosuppressants, and although there is no cure, the disease is generally well controlled, allowing a life normal.
Wegener's granulomatosis is part of a group of diseases called vasculitis, characterized by causing inflammation and damage to blood vessels, which can cause impairment in the functioning of various organs. Better understand the types of vasculitis that exist and how to identify them.
Main symptoms
Some of the main symptoms caused by this disease include:
- Sinusitis and nosebleeds; Cough, chest pain and shortness of breath; Formation of ulcers in the mucosa of the nose, which can lead to a known deformity with saddle nose; inflammation in the ears; Conjunctivitis and other inflammation in the eyes; Fever and night sweats; Tiredness and fatigue; Loss of appetite and weight loss; Joint pain and swelling in the joints; Presence of blood in the urine.
In rare cases, there may also be an impairment of the heart, leading to pericarditis or lesions in the coronary arteries, or also of the nervous system, leading to neurological symptoms.
In addition, patients with this disease have an increased tendency to develop thrombosis, and attention should be paid to symptoms that indicate this complication, such as swelling and redness in the limbs.
How to treat
The treatment of this disease includes the use of medicines that help to control the immune system, such as Methylprednisolone, Prednisolone, Cyclophosphamide, Methotrexate, Rituximab or biological therapies.
The antibiotic sulfamethoxazole-trimethoprim can be associated with treatment as a way to decrease relapses of some forms of diseases.
How the diagnosis is made
In order to diagnose Wegener's Granulomatosis, the doctor will evaluate the symptoms presented and the physical exam, which may give the first signs.
Then, to confirm the diagnosis, the main examination is to perform a biopsy of the affected tissues, which shows alterations compatible with vasculitis or necrotizing granulomatous inflammation. Tests may also be ordered, such as ANCA antibody measurement.
In addition, it is important that the doctor differentiates this disease from others that may have similar manifestations, such as lung cancer, lymphoma, cocaine use or lymphomatoid granulomatosis, for example.
What causes Wegener's granulomatosis
The exact causes that lead to the appearance of this disease are not known, however, it is known that it is related to changes in the immune response, which can be the components of the body itself or external ones that penetrate the body.
