Wegener’s granulomatosis is a rare autoimmune disease that causes polyangiitis, i.e. inflammation of blood vessels in different parts of the body. Depending on the degree of inflammation and the affected site, necrosis, which is tissue death, may still occur.
Symptoms of Wegener’s granulomatosis depend on the affected site but usually include shortness of breath, skin lesions, nosebleeds, inflammation of the ears and eyes, fever and general malaise, for example.
As it is an autoimmune disease, its treatment is mainly done with drugs to regulate the immune system response, such as corticoids and/or immunosuppressants, which manage to control the symptoms, allowing you to lead a normal life.
The main symptoms of Wegener’s granulomatosis are:
- Nosebleeds, nose pain and sinusitis;
- Cough, chest pain and/or shortness of breath;
- Ulcers on the mucous membrane of the nose;
- Inflammation of the ears;
- Conjunctivitis and other eye inflammations;
- Fever and night sweats;
- Tiredness and fatigue;
- Loss of appetite and weight loss;
- Joint pain and joint swelling;
- Presence of blood in the urine.
In rare cases, there may also be involvement of the heart, leading to pericarditis or coronary lesions, 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.
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 to components of the body itself or external components that penetrate the body.
How to confirm the diagnosis
The diagnosis of Wegener’s granulomatosis is usually made by an immunologist, who evaluates the symptoms and performs a physical examination.
Then, to confirm the diagnosis, the main exam is to perform a biopsy of the affected tissues, which demonstrates alterations compatible with vasculitis or necrotizing granulomatous inflammation. Exams, such as ANCA antibody dosage, may also be requested.
In addition, it is important for the doctor to differentiate this disease from others that may have similar manifestations, such as lung cancer, lymphoma, cocaine use or lymphomatoid granulomatosis, for example.
How the treatment is done
The treatment of Wegener’s granulomatosis includes the use of drugs that help control the immune system, such as Methylprednisolone, Prednisolone, Cyclophosphamide, Methotrexate or Rituximab, in addition to some biological therapies.
The antibiotic sulfamethoxazole + trimethoprim may also be indicated in some cases, as it helps prevent secondary infections and control symptoms, although the reason for this is not yet known.