2023-12-28 11:33:21
Granulomatosis with polyangiitis is an uncommon disease that causes inflammation of the blood vessels in the nose, sinuses, throat, lungs and kidneys.
The disease, formerly called Wegener’s granulomatosis, is a group of blood vessel disorders called vasculitis. It causes slow blood flow to some organs of the body. Damaged tissue can develop areas of inflammation called granulomas, which may affect how these organs function.
But early diagnosis and treatment of granulomas with polyangiitis may lead to complete recovery. This disease may be fatal without treatment.
Symptoms
Signs and symptoms of granulomas with polyangiitis can appear suddenly or over several months. The first warning signs usually include inflammation of the sinuses, throat or lungs. The condition often worsens quickly; This affects the blood vessels and the organs that supply them with blood, such as the kidneys.
Signs and symptoms of granulomas with polyangiitis may include:
- Pus-like discharge from the nose with crusting, nasal obstruction, sinus infections, and nosebleeds.
- Cough, sometimes accompanied by bloody phlegm.
- Shortness of breath or wheezing.
- fever.
- Exhaustion.
- Joint pain.
- Numbness in the limbs, fingers or toes.
- Weight loss.
- Blood in urine.
- Skin sores, bruises or rash.
- Eye redness, burning sensation, or pain, as well as vision problems
- Ear infection and hearing problems
The disease affects only the lungs in some people. When the kidneys are affected, tests on blood and urine can detect the problem. If the disease is left untreated, kidney or lung failure may occur.
the reasons
The cause of granulomas associated with vasculitis is not known. It is not contagious, and there is no evidence that it is hereditary.
The condition can lead to inflammation, narrowing of blood vessels and the release of clumps of inflamed and harmful tissue (granulomas). Granulomas can destroy normal tissue, and narrowed blood vessels reduce the amount of blood and oxygen reaching the body’s tissues and organs.
Risk factors
Granulomas with polyangiitis may occur at any age. It often affects individuals between the ages of 40 and 65 years.
Diagnosis
The doctor will ask regarding the patient’s signs and symptoms, conduct a physical examination, and take a medical history.
Laboratory tests
Blood tests may check for:
- Signs of inflammation, such as high levels of C-reactive protein or an elevated erythrocyte sedimentation rate, commonly referred to as the erythrocyte sedimentation rate.
- Antineutrophil cytoplasmic antibodies, which appear in the blood of most people with active granulomatosis with polyangiitis.
- Anemia, common in people with this disease.
- Signs that the kidneys are not filtering waste from the blood properly.
Urine tests may reveal whether the urine contains red blood cells or an excessive amount of protein, which may indicate that the disease affects the kidneys.
Imaging examinations
A chest X-ray, CT scan, or MRI can identify which blood vessel or vessel is affected. It may also help your doctor monitor whether you are responding to treatment.
Biopsy
This is a surgical procedure in which the doctor takes a small sample of tissue from the affected area of your body. A biopsy can confirm the diagnosis of granulomas with polyangiitis.
Processing
Granulomas with vasculitis may be cured in a few months with early diagnosis and appropriate treatment. Treatment may include long-term prescription medication to prevent relapse. Even if the patient is able to stop treatment, he will need to be followed up regularly by a doctor, and perhaps by several doctors, depending on which organs are affected. To monitor the situation.
pharmaceutical
Corticosteroids such as prednisone help suppress the immune system and reduce inflammation of the blood vessels. Common side effects include weight gain, risk of infection, and osteoporosis.
Other medications that suppress the immune system include cyclophosphamide, azathioprine (Azasan, Imuran), mycophenolate (CellCept) and methotrexate (Trexall). Rituximab is another option for treating granulomatosis with polyangiitis. It is given by injection, usually combined with corticosteroids.
Once the condition is under control, the patient may remain on certain medications long-term to prevent relapse. These include rituximab, methotrexate, azathioprine, and mycophenolate.
Side effects of medications that suppress the immune system include an increased risk of infection. Cyclophosphamide can cause nausea, diarrhea, and hair loss. Your doctor may prescribe other medications to help prevent side effects from the prescribed treatment.
Plasma exchange
Also called plasmapheresis, this treatment removes the liquid portion of blood (plasma) that contains the substance that makes up the disease. The person will receive new plasma or a protein produced by the liver (albumin), which allows the body to produce new plasma. In people with chronic granulomatosis with polyangiitis, plasmapheresis can help the kidneys recover.
This content is from Mayo Clinic*
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