GPA: severe damage to lungs, kidneys due to autoimmune disease

The autoimmune disease GPA – previously called Wegener’s granulomatosis – is a very serious disease and potentially life-threatening. Inflammation occurs through the mucous tracts and bloodstreams, mainly affecting the lungs and kidneys. The skin and eyes can also be affected, causing tissue to granulate, bleeding and ulcers to occur internally and externally. What does GPA mean, why is rapid diagnosis necessary and how can it be treated?

GPA: severe lung damage

  • Wegener’s granulomatosis
  • Systematic attack
  • Initial GPA complaints
  • Investigate autoantibodies
  • Prednisone treatment

Wegener’s granulomatosis

The disease was initially called Wegener’s granulomatosis, because it was discovered by the German pathologist Wegener. Granulomatosis means that tissue granulates due to inflammation. This is accompanied by a lot of pus. Because it often involves systematic damage to the body, the consequences are also dramatic for the person. The condition as discovered by Wegener affects various organs and can cause noticeable skin wounds. It falls under the group of autoimmune diseases, because the resistance affects foreign elements. What are the characteristic features of the disease?

Systematic attack

The inflammation can be so serious that it causes a lot of damage internally. It mainly occurs in the nose and lung, but can spread to other parts of the body. Think of the kidneys, skin, eyes, nerves and the fecal tract. It can occur in any part of the body, but in most cases it involves a growing inflammation of tissues and organs. The inflammation spreads quickly throughout the body, affecting the lining of veins and mucous membranes. Initially these are vague complaints, which are caused by the dormant condition. As the disease grows, the physical problems also increase.

Initial GPA complaints

Before the condition strikes as a major illness, there are recognizable signs for diagnosis. This concerns complaints such as persistent fever with a cold, which are accompanied by a decrease in weight. In addition, fatigue increases, partly due to deteriorated lung function. Due to increasing stress, acidification and inflammation, the joints are affected, causing the person to experience complete discomfort. In the subsequent stages, additional body parts will be affected. Think of the throat, eyes and nose where sinusitis with various bleeding conditions may apply. In three quarters of cases, the lungs, nose, throat and kidneys are affected. In a quarter of the cases there is 100% damage to the respiratory tract. The skin will also react due to vascular disorders, which may include blood spots, bruises, ulcers and pus. Sometimes it leads to large painful ulcerated open spots on the hands and legs together with nerve loss. Please note that the pericardium can also be affected by the condition. Life-threatening cardiac tamponade can be caused by pericarditis.

Investigate autoantibodies

Because the consequences of the disease are serious, it is necessary to recognize it quickly. The initial complaints in combination with progressive deterioration of body functioning should be taken seriously. There must be a medically appropriate response. The sooner the correct medication is administered, the better it is for the patient. To obtain a definitive answer, the ACPA test is carried out. This tests for the presence of anticytoplasmic antibodies. In ninety percent of the cases, the patient will carry these antibodies. However, there is also uncertainty in this, as the antibodies do not necessarily have to be present at an early stage of the disease. In other words, the disease must be recognized on the basis of the symptoms. Sometimes the diagnosis can take up to a year, because the symptoms are less severe at first and antibodies are not produced.

Prednisone treatment

If it is a mild variant of the disease, it is still a very serious disease. This must certainly be taken seriously, as functioning can still be significantly impaired. Long-term treatment with Prednisone together with methotrexate is standard practice, whereby the medication must be taken for several months in a row. Gradually the severity of the disease will decrease, and the medication will also be reduced. This process can take a year. If it concerns a very severe form of the disease, or systematic GPA, then every effort must be made to prevent death. In that case, high doses of Prednisone are combined with heavy immune system suppressants. If the disease decreases after the medication has taken effect, less severe drugs can also be chosen. The treatment process will last at least one and a half years, after which regular check-ups must also take place. Please note that the EGPA variant also exists and is ten times rarer than GPA.

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