Goodpasture Syndrome Causes Signs Diagnosis Therapy

The treatment of Goodpasture syndrome comes down to the exclusion of developing complications and their prevention. The provision of emergency care to patients is of no small importance. In the acute period of the syndrome, pulse therapy is used:

  • Methylprednisolone;
  • Cyclophosphamide with Methylprednisolone.

Symptomatic treatment of the syndrome includes:

  • plasmapheresis;
  • erythrocyte mass transfusion;
  • the use of iron preparations.

Marked deviations in the work of the kidneys require hemodialysis sessions. In terminal renal failure, nephrectomy followed by transplantation of a donor kidney may be recommended. However, this treatment option does not exclude the development of a relapse of necrotizing glomerulonephritis in the transplanted organ.

The course of the disease in most cases is steadily progressing, so the prognosis is not very encouraging. A diagnosis of Goodpasture syndrome in most cases is a sentence. The death of patients occurs due to profuse pulmonary hemorrhage, renal failure, respiratory failure. With a malignant form of the disease, a fatal outcome occurs in a few weeks.

1. Enhanced doses of immunosuppressants (drugs that reduce the activity of the immune system) and corticosteroids.

2. Plasmapheresis: hardware purification of blood outside the patient’s body with its subsequent return to the bloodstream.

3. Hemodialysis: purification and filtration of blood using an “artificial kidney” apparatus.

4. Necessary symptomatic therapy.

  • treatment regimen: 30 mg per 1 kg of body weight (intravenously, 20-30 minutes for the entire volume);
  • Duration: 3 days.

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2. Prednisolone (a synthetic glucocorticosteroid)

  • treatment regimen: from 0,25 to 2 mg per 1 kg of body weight;
  • Duration: for life with a gradual decrease in dosage in 12-month courses.

3. Azathioprine, imupran (immunosuppressants)

  • treatment regimen: 1-2 mg per 1 kg of body weight (when the level of GFR decreases to 10 ml/min., the dosage is halved);
  • Duration: according to clinical indications;
  • special instructions: regular monitoring of platelet and white blood cell counts is necessary; most often used as part of complex therapy (along with prednisone).

4. Indomethacin, methindole (anti-inflammatory non-steroidal agents)

  • treatment regimen: 2-3 times a day, 25-50 mg;
  • Duration: according to clinical indications.

5. Dipyridamole, chimes

  • treatment regimen: 150-400 mg per day;
  • Duration: 3-8 months.

Diagnostic and observation methods

The diagnosis is made on the basis of serological analysis to detect serum antibodies in the blood. In order to differentiate diseases similar in clinical manifestations, such as hemorrhagic vasculitis, systemic lupus erythematosus and Wegener’s syndrome, Goodpasture syndrome is diagnosed with the following methods:

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  • blood and urine tests;
  • verification of the main indicators of the immune system (immunogram);
  • lung and k >UZI pochek e1500287608532 - Goodpasture Syndrome Causes Signs Diagnosis Therapy

The main laboratory markers of Goodpasture syndrome are: a high titer of antibodies to blood serum (up to 1000 units/ml and above) during enzyme-linked immunosorbent assay and histology results from a kidney biopsy.

When the diagnosis is confirmed, a monitoring study is carried out for further therapy and observation. The fundamental ones are:

    Determination of the titer of antibodies to the basement membrane of the glomeruli of the k >Opredeleniya mocheviny e1500287279712 - Goodpasture Syndrome Causes Signs Diagnosis Therapy

Further studies of immune status are prescribed during therapy. If the patient reaches a stable level of recovery, he is monitored for follow-up every 3, 6, 12 months. Unfortunately, patients with Goodpasture Syndrome die within the first year of diagnosis. According to the observations of specialists, patients with remission underwent a course of specific immunosuppressive treatment.

Disease therapy

Immunosuppressive therapy is widely used in the practice of this syndrome. The duration of the course is strictly individual and takes from several months to 1 year or more. The general therapeutic program for Goodpast syndrome includes:

  • cytostatic treatment;
  • treatment with large doses of corticostero >Protsedura gemodializa e1500286897867 - Goodpasture Syndrome Causes Signs Diagnosis Therapy

Steroid preparations with hormones reduce the production of factors that damage cells and vascular walls. Cytotoxic drugs are aimed at eliminating immune cells in severe forms of the disease. If it is impossible to reduce the production of immune complexes, the method of blood purification is used – plasmapheresis. Hemodialysis with Goodpasture syndrome with a predominance of renal failure is required to filter and remove toxic substances from the blood.

Goodpasture Syndrome needs further study and observation. It requires attention from patients, despite the fact that pathology is growing steadily. The prognosis of the course and outcome of the disease is unfavorable, since the main reason for the death of patients is pulmonary bleeding, the development of renal failure. In order to prevent the development of the disease, it is necessary to remember the typical manifestations, undergo a comprehensive examination in time, fight bad habits, and treat acute viral infections to the end.

Goodpasture syndrome – what is it in simple words?

Goodpasture Syndrome is one of the varieties of pulmonary-renal syndrome of autoimmune origin. Pathology is accompanied by the simultaneous development of alveolar bleeding along with glomerulonephritis. Both of these pathologies are provoked by the presence in the bloodstream of specific antibodies to the glomerular basement membrane.

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With Goodpasture syndrome, the patient’s body is exposed to antigens of the alveolar capillaries on circulating antibodies. In the bloodstream, they are recorded in individuals with a hereditary predisposition to the disease. More often, the pathology manifests itself in carriers of the HLA-DRw15, -DR4 and -DRB1 alleles.

Antibodies bind to the basement membranes, fix a compliment, causing an inflammatory response at the cellular level. These changes lead to the development of glomerulonephritis and/or inflammation of the pulmonary capillaries. Goodpasture syndrome develops with its characteristic symptoms and manifestations.

The etiological mechanisms that provoke Goodpasture’s disease have not been reliably identified by doctors. Ongoing observations of patients with this pathology indicate the relationship of the syndrome with the previous viral infection. When collecting an anamnesis, it turns out that the patient recently had the flu, hepatitis A.

Among other provoking factors, doctors distinguish:

  • taking individual drugs (carbimazole, penicillamine);
  • work in hazardous work: continuous inhalation of organic solvents, gasoline, varnishes.

Under the influence of one of the etiological factors, with a change in the stability of the immune system in the body, the synthesis of autoantibodies to the basement membranes of the alveoli of the lungs and renal glomeruli begins. The structural component a-3 of the type IV collagen chain, which is located in the membranes of the pulmonary and renal capillaries, is supposedly an antigen.

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Svetlana Borszavich

General practitioner, cardiologist, with active work in therapy, gastroenterology, cardiology, rheumatology, immunology with allergology.
Fluent in general clinical methods for the diagnosis and treatment of heart disease, as well as electrocardiography, echocardiography, monitoring of cholera on an ECG and daily monitoring of blood pressure.
The treatment complex developed by the author significantly helps with cerebrovascular injuries and metabolic disorders in the brain and vascular diseases: hypertension and complications caused by diabetes.
The author is a member of the European Society of Therapists, a regular participant in scientific conferences and congresses in the field of cardiology and general medicine. She has repeatedly participated in a research program at a private university in Japan in the field of reconstructive medicine.