Goodpasture Syndrome

What is Goodpasture Syndrome?

Goodpasture Syndrome is an immuno-inflammatory disease of the small vessels of the lungs and kidneys.

Symptoms of Goodpasture Syndrome

The classical triad expressing the clinical and pathogenetic essence of this disease is pulmonary hemorrhage, glomerulonephritis and antibodies to antigens of the main membrane of the capillaries of the lungs and kidneys. The disease is rare and can affect any age, but young men are more likely to get sick. The specific causes are unknown; The development of Goodpasture syndrome after a recent flu or the inhalation of hydrocarbons has been described more than once. In this regard, it is possible that such effects thus change the chemical structure of the antigens of the main membranes mentioned above, that they acquire auto-antigenic properties and cause the production of corresponding antibodies with pathogenic properties.

Using the immunofluorescence method, linear deposits of antibodies to the basement membranes are detected in the tissues of the kidneys, which in some cases are combined with deposits of the C3 complement fraction. At the same time, electron microscopic examination does not detect deposits of immune complexes. Circulating antibodies to the basal membranes of the tubules, glomeruli and pulmonary alveoli in the blood serum are found in more than 90% of patients (especially in the early stages of the disease), but they do not reflect the severity of organ changes or the general prognosis, thus having mainly diagnostic value. The level of serum complement and circulating immune complexes is normal. In a histological examination, the renal glomeruli in some patients may look normal, but in most cases there is a clear pathology – from focal proliferative changes to necrotic glomerulonephritis. The most frequently developed extracapillary “half moon”. In the lungs, hemorrhages are found in the cavity of the alveoli with or without alveolitis.

At the onset of the disease, clinical manifestations are primarily associated with lung damage – cough, shortness of breath, and especially hemoptysis; less common mild cyanosis. With auscultation, wet rales are possible. X-ray characteristic infiltrates of different sizes in both lungs, especially in the basal areas. Macrophages containing iron are usually found in sputum. Increased iron deposition in the lung tissue can be detected by scanning with ^ Re. Many patients have fever, joint pain, severe general weakness, but in some cases these symptoms are mild or absent. In most patients, from the first days or weeks of the disease, signs of glomerulonephritis are recorded. In blood tests, increased ESR and leukocytosis are detected; later, as a result of pulmonary bleeding, hypochromic anemia is possible.

The course of the disease is generally unfavorable, although not uniform. At the first stages, the main threat is pulmonary hemorrhage, and often the patient dies as a result of the first and only profuse bleeding. A number of survivors of this bleeding may develop a relative remission of the pulmonary process, but in many cases hemoptysis and pulmonary hemorrhages recur. Renal damage in some patients remains relatively mild, but in most cases it progresses rapidly with the development of oliguric renal failure, leading to death within a few months and even weeks. The average life expectancy in inadequately treated patients is less than six months.

Diagnostics of Goodpasture Syndrome

In the differential diagnosis of Goodpasture syndrome, it should be borne in mind that a combination of pulmonary hemorrhage with severe renal pathology (including kidney failure) can occur with Wegener’s granulomatosis, polyarteritis nodosa, SLE, hemorrhagic vasculitis, cryoglobulinemia, pulmonary artery embolism due to thrombosis, , diseases of legionnaires and even in severe circulatory failure with severe stagnation in the lungs and kidneys. However, all these diseases differ from Goodpasture syndrome in clinical signs and in the absence of antibodies to antigens of the basement membranes.

Treating Goodpasture Syndrome

Modern drug therapy has somewhat improved the prognosis of Goodpast syndrome. In threatening pulmonary hemorrhages, parenteral administration of methylprednisolone according to the type of pulse therapy (500-1000 mg per day) is indicated, after which they switch to long-term oral administration of corticosteroids (40-80 mg of prednisolone per day with a very slow and gradual dose reduction). In the absence of severe bleeding, steroids are prescribed orally from the start of treatment. There are indications of an increase in the effect in cases of their combination with prolonged use of immunosuppressants – azathioprine or cyclophosphamide, 150-200 mg per day. Significant improvement in renal changes and practical remission of the pulmonary process are described in patients who were prescribed repeated courses of plasmapheresis (with an exchange of 2-4 liters of plasma per day of the procedure) in combination with the drug treatment discussed above. A positive effect was observed even in patients with already formed proliferative changes (“half moon”) in the renal glomeruli. It should be remembered that with pulmonary hemorrhages, anticoagulants, which are prescribed for other vasculitis, are contraindicated. In patients with renal failure amid irreversible morphological changes (severe fibrosis, glomerular obliteration, tubular atrophy), improvement can only be expected as a result of regular hemodialysis or kidney transplantation (although glomerulonephritis subsequently does not exclude the development of a transplanted kidney). Summarizing, it should be recognized that modern therapy has led to some improvement in the course of Goodpast syndrome, but has not led to fundamental success. Nevertheless, it can be assumed that the disease is in principle reversible, in favor of which its individual spontaneous remissions testify.