Application of plasmapheresis in the complex therapy of idiopathic fibrosing alveolitis

Idiopathic fibrosing alveolitis (IFI) is one of the most common and, at the same time, poorly understood diseases from the group of interstitial lung diseases. Idiopathic fibrosing alveolitis is characterized by inflammation and fibrosis of the pulmonary interstitium and air spaces, disorganization of the structural and functional units of the parenchyma, which leads to the development of restrictive changes in the lungs, impaired gas exchange, progressive respiratory failure and, ultimately, to the death of the patient.

In the study of the pathogenesis of idiopathic fibrosing alveolitis, most researchers are currently inclined to an autoimmune reaction in combination with a viral infection. The presence of rheumatoid and antinuclear factors in the blood of patients with idiopathic fibrosing alveolitis, an increased amount of circulating immune complexes, y-globulins, as well as the detection of histiolymphocytic infiltration in the interstitium of the lungs indicate immune disorders in this disease.

In the basic treatment of idiopathic fibrosing alveolitis, long-term use of anti-inflammatory drugs capable of affecting the immunological links of pathogenesis is currently actively used: corticosteroids and cytostatics. However, it should be recognized that modern medicine does not yet have effective methods of therapy for idiopathic fibrosing alveolitis. The entire arsenal of drug therapy used has virtually no effect on the prognosis of the disease.

The disease is extremely severe, accompanied by increasing respiratory failure, which in most cases leads to death.
In this regard, the development of new methods and approaches to treatment is very relevant. Extracorporeal methods of treatment are often used as additional means of anti-inflammatory action.

In the State Autonomous Healthcare Institution of the Republic of Tatarstan, when treating patients with idiopathic fibrosing alveolitis, we actively use the method of combining basic therapy with a course of plasmapheresis operations. Over the past 10 years, 480 plasmapheresis operations have been performed in the Gravitational Blood Surgery Office (GBSRO) on 91 patients with IFA aged from 22 to 70 years, including 64 women and 27 men. All patients received basic therapy according to the recommendations of the European Respiratory Society (ERS) and the American Thoracic Society (ATS) (2000), which included glucocorticosteroids 0.5-1.0 mg/kg per day in terms of prednisolone; in case of a pronounced tendency to fibrosis, cytostatics were additionally prescribed - azathioprine 2-3 mg/kg per day, maximum daily dose - 150 mg or cyclophosphamide 2 mg/kg per day, maximum daily dose - 150 mg.

Plasmapheresis operations were performed using a multifunctional centrifuge with automatic cooling SORVAL RS ЗС PLAS and on PCS 2 - Hemonetics devices.

The plasmapheresis course consisted of 2-3 operations with intervals of 2 to 4 days. The volume of plasma exfusion during one procedure was 35-50% of the circulating plasma volume, which was replaced in a moderate hypervolemic regime using 0.9% sodium chloride solution and rheopolyglucin in a ratio of 2:1.

The course of plasmapheresis operations was combined with basic therapy with glucocorticosteroids (GCS) and cytostatics (azathioprine or cyclophosphamide). Repeated courses were carried out after 4-6-12 months, i.e. the patients were on "programmed" plasmapheresis.

As a result, the following was noted:

• reduction of clinical manifestations - reduction of weakness, shortness of breath, cough, increased tolerance to physical activity;
• improvement of the indices of external respiration function, diffusion capacity of the lungs, and blood gas composition - an increase in the indices of forced expiratory volume in the first second (FEV1) by 12.7% of the initial value, vital capacity of the lungs (VC) by 9.2% of the initial value, and an increase in the level of blood saturation (SPO2);
• positive dynamics on X-rays and computed tomography (CTG) scans of the lungs - slowing down or stopping the fibrosis of lung tissue;
• reduction of the dose of basic therapy drugs;
• stabilization of the process - reduction or cessation of disease progression.

There is no need to expect the reverse development of already existing organic lung lesions - fibrosis, but it is quite possible to influence the initial stages of the disease - alveolitis and interstitial edema. With the removal from the body of both primary agents toxic to alveolar structures and, obviously, secondary products of the immune response, one can expect an improvement or, at least, a cessation of the spread of pathological processes in the lung parenchyma.

Clinical experience confirms these assumptions, plasmapheresis courses improve the gas exchange function of the lungs, slow down its progression with a significantly lower level of drug support with hormonal and cytostatic drugs. According to our observations, with "programmed" plasmapheresis, such results are achieved much faster using smaller doses of basic drugs.

This allows us to recommend “programmed” plasmapheresis in cases of pronounced inflammatory infiltration of lung tissue, detected on radiographs and computed tomography (CTG); with long-term administration of large doses of glucocorticosteroids and/or cytostatics, as well as in the absence of effect from drug therapy.

"Programmed" plasmapheresis in fibrosing alveolitis increases the effectiveness of standard drug anti-inflammatory therapy and allows to reduce its volume, reduce tolerance to drugs, almost completely avoid the prescription of cytostatics, which promptly prevents exacerbations and improves the quality of life, and even maintains the ability to work of patients. The overall life expectancy of these patients also increases significantly with complex therapy, including plasmapheresis.

Anesthesiologist-resuscitator of the gravitational blood surgery room Olga Vladimirovna Sagitova. The use of plasmapheresis in the complex therapy of idiopathic fibrosing alveolitis // Practical Medicine. 8 (64) December 2012 / Volume 1