Hammain-Rich Syndrome: Description

Hammain-Rich syndrome is a fairly rare disease (the incidence of only ten cases per hundred thousand people), manifested in the form of lung sclerosis and the formation of respiratory failure. Experts associate his appearance with autoimmune processes.

Definition

Hammain-Rich syndrome is a disease associated with a pathological process in the lung tissue that leads to its sclerosis and the development of respiratory failure. The study began only in the middle of the last century, when in scientific journals, publications with descriptions of similar symptoms became more frequent.

This pathology is more common in middle-aged and elderly men. It is assumed that this may be due to bad habits, longer work experience and specialties of occupations with unsuitable working conditions (welders, miners, builders, etc.).

Synonyms

The doctor who first began to study this pathology was Scudding. He proposed a term such as "fibrosing alveolitis". But very soon it began to be used in a rather broad sense, referring to a group of diseases with similar symptoms. Therefore, a more specific name was needed.

The pathology was renamed Scudding's disease, and then into the Hammain-Rich syndrome, as it turned out that the two doctors described a similar pathology back in 1935. There are other synonyms of this nosological unit, for example, progressive interstitial lung fibrosis, fibrotic dysplasia, cirrhosis of the lung, non- pulmonary interstitial pneumonia and others. In modern medicine, it is legal to use them all.

Etiology

Hammain-Rich syndrome is still one of the diseases with an unknown etiology, since the phenomenon of fibrosis occurring in this disease is a nonspecific reaction of the lung tissue to the pathological process. The greatest popularity in the scientific community was the theory that the disease is caused by an autoimmune process based on the allergic reaction of the third type (immunocomplex).

There are supporters of the polyethiologic theory. They argue that the lung tissue is exposed to several agents of a bacterial, fungal or viral nature, which leads to a hyperergic reaction of the immune system.

A number of scientists have previously considered the syndrome of Hammen-Rich as one of the variants of collagenosis, in which the pathological process is limited only to the lungs.

Pathogenesis

Idiopathic fibrosing alveolitis (Hammain-Rich syndrome) appears due to the formation of the so-called alveolar-capillary block. The rate of diffusion of gases into the blood and, as a consequence, hypoxia depends on how pronounced the pathological changes are. This process is not unique to this particular disease, but a typical response of the respiratory system to damage.

Reduction of diffusion of gases through the alveoli occurs for several reasons. Firstly, this is a change in the thickness of membranes and partitions, since fibrinogen present in the plasma impregnates these areas, making them more dense and rigid. Secondly, the epithelium lining the respiratory sacs (alveoli) from the inside becomes cubic and loses its properties. Thirdly, the process of perfusion of gases is broken, that is, air that enters the lungs, does not come into contact with the circulatory system, or this process takes place too quickly. In addition, due to increased pressure in the small circle of blood circulation, a reflex spasm of the lung vessels is observed, which further exacerbates pathophysiological mechanisms.

Symptomatics

The syndrome of Hammam-Rich in children is rare. This disease mainly develops in middle-aged and elderly people, but it can also be in preterm infants with reduced compensatory possibilities.

The onset of the disease is slow. The person has episodes of dry cough and a feeling of squeezing in the chest. This is followed by shortness of breath on exhalation and a slight rise in temperature, weakness, rapid fatigue. At a primary examination, blue fingertips and a nasolabial triangle are clearly visible. There may be a change in the terminal phalanges of the fingers and the shape of the nails.

There is an accelerated palpitation, small gurgling rales in the lungs. With the progression of the disease, episodes of coughing become more frequent, sputum appears, and in rare cases hemoptysis (if there is a capillary rupture).

Diagnostics

Diffuse interstitial lung fibrosis (Hammain-Rich syndrome) can be detected using laboratory studies only in terminal stages, since changes in the blood test will be non-specific:

• Increase of proteins;
• Acceleration of SSE (sedimentation rate of erythrocytes);
• Change in the ratio of protein fractions.

These indicators indicate the presence of an inflammatory process, but nothing is reported about its nature or location. A little help can be seen from the radiography of the lungs. At the initial stages, there is an increase in the pattern at the root of the lungs, then a mesh grit of the lung tissue appears. Over time, the images appear areas of increased transparency, which take the form of cells. The heart will also be changed by increasing the right divisions.

Differential diagnostics

Hammer-Rich's disease is a disease that is difficult to diagnose due to the nonspecific changes occurring in the lungs. In order to accurately be sure of the diagnosis, you need to exclude everything else.

The doctor needs to make comparisons:

• With allergic and toxic fibrosing alveolitis;
• Bilateral pneumonia;
• Sarcoidosis;
• Disseminated pulmonary tuberculosis;
• Systemic connective tissue diseases (lupus, rheumatoid arthritis, scleroderma, etc.);
• Lung cancer;
• Hemosiderosis of the lungs;
• Group of pneumoconiosis.

Only if all the above options are discarded, the Hammer-Rich syndrome is diagnosed. Often it is necessary to carry out pathoanatomical research. For this, the doctor prescribes an open or closed puncture biopsy, depending on the intended stage of the disease.

Pathomorphology

Symptoms and diagnosis of the Hammain-Rich syndrome depend on how much the global changes have occurred in the lung tissue. In order to evaluate them, extract a piece of the lung and examine it under a microscope. The results of this study may not coincide with the clinical manifestations, but they are more accurate, because they reflect directly the condition of the organ.

If the disease is in the stage of fibrosing alveolitis, the pathologist will see tissue swelling, a fluid with a high content of fibrin in the lumen of the alveoli, impregnation of the septa by cells of the inflammatory response. If the pathological process progresses, then histologically it is manifested by a change in the cellular structure of the endothelium of the alveoli and capillaries, a thickening of the basal membrane. Also possible partial destruction of the alveoli in the places of their contact with the vessels.

There are five degrees of changes in the lungs:

1. Edema and cell infiltration of the walls of the alveoli.
2. Filling of alveoli with liquid and exfoliated cells or absorption of exudate by septa and their compaction.
3. The destruction of the alveoli, the formation of cysts in the small bronchi.
4. Multiple medium and small cysts.
5. "Cellularity" of the lungs due to large cysts.

Current and forecast

Kelloidosis (Hammain-Rich syndrome) has a rather unfavorable prognosis, since it is impossible to stop the progression of the disease. Even with medication, the pathological process can be slowed down, but sooner or later the disease will still win. The patient may be from several months to several years of life. During this time there will be exacerbations and periods of unstable remission.

Over time, progressive cardiopulmonary insufficiency leads people to disability, and then to death from asphyxiation. At best, the patient will have another five to seven years from the date of diagnosis. Provided that he will comply with all the recommendations of the attending physician. In rare cases, the end comes in a couple of months, regardless of the therapy. If treatment is prescribed in the early stages of the disease, then in rare cases it is possible to "conserve" it at this stage and save a person's life and relative health.

Treatment

What should the patient do after he was diagnosed with the Hammain-Rich syndrome? Treatment begins immediately. You should not hesitate with the beginning of therapy, as the disease progresses with age more and more. Specific treatment has not yet been developed, so doctors try to stop the symptoms and slow down the rate of fibrosis of the tissue.

At the initial stages of the disease, steroid drugs were most effective. They reduce inflammatory manifestations: exudation of fluid, cellular infiltration, slow down the formation of connective tissue and the production of antibodies. Therapy is performed bolus, that is, start with large doses (up to 60-80 mg per day) and gradually reduce the amount of the drug to 20 mg. It is important at the same time to monitor the activity of the adrenal cortex in order not to cause the Cushing's syndrome.

In the later stages, when fibrosis is sufficiently pronounced, preference is given to drugs that depress immunity. Combined therapy from steroids and cytostatics was especially popular. It proved to be more effective than using these medications separately.

As symptomatic therapy, decongestants, oxygen inhalations under pressure, B vitamins are prescribed, antibiotics (when secondary infection is attached), cardiac glycosides and diuretics (with the increase of heart failure) are used as needed.