Hamman-Rich syndrome is a fairly rare disease (the frequency of occurrence is only ten cases per hundred thousand people), manifested in the form of lung sclerosis and the formation of respiratory failure. Experts attribute its appearance to autoimmune processes.
Definition
Hamman-Rich syndrome is a disease associated with the pathological process in the lung tissue, which leads to its sclerosis and the development of respiratory failure. It began to be studied only in the middle of the last century, when publications with descriptions of similar symptoms became more frequent in scientific journals.
This pathology is more common in middle-aged and elderly men. It is believed that this may be due to bad habits, longer working experience and features of professions with inappropriate 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 they began to use it in a rather broad sense, designating a group of diseases that have similar symptoms. Therefore, a more specific name was needed.
The pathology was renamed into Scadding's disease, and then into Hamman-Rich syndrome, as it turned out that these two doctors described a similar pathology back in 1935. There are other synonyms of this nosological unit, for example, progressive interstitial pulmonary fibrosis, fibrotic dysplasia, lung cirrhosis, purulent interstitial pneumonia and others. In modern medicine, it is authorized to use all of them.
Etiology
Hamman-Rich syndrome is still one of the diseases with an unexplained etiology, since the fibrosis occurring in this disease is a non-specific reaction of the lung tissue to the pathological process. The theory that the disease is caused by an autoimmune process based on an allergic reaction of the third type (immunocomplex) has gained the most popularity in the scientific community.
There are proponents of a polyetiological theory. They argue that 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 previously considered Hamman-Rich syndrome as one of the options for collagenosis, in which the pathological process is limited only to the lungs.
Pathogenesis
Idiopathic fibrosing alveolitis (Hammen-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 pathological changes are. This process is not unique to this particular disease, but a typical response of the respiratory system to damage.
A decrease in gas diffusion through the alveoli occurs for several reasons. Firstly, this is a change in the thickness of the membranes and partitions, since the fibrinogen present in the plasma impregnates these areas, making them more dense and stiff. Secondly, the epithelium lining the respiratory sacs (alveoli) inside becomes cubic and loses its properties. Thirdly, the process of perfusion of gases is disrupted, that is, the air that enters the lungs does not come into contact with the circulatory system or this process occurs too quickly. In addition, due to increased pressure in the pulmonary circulation, a reflex spasm of the pulmonary vessels is observed, which further aggravates the pathophysiological mechanisms.
Symptomatology
Hamman Rich syndrome in children is quite rare. This disease mainly develops in middle-aged and elderly people, but it can also occur in premature babies with reduced compensatory capabilities.
The onset of the disease is slow. A person develops episodes of dry cough and a feeling of constriction in the chest. Then, shortness of breath on exhalation and a slight increase in temperature, weakness, rapid fatigue join this. At the initial examination, the blue tips of the fingers and the 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 heartbeat, small gurgling wheezing in the lungs. With the progression of the disease, cough episodes become more frequent, sputum appears, and in rare cases hemoptysis (if there is a rupture of the capillary).
Diagnostics
Diffuse interstitial pulmonary fibrosis (Hammen-Rich syndrome) can be detected using laboratory tests only in the terminal stages, since changes in the blood test will be non-specific:
- increased protein;
- ACE acceleration (erythrocyte sedimentation rate);
- change in the ratio of protein fractions.
These indicators indicate the presence of an inflammatory process, but do not report anything about its nature or location. A little radiography of the lungs may help. In the initial stages, an increase in the pattern at the root of the lungs is noted, then a mesh granularity of the lung tissue appears. Over time, areas of increased transparency appear on the images, which take the form of cells. The heart will also be changed by increasing the right departments.
Differential diagnosis
Hamman Rich's disease is a disease that is difficult to diagnose just because of the non-specificity of changes in the lungs. In order to 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 diseases of the connective tissue (lupus, rheumatoid arthritis, scleroderma, etc.);
- lung cancer
- hemosiderosis of the lungs;
- a group of pneumoconiosis.
Only if all of the above options are discarded, is Hammer-Rich syndrome diagnosed. Often it is necessary to conduct a pathological study. For this, the doctor prescribes an open or closed puncture biopsy, depending on the expected stage of the disease.
Pathomorphology
Symptoms and diagnosis of Hammen-Rich syndrome depend on how global changes have occurred in the lung tissue. In order to evaluate them, a piece of the lung is removed and examined under a microscope. The results of this study may not coincide with the clinical manifestations, but they are more accurate, since they directly reflect the state of the organ.
If the disease is at the stage of fibrosing alveolitis, then the pathologist will see tissue edema, a fluid with a high content of fibrin in the lumen of the alveoli, impregnation of the walls with cells of the inflammatory response. If the pathological process progresses, then histologically this is manifested by a change in the cellular structure of the endothelium of the alveoli and capillaries, a thickening of the basement membrane. Also possible partial destruction of the alveoli in places of their contact with the vessels.
There are five degrees of changes in the lungs:
- Edema and cell infiltration of the walls of the alveoli.
- Filling the alveoli with fluid and exfoliating cells or absorbing exudate with septa and densifying them.
- The destruction of the alveoli, the formation of cysts in the small bronchi.
- Multiple medium and small cysts.
- "Cellularity" of the lungs due to large cysts.
The course and forecast
Kelloidosis (Hammen-Rich syndrome) has a rather poor 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 win anyway. A patient can have from several months to several years of life. During this time, there will be exacerbations and periods of unstable remission.
Over time, progressive cardiopulmonary failure leads to disability, and then death from asphyxiation. In the best case, the patient will have another five to seven years from the time of diagnosis. Provided that he will comply with all the recommendations of the attending physician. In rare cases, the end occurs within 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 to save a person’s life and relative health.
Treatment
What should the patient do after he has been diagnosed with Hammen-Rich syndrome? Treatment begins immediately. Do not hesitate to start therapy, as the disease progresses more and more over the years. Specific treatment has not yet been developed, so doctors are trying to stop the symptoms and slow down the rate of tissue fibrosis.
In the initial stages of the disease, steroid drugs have proven themselves most effectively. They reduce inflammatory manifestations: fluid exudation, cell infiltration, slow down the formation of connective tissue and antibody production. Therapy is carried out in a bolus, that is, they begin with large doses (up to 60-80 mg per day) and gradually reduce the amount of the drug to 20 mg. It is important to monitor the activity of the adrenal cortex, so as not to cause Cushingoid syndrome.
In the later stages, when fibrosis is quite pronounced, preference is given to drugs that suppress immunity. Combination therapy from steroids and cytostatics is especially common. It turned out to be more effective than using these drugs separately.
As a symptomatic therapy, decongestants, oxygen inhalation under pressure, B vitamins are prescribed, and antibiotics (when a secondary infection is attached), cardiac glycosides and diuretics (with an increase in heart failure) are used as needed.