Fibrothorax

Diseases of the pleural cavity are a frequent complication that develops after various bronchopulmonary pathologies. One of these complications can be fibrothorax - we are talking about an obliterating fibrous process in the pleural cavity. Obliteration is formed due to the accumulation of fibrous mass released from liquid pleural secretions, as well as fibrinous lumps with hemothorax.

Fibrothorax can be the result of exudative pleurisy (in particular, tuberculous), pleural empyema, various injuries or operations on the chest.

Simply put, fibrothorax is a filling (overgrowth) of the pleural cavity with a high probability of further ossification. Normal sliding of the pleural sheets becomes difficult, and the lungs during inhalation lose the ability to fully open. The process is accompanied by severe pain, it can spread to the lung tissue: while the organs inside the chest are able to move to the affected side. [1]

Epidemiology

Respiratory pathologies are among the most common groups of diseases. Over the past few years, their share has grown by about 10%. Experts associate such an increase in morbidity with a deterioration in the socio-economic situation and the environment, a decrease in living standards, and activation of migration.

In the vast majority of cases, fibrothorax acts as a complication of other diseases. The frequency in the morbidity structure as a whole is estimated at about 2-4%. Among patients of surgical clinics, this indicator may be somewhat high, however, such statistics are currently lacking. [2]

Causes fibrothorax

Fibrothorax in the overwhelming majority of cases develops as a complication of other pathologies of the respiratory system and, in particular, the pleura. So, often the initial causes of fibrothorax are:

• inflammation of the pleura (dry or exudative);
• empyema of the pleura;
• aseptic pleurisy with inflammation of the pancreas;
• tumor process (mesothelioma);
• cancer metastases;
• penetrating chest injuries;
• pulmonectomy (complete or partial);
• pleural tuberculosis;
• pleurisy resulting from autoimmune processes.

The initial signs of fibrotic disorder usually appear several months after the underlying disease. For example, fibrothorax after pulmonectomy can develop approximately 6-18 months after surgery.

As we have already noted, fibrothorax practically does not occur as an independent pathology, but acts as a complication of other diseases. Such provoking diseases can be both pulmonary disorders and other diseases that do not directly damage the lung tissue.

Risk factors

Fibrothorax is a complex and multifactorial disease. The most significant predisposing factors for the development of pathology are:

• prolonged or frequent inflammatory pathologies of the respiratory system;
• metabolic disorders (first of all - diabetes mellitus, obesity);
• elderly age;
• cardiac insufficiency, impaired left ventricular function;
• acute renal failure, acute stages of chronic renal failure; [3]
• patients who are on hemodialysis;
• pathology of the peripheral vascular network;
• lung surgery (in particular, pulmonectomy).

The above points refer to the so-called general risk factors.

In addition, the risk of developing fibrothorax increases with respiratory failure, prolonged artificial pulmonary ventilation against the background of a long stay in the intensive care unit.

Pathogenesis

Fibrothorax is an obliteration of the pleural cavity with adhesions and connective fibrous tissue, as a result of which the respiratory system loses the ability to perform its functions. Most often, the problem arises in the form of a long-term effect of pulmonectomy.

Another common cause of the development of pathology are inflammatory pleural processes - in particular, empyema, hemothorax. As a result of such reactions, massive pleural moorings are formed, and the size of the lung is significantly reduced due to pleurogenic fibrosis.

The pleural cavity of a healthy person is an anatomical slit-like space bounded by the parietal and visceral pleural sheets (serous membrane). Normally, this space should not contain any inclusions. However, under the influence of certain etiological factors, pathological changes occur in the pleura, which entails increased formation of fibrosis and filling (fusion) of the cavity.

Symptoms fibrothorax

The symptomatology of fibrothorax is nonspecific, which presents some difficulties for the diagnosis of the disease. In general, the clinical picture can be reflected in the following symptoms:

• Signs of pleural involvement:
  • chest pain;
  • decrease in the volume of the affected side of the chest, respiratory delay of one half;
  • retraction of the spaces between the ribs.
• Signs of respiratory distress:
  • Difficulty breathing even with minimal physical activity
  • cyanosis of the digital phalanges and nails, as well as the area of the nasolabial triangle;
  • increased fatigue, feeling of weakness.

The severity of symptoms can be different, depending on the scale of the pleural lesion and on the presence of complications in the form of respiratory failure. Most often we are talking not about one, but about several obvious signs that form the corresponding syndrome.

Fibrothorax syndrome as a whole is a collection of symptoms that are united by one pathogenetic mechanism. Combinations of such symptoms are different within the above manifestations.

The initial manifestations of fibrothorax can be as follows:

• the patient complains of intrathoracic pain, aggravated by coughing, as well as shortness of breath of varying intensity;
• with severe fibrothorax, the patient has tachycardia, dizziness, chest pains, pale skin, blue circles under the eyes;
• if fibrothorax develops against the background of an infectious-inflammatory process, then there is an increase in temperature, chills, general weakness, intoxication signs.

Regardless of the scale of the progression of the disease, all patients have some degree of respiratory failure and chest pains. However, these symptoms cannot be called specific, so an X-ray examination or computed tomography must be performed to make a diagnosis.

Changes in the chest, characteristic of fibrothorax

Chest examination	Retraction and respiratory delay of the affected side, retraction of the spaces between the ribs at the time of inhalation.
Palpation examination	Voice tremor is weak or (-).
Percussion	Dull or dull sound.
Auscultation	Breathing is weak or not heard, pleural noise is present, bronchophonia is weak or (-).
X-ray	The focus of fibrothorax is displayed as a homogeneous darkening.

• Right-sided fibrothorax

Fibrothorax can equally often occur on the left and right. With a right-sided pathological process, shortness of breath is often the leading symptom - both during physical activity and in a quiet period. With the progression of the disease, other symptoms appear, such as tachycardia, edema in the lower extremities, swelling and pulsation of the venous vessels of the neck, pain behind the sternum on the right side. Patients complain of severe weakness, fatigue, and inability to exercise.

• Left-sided fibrothorax

Left-sided localization of fibrothorax often requires differential diagnosis with diseases not only of the respiratory, but also of the cardiovascular system. Patients may complain of heart pain radiating to the left shoulder or left side of the abdomen.

Not uncommon with left-sided fibrotic lesions - heart palpitations and shortness of breath. The pain is usually exacerbated by coughing, sneezing, and sudden body movement.

Forms

Fibrothorax can be classified according to a number of characteristics that depend on a specific criterion.

Depending on the causative factor, the disease is:

• postoperative (as a result of pulmonectomy);
• pathological (due to another underlying disease).

Depending on the development of the inflammatory process, fibrothorax can be:

• growing;
• stable.

If we take into account the localization of pathology, then there are fibrothorax of the following types:

• apical;
• interlobar;
• paracostal;
• supraphrenic;
• paramediastinal.

Idiopathic or primary fibrothorax is a type of disease that occurs for no apparent reason.

Complications and consequences

The main adverse effects of fibrothorax are:

• chronic respiratory failure (oxygen deficiency in the body);
• pulmonary pressure increase;
• chronic cor pulmonale;
• secondary infection and the development of an inflammatory process in the lungs.

During the development of the previous inflammatory process and fibrothorax, the vasculature and the heart may shift to the side. This condition is threatened by increased chest pressure and impaired cardiac blood flow. In this situation, it is important to prevent the formation of cardiopulmonary failure: if displacement of organs is detected, the patient is recommended to undergo an emergency operation.

The likelihood of adverse effects and complications depends on the degree and extent of fibrothorax. When pathology is detected at later stages, they often talk about an unfavorable further development of events. Timely access to doctors and adequate treatment increases the patient's chances of relative recovery, namely, inhibition of fibrosis processes. However, even a recovered patient needs regular medical supervision.

Diagnostics fibrothorax

The main research methods for suspected fibrothorax are:

• Basic methods:
  • general examination of the patient;
  • examination of the chest area;
  • palpation method;
  • percussion research;
  • listening (auscultatory method).
• Additional methods:
  • chest x-ray;
  • analyzes of blood and sputum discharge.

Fibrothorax is usually characterized by patient complaints of shortness of breath, chest pain. On general examination, attention is drawn to the paleness of the skin, cyanosis. [4]

During a medical examination, a sinking of the affected side of the chest, rapid shallow breathing, lagging of the affected side with a deep breath is found.

What percussion sound appears with fibrothorax? As a rule, there is a dullness of the percussion sound.

On auscultation, breathing is weak, vesicular, or not at all amenable to listening. Pleural friction noise is noticeable. Bronchophonia is weak or absent.

On palpation, there is a sharp weakening of voice tremor, or its absence. [5]

Blood tests, sputum discharge do not undergo any specific changes.

Instrumental diagnostics is represented mainly by plain X-ray or tomography of the chest cavity. In order to differentiate fibrinous accumulations and fluid secretions in the pleura, some patients undergo a pleural puncture. This diagnostic procedure involves performing a puncture with pumping out the intracavitary contents. Then the material is sent for laboratory research for the presence of infectious signs, atypical cells or fibrin filaments. [6]

The scale of further measures depends on the results of the initial diagnosis.

• Fibrothorax on x-ray

X-ray picture with fibrothorax is displayed in the form of a homogeneous shadow. There is a reduced and to one degree or another intense darkening of the affected lung field, high standing of the diaphragmatic dome, obliterating the closure of the bone-diaphragmatic sinuses, shift of the mediastinal organs to the affected side. The appearance of zones of pleural calcification is possible.

There is a noticeable thickening of the surfaces of the pleura, areas with formed adhesions.

X-ray signs of fibrothorax can be supplemented by tuberculous foci in the lungs, pleura, and bone tissue. Sometimes it is possible to fix the presence of calcifications - dense deposits of calcium salts. [7]

Differential diagnosis

Fibrothorax should be distinguished from such pathologies:

• focal or lobar compaction of the lung tissue;
• cavities in the tissues of the lungs;
• obstructive or compression atelectasis;
• hydrothorax;
• pneumothorax;
• exudative narrowing of the bronchi;
• bronchial obstruction;
• respiratory distress syndrome.

Treatment fibrothorax

It is impossible to cure fibrothorax with medication, regardless of the stage of the disease. There are no such drugs that can absorb fibrous growths and cleanse the pleural cavity, restoring the functionality of the pleura.

Nevertheless, drugs for fibrothorax are prescribed - first of all, in order to influence the original cause of the problem.

For example, if a chronic microbial disease was the precursor of the disease, then antibiotics are appropriate, in accordance with the resistance of the identified bacteria.

With an intense inflammatory process, non-steroidal anti-inflammatory drugs can be used.

At the initial development of the tumor, chemotherapy and other appropriate treatment are prescribed, depending on individual indications.

With an initial inflammatory process of rheumatic or other autoimmune etiology, the use of corticosteroids is appropriate.

If the root cause of fibrothorax is no longer relevant, or it is not possible to establish it, then with a stable deterioration in the dynamics of pathology, they resort to the help of a surgeon. An operation cannot be dispensed with if there is an obvious compression of the lung - in a similar situation, decortication, or pleurectomy, is performed. The intervention is the removal of pleural areas to release the lung, which makes it possible to restore its function.

For patients who develop fibrothorax after pulmonectomy, additional intervention is inappropriate. Therefore, in such situations, the emphasis is on symptomatic therapy. The main tasks of such treatment are as follows:

• minimize the manifestations of shortness of breath;
• optimize the functionality of a healthy lung;
• unload the cardiovascular apparatus.

Medicines

Medicines will not be able to eliminate the developing fibrous process - for this it is necessary to perform an operation, which is not always safe and appropriate. But this fact does not mean that drug treatment is not carried out: drugs are prescribed to improve the patient's quality of life, to relieve symptoms.

• To combat the infectious and inflammatory process with fibrothorax, antibiotics are prescribed, which have a broad spectrum of antibacterial activity. These drugs include Ceftriaxone, Doxycycline, Azithromycin. Often, drugs with anti-tuberculosis action are also used - in particular, Isoniazid, Rifampicin, etc.

Ceftriaxone	A cephalosporin antibiotic of the 3rd generation with a prolonged action and a broad spectrum of activity. The drug is used on average 1-2 g every 24 hours, for several days (usually no more than a week). The most common side effects of Ceftriaxone are diarrhea, skin rashes, elevated liver enzymes, eosinophilia, and leukopenia.
Azithromycin	Representative of macrolide antibiotics, characterized by a wide range of antibacterial activity. The drug is taken once a day, every day, between meals. The duration of treatment is determined by the doctor (usually 3-5 days). The likelihood of developing side effects is low. In rare cases, dyspepsia, skin rash, vaginitis, neutropenia are possible.

• To eliminate symptoms of intoxication, oral and intravenous drip rehydration, taking anti-inflammatory and antipyretic drugs (acetylsalicylic acid, Paracetamol, Ibuprofen, etc.) are appropriate.

Paracetamol	An analgesic and antipyretic agent that can be taken 1-2 tablets 3 times a day. It is advisable to maintain an interval between doses of at least 4 hours. Adverse reactions during treatment with Paracetamol are very rare: it can be allergies, nausea, increased activity of liver enzymes.
Regidron	Oral rehydration solution is used to restore fluid-electrolyte balance and correct acidosis. Regidron is taken according to the doctor's prescription. Side effects are extremely unlikely.

• To support hepatic function, hepatoprotectors (Essentiale, Karsil, Hepabene) are prescribed, and in more complex cases of fibrothorax, corticosteroid drugs.

Essentiale forte N	Hepatoprotective drug, which is taken 2 capsules three times a day, with food. Duration of admission is 2-3 months, at the discretion of the doctor. Possible side effects: digestive disorders, rarely an allergic reaction.
Prednisolone	A hormonal glucocorticoid drug with anti-inflammatory, anti-allergic, desensitizing and immunosuppressive effects. The dosage is set individually. The treatment is completed by gradually decreasing the dose. With prolonged use of the medication, vision may be impaired, dyspepsia, sleep disturbances, thrombosis, changes in blood pressure, and disorders of the nervous system may appear.

Physiotherapy treatment

The use of oxygen for therapeutic and prophylactic purposes is called oxygen therapy. This method is especially important in fibrothorax, as it helps to replace the lack of oxygen in the tissues.

Against the background of oxygen therapy, patients have an increase in the oxygen content in the plasma, breathing improves, the level of oxyhemoglobin in the blood rises, and metabolic acidosis decreases by minimizing the amount of under-oxidized products in the tissues.

Oxygen therapy is not able to stop the progression of fibrothorax, but it significantly improves the quality of life of patients. As a result of treatment, well-being significantly improves, sleep is normalized. Both stationary and mobile devices can be used to perform the procedure, which greatly simplifies the situation.

In addition to oxygen treatment, the patient is recommended to master the technique of correct breathing, and breathing exercises are prescribed to improve pulmonary ventilation.

Surgery

All patients in whom fibrothorax causes compression of the lung are prescribed an operation called decortication pleurectomy. Surgery is the removal of a portion of the pleura necessary to release the lung and restore its function.

During pleurectomy, part of the parietal and visceral pleura is removed. With a lateral approach, the doctor performs tissue dissection with resection of the fifth to sixth rib. Part of the pleura is exfoliated in a blunt manner, separated from the lung, and then removed. [8]

In order to fully expand the lung, the surgeon makes decortication - removal of fibrous accumulations. The chest cavity is drained with a pair of drains.

As a result of the intervention, the affected lung straightens, the respiratory system begins to function normally.

If fibrothorax develops after pulmonectomy, then surgical intervention is not performed due to inappropriateness. In such a situation, symptomatic supportive therapy is prescribed - in order to reduce shortness of breath and reduce the load on the cardiovascular apparatus.

Prevention

Fibrothorax formation can be prevented in many cases. To minimize the likelihood of the development of a pathological process, it is necessary to heed the following recommendations:

• promptly consult a doctor for the diagnosis and treatment of respiratory disorders;
• strengthen immune defenses, avoid hypothermia;
• give up bad habits - in particular, smoking;
• avoid passive inhalation of cigarette smoke;
• to avoid chemical damage to the respiratory organs, if necessary, use personal protective equipment;
• timely eliminate any infectious and inflammatory processes in the body;
• eat well, lead an active lifestyle, avoid hypodynamia;
• systematically undergo comprehensive preventive diagnostics of the body, annually make a fluorographic image of the lungs.

Forecast

With a timely arrest of the development of fibrothorax, the prognosis can be called more or less favorable. The likelihood of complications of the pathology is assessed in accordance with the severity of the initial disease, the age and general health of the patient, as well as the degree of spread of the fibrotic process.

Most often, the phenomenon of fibrosis progresses non-linearly with time. The more stages fibrothorax goes through, the more severe its course and worse prognosis.

If a patient is diagnosed with fibrothorax, treatment should always be prescribed by a qualified physician. However, in this situation, there are no therapeutic standards. Since in many cases the development and progression of the disease is preceded by inflammatory processes, anti-inflammatory drugs may be prescribed. Corticosteroid agents are indicated only for the treatment of patients with autoimmune processes and acute intoxication of the respiratory system.

A favorable outcome of the disease can be said when carrying out decortication of the lung. After such intervention, the patient can actually "forget" about the problem and continue to live absolutely fully. If we consider the non-surgical management of patients diagnosed with fibrothorax, then even with minimal severity of respiratory failure, there is a danger of further progression of the pathology. The most unfavorable outcome is observed in patients who fell ill after pulmonectomy, or with obvious intense respiratory failure.