Generality
Fibrosis is the name that doctors use to indicate the abnormal formation of connective-fibrous tissue in an organ or in a tissue of the human body, without particular mention of the behavior of parenchymal tissue.
There are numerous types of fibrosis. Among the most well-known and common types, we note: pulmonary fibrosis, cardiac fibrosis, arthrofibrosis, Dupuytren's disease, cystic fibrosis, Peyronie's disease, gliosis, systemic nephrogenic fibrosis, etc.
Brief review of the meaning of parenchyma and fibrous tissue
In the medical field, the term " parenchyma " identifies the tissue that gives a certain organ its specific functional characteristics. To understand this statement, it is useful to give a couple of examples:
- The parenchyma of the heart is the set of myocardial myocytes;
- The parenchyma of the lungs includes the alveolar tissue, the cells that make up the bronchioles, the cells that make up the alveolar ducts, the so-called interstitium, etc.
In contrast, the term " fibrous tissue " and its synonym " connective-fibrous tissue " indicate a tissue lacking in function, composed of a cluster of collagen fibers .
The formation of connective-fibrous tissue is due to a hyperactivity of some particular cells, belonging to the so-called connective tissue and known as fibroblasts .
What is fibrosis?
Fibrosis is the medical term that identifies the anomalous formation of large quantities of connective-fibrous tissue in an organ, in a portion of an organ or in a particular tissue of the body, without any reference to the behavior of the parenchyma .
The processes of fibrosis alter the architecture and, consequently, also the function of the organ or tissue that strike.
Fibrosis can be the result of a pathological state, which involves the unusual deposition of connective-fibrous tissue ( pathological fibrosis ), or it can be the effect of normal healing mechanisms, which are triggered in an organ or in a tissue, on the occasion of recovery from injury or damage ( cicatricial fibrosis ).
Connective-fibrous tissue and scar tissue: are they the same thing?
The scar tissue that can be observed, after the healing of a deep cut on the skin, is an example of connective-fibrous tissue.
Therefore, scar tissue, fibrous tissue and fibrous connective tissue are three different ways of identifying the same thing.
In general, the term "scar tissue" is used when the formation of connective-fibrous tissue is dependent on the scarring of a lesion.
Fibrosis and fibroid
A fibroma is a benign tumor composed of the same cells that make up the fibrous connective tissue .
Fibroids result from particular fibrosis processes.
The medical conditions that are characterized by the presence of one or more fibroids are called fibromatosis .
Fibrosis, sclerosis and cirrhosis: the differences
It is a mistake to confuse fibrosis with sclerosis and cirrhosis . In fact, these three processes are similar, but they are not the same thing at all. Recalling that fibrosis refers to the disproportionate appearance of scar tissue without any reference to the parenchyma, the meanings of sclerosis and cirrhosis are reported below:
- With the term sclerosis, doctors indicate the hardening of an organ or a considerable part of it, due to the neoformation of connective-fibrous tissue and to the simultaneous regression of the normal parenchyma. In other words, when it is a victim of sclerosis, an organ or tissue sees its parenchymal component (ie the functional component) decrease and the connective-fibrous component abnormally increased.
An example of sclerosis is arteriosclerosis .
- With the term of cirrhosis, on the other hand, doctors mean an increase in the connective-fibrous tissue in an organ or part of it, associated with a reduction in parenchymal tissue and attempts by the latter to regenerate itself.
An example of cirrhosis is liver cirrhosis .
Examples
The fibrosis process can take place in various organs or tissues of the human body.
For example, it can affect: the lungs, the heart, the pancreas, some particular cells of the central nervous system, the most important joints, the hands, the skin, the mediastinum, the eyes, the penis, the retroperitoneum and the bone marrow.
Most known forms of fibrosis
The most known forms of fibrosis are:
- Pulmonary fibrosis and diseases that could precede it, such as pulmonary interstitial disease and pneumoconiosis ;
- Cardiac fibrosis ;
- Dupuytren's disease ;
- Peyronie's disease ;
- Arthrofibrosis ;
- Myelofibrosis ;
- Cystic fibrosis ;
- Retroperitoneal fibrosis ;
- Systemic nephrogenic fibrosis ;
- Gliosis ;
- The keloids ;
- Mediastinal fibrosis ;
- The adhesive capsulitis of the shoulder .
Pulmonary fibrosis
Pulmonary fibrosis is a respiratory disease, which arises due to the appearance of connective-fibrous tissue on the lungs, to be precise all around the pulmonary alveoli, that is the supporters of the withdrawal of oxygen from the inspired air.
The lungs of people with pulmonary fibrosis are not very elastic, hard and covered with retraent scars, which "crush" the alveoli, preventing their normal function and hindering the breathing process.
In pulmonary fibrosis, the formation of connective-fibrous tissue may depend on unrecognizable, unidentifiable factors; or it may depend on very specific factors, such as: prolonged exposure to toxic substances, anti-tumor radiotherapy, the use of chemotherapy drugs, some autoimmune diseases or some infectious diseases.
If the causes are not identifiable, one speaks more correctly of idiopathic pulmonary fibrosis ; if instead the causes are recognizable circumstances, one speaks more properly of secondary pulmonary fibrosis .
The typical symptoms of pulmonary fibrosis are: dyspnea, dry cough, recurrent sense of fatigue and weakness, weight loss without reason, chest pain and muscle and joint pain.
Diagnosis requires a fairly long examination.
Unfortunately, it is almost impossible to cure pulmonary fibrosis. The connective-fibrous tissue, in fact, has a permanent character and, to eliminate it, it would be necessary to resort to the difficult and dangerous intervention of lung transplant .
Therefore, the only treatments currently available are therapies with the aim of alleviating the symptoms and improving, as far as possible, the quality of life of the patients.
Cardiac fibrosis
Also known as myocardial fibrosis, cardiac fibrosis is the affection of the heart which is characterized by the presence of connective-fibrous tissue at the level of the myocardium .
Generally, in cardiac fibrosis, scar tissue appears due to cardiovascular distress (eg, hypertension, coronary artery disease, myocardial infarction, etc.).
Sufferers of cardiac fibrosis have a heart in which the muscles are more rigid and less contractile and where the heart valves are less efficient. This obviously has a negative effect on the blood pump function, performed by the body concerned.
Responsible for dyspnea, persistent cough, recurrent fatigue, weight loss etc., cardiac fibrosis can lead to the onset of heart failure .
Unfortunately, there are no cures able to restore the normal architecture of the myocardium; the only treatments available to patients, have the power to alleviate the symptoms and slow down the progression of heart failure, when it is established.
Dupuytren's disease
Also known as Dupuytren's contracture, Dupuytren 's disease is a disease affecting the hand, which is characterized by a process of fibrosis on the so-called palmar aponeurosis and the consequent permanent curvature of one or more fingers in the direction of the palm.
Who suffers from Dupuytren's disease, besides presenting one or more fingers curved towards the palm, complains:
- The presence of one or more nodules in the tendons of the fingers of the hand. These nodules are fibroids, therefore they depend on the process of fibrosis;
- Pain, itching and / or soreness at the palm of the hand;
- Difficulty in gripping objects and carrying out numerous activities that require the use of hands (ex: driving, playing a musical instrument, holding cutlery, etc.).
The diagnosis of Dupuytren's disease is simple, as the signs are unequivocal.
The therapeutic choice for the treatment of Dupuytren's disease is very wide: in fact, non-surgical treatments, such as radiotherapy and collagenase injections of Clostridium histolyticum, and surgical treatments with variable invasiveness, such as percutaneous fasciotomy with needle, exist palmar fasciotomy and fascectomy.
The adoption of a treatment rather than another depends exclusively on the severity of the symptomatology and on what emerged during the diagnosis.
Dupuytren's disease is a form of fibromatosis, called palmar fibromatosis .
Peyronie's disease
In the medical field, the term "Peyronie's disease" indicates an abnormal curvature of the penis, which can be seen above all during erections, which is due to the formation of connective-fibrous tissue within the cavernous bodies.
Also known as induratio penis plastic and considered one of the main causes of curved penis, Peyronie's disease is often associated with: pain in the penis (especially during erections), the presence of an unusual protuberance on the penis that can be felt, erection problems and reduction of penis size.
The precise factors triggering Peyronie's disease are few clear; according to most experts, the malformation in question would be the result of one or more traumas to the penis.
These traumas to the penis can take place during sexual intercourse or during road accidents, workplace accidents, sports injuries or incidental clashes with other people or objects.
The diagnosis of Peyronie's disease is quite simple, as the clinical signs are unequivocal.
The choice of the most appropriate therapeutic treatment depends on the severity of the disease: for less severe cases, a pharmacological treatment is recommended; while, for more severe cases, surgery is needed.
arthrofibrosis
Arthrofibrosis is the fibrosis of the joints, resulting almost always from traumas to them.
The joints that are victims of arthrophibrosis lose part of their mobility, are painful and swollen, and no longer fulfill their physiological functions.
Among the joints most prone to arthrofibrosis are: the knee, the shoulder, the hip, the ankle, the wrist and the articular elements that distinguish the spine.
Depending on the severity of the arthrofibrosis, the treatment can be conservative or surgical: it is conservative for the less severe cases, while it is surgical for the most serious circumstances or those that do not respond to conservative therapy.
In general, conservative treatment includes: physiotherapy, administration of NSAIDs against pain, cryotherapy and corticosteroid injections.
Surgical treatment, on the other hand, consists of an adhesiolysis procedure .
Myelofibrosis
Myelofibrosis is a serious malignant tumor of the bone marrow, which alters the process of hematopoiesis to the point of drastically reducing the levels of red blood cells, white blood cells and platelets, present in the blood.
It is one of the forms of fibrosis, since, in its presence, there is a progressive replacement of the sick bone marrow with connective-fibrous tissue.
From causes still unclear, myelofibrosis causes anemia, leukopenia and thrombocytopenia ; these three conditions are responsible for numerous symptoms, including chronic fatigue and weakness, shortness of breath, splenomegaly, hepatomegaly, pale skin, easy bleeding, night sweats, fever, recurrent infections and bone pain.
Healing from myelofibrosis is possible, but a bone marrow transplant is needed, a high-risk treatment for the patient with uncertain success.
For this reason, doctors have come up with alternative solutions, which although they do not allow for recovery, are nevertheless very effective in moderating symptoms and improving the quality of life of patients.
Cystic fibrosis
Cystic fibrosis is a serious hereditary disease, in the presence of which there is an overproduction of abnormal mucus (denser, more viscous and less smooth), by the exocrine glands such as the pancreas, the salivary glands, the sweat glands, intestinal glands and bronchial glands.
The abundance and atypical density of the mucus produced make the physiological outflow of the secretion, from the deputed glandular ducts, more complicated and less efficient; the accumulation of mucus from the ducts for excretion involves the occurrence of phenomena of glandular obstruction .
The glandular obstruction that characterizes cystic fibrosis is responsible for: intestinal and bronchial symptoms, ease of infection, decreased fertility in women, halitosis, liver cirrhosis, muscle pain, nausea, steatorrhea, fever, pharyngitis, etc.
Caused by a genetic mutation of the CFTCR gene, cystic fibrosis is included in the list of fibrosis because, during the course of the disease, an unusual connective-fibrous tissue forms on the pancreas.
Being an inherited genetic condition, cystic fibrosis is incurable; however, today the people who are affected can count on different drugs and therapeutic aids, able to alleviate the symptoms and reduce the risk of complications.
Retroperitoneal fibrosis
Retroperitoneal fibrosis is the pathological condition that is characterized by the formation of connective-fibrous tissue, at the level of the retroperitoneum .
In human anatomy, the retroperitoneum is the compartment of the body, located behind the peritoneum, which contains: the large vessels (eg: aorta and inferior vena cava), the kidneys, the adrenal glands, the pancreas, most of the duodenum, the colon ascending and descending, the rectum and the ureters.
In terms of symptoms, retroperitoneal fibrosis is typically responsible for: pain in the lower back, hypertension, renal insufficiency and deep vein thrombosis.
Treatment varies depending on the severity of the condition: in less severe cases, a glucocorticoid-based drug therapy is provided; in the most severe cases, however, surgical treatment is almost always necessary.
Systemic Nephrogenic Fibrosis
Systemic nephrogenic fibrosis is a rare, highly debilitating condition that results from the simultaneous formation of connective-fibrous tissue on the skin, joints, eyes and internal organs.
Due to the present connective-fibrous tissue, the aforementioned organs and structures of the body develop permanent damage, which affects functionally.
What makes the systemic nephrogenic fibrosis particularly interesting is the mechanism of onset, as it is very probably linked to the use of gadolinium contrast agents in patients with severe renal insufficiency (see magnetic resonance with contrast).
The typical clinical manifestations of systemic nephrogenic fibrosis consist of: presence of hard skin, widespread itching and burning skin, hyperpigmentation of the skin, reduced joint mobility, scleromyxedema, eosinophilic fasciitis, cardiorespiratory problems and liver problems.
Unfortunately, the numerous studies aimed at finding effective treatments against systemic nephrogenic fibrosis have not led to satisfactory results. Therefore, patients with systemic nephrogenic fibrosis are destined to live with this disease.
