Generalities and Classification
"Connective tissue" is a generic term used to indicate a set of different autoimmune diseases, characterized by inflammation of the connective tissue.
To be precise, some of the diseases classified as connectivities also involve tissues other than the connective tissue, such as the muscular or epithelial ones. Therefore, in these cases, the term "connective tissue" acquires an even broader and more general meaning.
However, the connective tissues can be divided into three macro-groups, depending on the symptoms, more or less defined, that they are able to cause. In this regard, we can distinguish:
- Differentiated or defined connectivity : group to which different pathologies characterized by a well-defined clinical picture belong.
- Undifferentiated connectivity : characterized by a symptomatology that does not allow to identify a specific and well-defined form of connectivity.
- Mixed connective tissue disease: characterized by the simultaneous presence of symptoms belonging to different types of autoimmune rheumatic diseases.
Differentiated connectivity
The differentiated (or defined, if you prefer) connectivities include a group of pathologies characterized by particular and specific clinical manifestations, which allow a certain diagnosis to be made.
Among the most well-known pathologies belonging to the group of differentiated connective tissues, we recall:
- Systemic sclerosis (or scleroderma ), characterized by symptoms such as thickening of the skin of the fingers, hands, arms and face, swelling of the joints, hair loss, heartburn, shortness of breath, skin xerosis, Raynaud's syndrome.
- Systemic lupus erythematosus, characterized by symptoms such as asthenia, fever, anorexia, myalgia, "butterfly" erythema, alopecia.
- Polymyositis, characterized by the appearance of asthenia, atrophy and muscle paralysis, hyposthenia, joint and muscle pain, heart disease, Raynaud's syndrome.
- Dermatomyositis, characterized by symptoms such as myalgia, muscular atrophy, muscle soreness, scleroderma, appearance of red spots on the eyelids, face, back, hands and chest.
Other diseases that fall into the group of differentiated connectivities are rheumatoid arthritis and Sjögren's syndrome .
However, for more information about these diseases, we recommend reading the dedicated articles already on this site.
Undifferentiated connectivity
Undifferentiated connective tissue is so defined because it presents a set of clinical manifestations that are not sufficient to accurately establish the type; therefore, it is not possible to perform a certain and well-defined diagnosis.
Undifferentiated connective tissue is usually not symptomatic and non-evolutionary, but this should not be underestimated. In fact, it may happen that undifferentiated initially diagnosed connective tissue evolves over time into a form of well-defined connective tissue pathology (differentiated connectivity). This happens because the differentiated connective tissues often present a subtle onset, accompanied by a not well defined symptomatology that does not allow their immediate diagnosis.
In addition to being non-specific, the clinical manifestations of undifferentiated connectivity may vary from one patient to another. However, among the most common symptoms, we remember:
- Temperature;
- Asthenia;
- Arthritis and arthralgia;
- Raynaud's syndrome;
- Pleurisy;
- pericarditis;
- Skin manifestations;
- Xerophthalmia;
- Xerostomia;
- Peripheral neuropathy;
- Positivity to immunological tests for the detection of anti-nucleus antibodies (ANA).
Mixed Connectivity
Mixed connective tissue is a particular type of connective tissue characterized by the appearance of clinical manifestations typical of various rheumatic diseases (such as, for example, symptoms typical of systemic lupus erythematosus, scleroderma, etc.). Furthermore, in the case of mixed connective tissue, this mixed symptomatology is associated with the presence at a blood level of high levels of a particular type of autoantibody: anti-U1-RNP antibodies.
Despite the variegated symptoms with which this form of connective tissue may occur, among the main symptoms that can arise, we recall:
- Temperature;
- Arthritis;
- myositis;
- Raynaud's syndrome;
- Edema of the hands and fingers;
- Thickening of the skin;
- vascular;
- Pleural and pulmonary manifestations;
- Cardiac manifestations;
- Skin manifestations of various types, such as rash, papules, purpura, erythema, etc.
Causes
As mentioned, connectivitis are pathologies of autoimmune origin, that is diseases in which the immune system of the affected individual generates autoantibodies that trigger abnormal immune responses towards the same organism.
Due to this abnormal immune attack, functional, anatomical, district, area, organ and / or tissue alterations are established.
What are the actual causes that underlie this mechanism has not yet been clarified. However, it is believed that the etiology of the connective tissues may be multifactorial and may therefore originate from a set of factors, such as genetic, environmental, endocrine, etc. factors.
Treatment
The treatment of connective tissues may vary depending on the type of rheumatic disease presented by the patient.
The differentiated connective tissues are treated in a specific way according to the type of pathology that has affected the patient.
Mixed connective tissue is usually treated according to the clinical picture that occurs and according to the "predominant" symptomatology that occurs in the individual.
Undifferentiated connective tissue, on the other hand, in the milder forms is mostly treated with anti-inflammatory and analgesic drugs. In more serious forms, on the other hand, it may be necessary to resort to more powerful drugs, capable of interfering with the activity of the immune system.
However, it can be stated that the main medicines used for the treatment of connectivity are:
- NSAIDs (non-steroidal anti-inflammatory drugs), such as acetylsalicylic acid, naproxen and ibuprofen. These active ingredients - in addition to possessing anti-inflammatory activity - possess analgesic and antipyretic activities, so they can also be useful to counter symptoms very common in connective tissue diseases, such as fever and pain.
- Steroid drugs, such as prednisone, betamethasone, methylprednisolone or triamcinolone. Corticosteroids are drugs that can be administered either orally, topically (to treat cutaneous manifestations) or parenterally. They are active ingredients with a strong anti-inflammatory activity, exercised through the reduction of the autoimmune response.
- Immunosuppressants, such as methotrexate, thalidomide, cyclosporine or rituximab. As you can easily guess from their own name, these active ingredients are used in the treatment of connective tissue since they are able to suppress the patient's immune system, thus slowing down the progression of the disease.
Naturally, the choice of the active ingredients to be used in the treatment of the different forms and types of connectivity is solely and exclusively up to the specialist doctor who treats the patient. This doctor will evaluate, case by case, which therapeutic strategy is best suited to each individual.
