Systemic scleroderma: diagnosis, treatment, classification, prognosis

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Systemic scleroderma SSD is a serious autoimmune disease related to connective tissue diseases with manifestations of densification and hardening, damage to blood vessels, tissue, alveoli of the lungs, myocardium, kidneys, digestive system organs in the form of fibrosis( connective tissue proliferation with scar deformities of internal organs due to reasoninflammation).

Also manifested as symptoms of peripheral vascular ischemia in the form of Raynaud's syndrome. Therefore, today we will review the history of the disease systemic scleroderma, its therapy, pathogenesis, causes and clinical recommendations.

Features of the disease in adults and children

Systemic scleroderma, which began in children at a young age, has certain differences, expressed in the predominance of articular and muscular dystrophies, low mobility, underdevelopment of hands and feet, contractures( restriction of joint mobility due to pathological changes in soft tissues).But internal injuries( visceral) are less common.

The onset of the disease is most often imperceptible and gradual, acute cases are rare. As the disease progresses and affects the pathological process of new organs, the disease takes over the entire body and manifests itself in multiple syndromes.

Let's talk about the classification that systemic scleroderma has.

About the features of systemic scleroderma the specialist in the video below tells the following:

Classification of

Forms of

There are two basic forms of the disease:

1. Diffuse form of systemic scleroderma with generalized aggressive and rapid skin lesions, development of thickening on limbs, face, body, gradual spread of pathological process to all organs.
2. Acrosclerotic( limited) - with separate areas of damage to the skin, thickening on the face, hands, feet, ankles and wrists. Syndrome CREST - subform of acrosclerotic scleroderma manifests itself:
   • calcinosis( deposition of a large number of calcium salts in the form of nodes under the skin, in the elbow, hip joints, fingers, along the tendons and fasciae);
   • Reynaud syndrome( spasm of arterioles and finger capillaries with circulatory disorders, pain, cold and blue skin);
   • a violation of the function of the esophagus;
   • sclerodactylia( firming of the skin on the fingers with atrophy of the subcutaneous tissues);
   • by telangiectasia( persistent dilatation of small vessels in the form of asterisks and meshes on the skin).

It is also important to know:

• The combination of signs of SSD and other pathologies of connective tissue( arthritis, lupus erythematosus, dermatomyositis) is called a cross( CROSS) syndrome.
• The visceral form of the SSD is expressed mainly by the manifestations of the internal organs, and the pathologies of the skin are practically not observed.
• Isolate juvenile scleroderma, which is characterized by separate foci of damaged skin, mild Raynaud's syndrome, but with inflammation, pain, joint movement( with limb deformities).Tissue lesions of internal organs are absent, functional disturbances prevail.

The course of the disease

The course of the disease is expressed in three main variants, which differ in the primary manifestations, symptoms, severity and severity, dynamics and prognosis:

• acute( actively developing);
• subacute form( progressive to moderate extent);
• chronic form( sluggish).

Now in more detail:

• With acute course throughout the year, progressive fibrosis of tissues and organs develops, active and pronounced manifestation of symptoms, development of the syndrome of "scleroderma kidney".
• Subacute is manifested by damage to the tissue of the joints, muscles, skin and organs, signs of immune inflammation( according to the analysis), weak vascular disorders;
• The chronic form develops slowly over a period of 5 to 10 years, gradually manifesting itself in trophic disorders, vascular damage( in the form of Raynaud's symptom, dense skin edema, scar formation, contracture, osteolysis( bone resorption), ulceration and narrowing of the esophagus, myocardial damage,lung tissue Skin deformities are often small, limited and for a long time are the only symptom of systemic scleroderma

Stages of

The disease occurs in 3 stages:

1 - primarysigns( mainly, joint and vascular( cold spasm like Reynaud, cyanosis in chronic), most favorable for recovery

2 - generalization or manifestations of a multiplicity of symptoms and the seizure of all systems and organs by the disease. The results are less noticeable.- organic changes( the final stage with severe vascular, dystrophic, sclerotic lesions and development of necrosis of individual parts of the body or inadequate organ function).The most unfavorable prognosis for recovery and quality of life.

Causes of

The cause of the disease, as well as of other connective tissue diseases, is unknown to medicine. It is assumed that the main role in the development of systemic scleroderma has an inherited defect of the immune system, leading to autoimmune disorders. This is manifested in the excess production of antibodies to the cells of the native organism, which the upset immune system takes for the alien, destroying them.

In the case of SSD, "strangers" are cells of connective tissue. In the blood plasma for a long time, special immune blocks move, which are introduced into the vessels, tissues, organs, gradually destroying them. A variant of the viral and genetic origin of the disease is also considered.

To the following risk factors:

• supercooling;
• vibration and shaking during operation;
• trauma( often - trauma to the skull);
• infectious diseases;
• contact with chemical, pharmaceutical substances;
• endocrine alteration of the body;mental trauma and nervous overload;
• allergic factor.

About the symptoms of systemic scleroderma we'll talk further.

Symptoms of

The main clinical signs of systemic scleroderma:

• cold sensitivity or Raynaud syndrome is a frequent primary symptom. It manifests itself in a sudden numbness of 2 to 4 fingers, feelings of cold, sharp blanching and blue fingertips. After the end of the attack - pain, burning, a feeling of heat and redness of the fingers. Later, as the disease develops, it seizes the feet, lips, face, and tongue.
• body temperature fluctuations;
• significant weight loss before kahesia( up to 10 kg or more);
• abnormal tissue nutrition;
• kidney damage in the form of acute nephropathy - renal dysfunction( the so-called scleroderma kidney) and in the form of chronic - with damage to glomeruli and tubules;
• cardiosclerosis, pericarditis, arrhythmias, rhythm disorders of myocardial contractions and conduction, circulatory failure in the coronary vessels, functional insufficiency of the mitral valve;
• skin lesion is a basic symptom( dense painful edema, induration - hardening, immobility of the face, manifestation of spotting due to uneven pigmentation, sharp vascular pattern, widening of the vessels on the face, atrophy);
• lung injury( emphysema, bronchiectasis, fibrosing alveolitis, pneumonia, pneumofibrosis);
• persistent polyneuropathy( tenderness, impaired sensitivity in the limbs, decreased tendon reflexes);
• hypothyroidism, autoimmune thyroiditis, sometimes - hyperthyroidism, sexual dysfunction;
• trigeminitis( inflammatory process in the trigeminal nerve);
• increased ESR;
• increase in protein in the blood( more than 84 g / l);
• high content of immunoglobulins in the blood;
• articular factor( polyartralgia, polyarthritis, periarthritis with motor impairment and fibrotic pathologies, osteolysis( bone resorption) finger phalanges, deformation of fingers;
• calcification of internal tissues, fingers, elbows in the form of white convex edema showing through the skin;
• esophagitis- inflammation of the esophagus with ulceration, difficulty in swallowing, pain and spasms, narrowing of the esophagus lumen;
• muscle damage in the form of myositis( pain, weakness, stiffness).

Diagnosis of systscleroderma

Baseline signs

1. Scleroderma skin lesions with uneven pigmentation, beginning with dense edema, hardening and atrophy of subcutaneous tissue on the hands, face assuming the form of a mask
2. Reynaud Syndrome
3. Joint and muscular lesions with development of soreness, swelling, persistent movement disordersin the joints that combine the signs of rheumatoid-like arthritis, periarticular deformities( atrophy and inflammation of the soft tissues surrounding the skeletal bones, fibrous myositis( inflammation of the musclests with scar formation)
4. Osteolysis of the fingers, their shortening and deformation.

About the diagnostic methods and treatment of systemic scleroderma will tell this video:

Laboratory data

• In the blood there is: sometimes - signs of anemia, a decrease or, conversely, an increase in the number of leukocytes, an ESR above 20 mm / h, an increased protein - more than 85 g /a multitude( more than 22%) of immunoglobulins, different antibodies to native DNA.
• In urine - increased excretion of hydroxyproline( a sign of destruction of bone tissue), a lot of protein.
• In immunological studies of blood it is found out: in half of patients - the presence of RF, 30 - 90% - antinuclear antibodies, in 3-7% - lupus cells.
• At X-ray examination,
  • is found to accumulate salts under the skin, mainly on the fingers, feet, elbows, knees;
  • bone resorption in the phalanges of the fingers, elbows, posterior ribs;
  • osteoporosis around joints( resorption of bone tissue), individual erosion of articular cartilage;
  • esophageal expansion or narrowing of the esophagus;
  • diffuse pneumosclerosis and increased heart volume.
• The electrocardiogram shows changes in myocardial tissue, atrioventricular blockade.

About the methods of treatment in the clinic and folk remedies for systemic scleroderma we will describe below.

Treatment of

Systemic scleroderma is an incurable disease, but the treatment of individual traits improves the condition and functioning of the organs, prolonging the patient's life and improving its quality.

Therapeutic and medicamentous

Therapeutic treatment of patients with diabetes is always complex, aimed at eliminating or alleviating the symptoms of the disease. Medicines for treatment are divided into three basic groups:

1. Antifibrotic: D-penicillamine, lidase, colchicine, diucifon.
2. Vascular agents: vasodilators, medications that prevent platelets from clumping( aggregating) and forming blood clots, overlapping vessels, angioprotectors for normalization of vascular function and condition( Nifedipine, Curantil, Trental, Reopoliglyukin.)
3. Means that remove inflammatory processes and inhibit certain immune responses(hormones, antitumor drugs - cytostatic)

In addition, they use:

• Hingamin, Delagil, Plakvenil - aminoquinoline preparations,
• agents, Reducing blood pressure( Normatens, Captopril, Kapoten)

Features of the use of

D-penicillamine is the main drug for scleroderma therapy that can stop the acute development of the disease in its aggressive course. D-penicillamine significantly reduces skin thickening, Raynaud's symptoms, prevents severepathology of internal organs, helps to soften the manifestations of arthralgia, myalgia, relieves puffiness, improves trophism, movement in the joints. Reduces palpitations, shortness of breath, symptoms of cardialgia, improves the function of swallowing. Under the influence of D-penicillamine there is a slowdown in the development of lung diseases, a marked increase in the life expectancy of patients.

Adverse adverse effects less if treatment begins with a low dose of 250 mg per day, with an increase in 2 - 3 months to 450 - 900 mg. The course lasts several months until a positive therapeutic result is observed. The medicine is drunk an hour before eating. High doses lead to an increase in adverse events: glomerulonephritis, myasthenia gravis, anemia, a decrease in leukocytes and platelets, fever, rashes on the skin, bleeding, convulsive attacks, hair loss. Contraindications: an initial renal failure, a malfunction of the liver, an immunity to the active substance.

Immunosuppressants are used only in the aggressive course of systemic scleroderma and the threat of life-threatening complications. Assign Azathioprine( imarant), Cyclophosphamide in an amount up to 200 mg per day, Chlorambucil in a daily dosage of 8-15 mg.

Glucocorticosteroids( mainly Prednisolone) are prescribed for prolonged use in acute and subacute form, or for 1 to 2 months with exacerbation of chronic scleroderma and marked inflammatory changes. Myositis, pericarditis require the use of an initial daily dose of 30 to 50 mg( 3 to 4 doses) with a gradual decrease. In acute course with severe fibrosis of organs and tissues, hormonal preparations give effect only when combined with D-penicillamine. Long-term use of glucocorticosteroids in systemic scleroderma is not recommended.

Aminoquinoline agents help with long-term use, as estimated by many patients. Delagil in a daily dosage of 0.25 g, Plakvenil - up to 0.4 g is prescribed in the complex treatment of SSD, often if the patient does not tolerate D-penicillamine, with significant changes in the joints.

• For the improvement of cutaneous and joint lesions, Lidase 64 -122 ED( courses of 12-14 injections) or lidase using electrophoresis is injected subcutaneously. Intervals up to 3 months. Applied, in general, with chronic, focal scleroderma. Contraindications - acute process severity, significant permeability of blood vessels.
• Since the onset of the disease, it is necessary to improve microcirculation in the vessels, preventing and eliminating their spasm during the Reino phenomenon. Apply Prazosin, Reserpine, Methyldofu, Nifedipine, Diltiazem. All these medicines have serious undesirable effects, so the course of treatment is short-lived. Patients well tolerate drugs with Nifedipine prolonged action in daily dosages of 30-60 mg( up to 90 mg).Very often this drug shows excellent results of treatment.
• Ketanserin - a blocker of serotonin receptors - has proved itself well in the SSD.Iloprost reduces the severity of attacks, promotes healing of ulcers.
• Pentoxifylline is used to activate blood circulation in capillaries.

Other

• methods Physiotherapy is prescribed necessarily to improve the mobility of the joints.
• In order to slow the damage of the joints, relieve pain, inflammation, swelling - use either non-hormonal anti-inflammatory drugs or prescribe prednisolone( not more than 10 mg per day).
• Reflex esophagitis, which accompanies systemic scleroderma, requires special attention. Use drugs that reduce the acidity of gastric juice( cimetidine, Ranisan, ranitidine if necessary, omeprazole - on the capsule before bedtime).It is recommended to take food often, but in small portions, raise the head of the bed, give up coffee, chocolate, strong tea, products that are relaxing on the esophageal sphincter.
• Daily multi-massage with oils helps to maintain the mobility of the fingers, wrists, feet, promotes the elasticity of the skin. To accelerate the healing of ulcers( not infected) on the fingers, occlusive dressings are used, ulcers are washed, ulcerated tissues are removed, enzymes are applied, the skin around the nitroglycerin ointment is lubricated, vasodilating agents - Reserpine, Octadin are used. If ulcers are infected - antibacterial topical treatment is performed( Oflomelid, Stellanin)
• To reduce drying of the skin, it is necessary to use soft non-alkaline agents with additives of oils, creams, ointments on a hydrophilic basis when washing.
• Such drugs as penicillamine, alkaloid colchicine, vitamins B10 and E improve the skin condition.
• Sympathectomy, used in SDS, gives an unstable improvement, without preventing the development of vascular lesions. Until now, the ways of treating the phenomenon of Raynaud do not give a full recovery. With the development of gangrenous lesions of fingers with complete vascular ischemia, amputation of fingertips is often resorted to.
• Patients STD recommended - wear warm clothes, in the cold use warmed gloves and socks, give up smoking habits, avoid, if possible, psychoemotional stress, use Ergotamine, amphetamines and β-blockers.
• For pulmonary infections, antimicrobial treatment is provided. To eliminate hypoxia in the presence of oxygen deficiency, oxygen therapy is performed. All patients with SSD and lung lesions are given pneumococcal vaccine.
• If severe pathologies of internal organs are absent, do lung transplantation, block "heart-lungs".Early detection of renal impairment in the SDS makes it possible to preserve the kidney function, to prevent hypertensive encephalopathy. The use of antihypertensive drugs - Captopril, Clonidine, Enalapril, Propranolol, Minoxidil, Lizinopril - stabilizes blood pressure and restores kidney function.
• Active development of renal failure requires immediate hemodialysis procedure. The kidney function usually improves after 4 to 6 months, and hemodialysis is canceled. Conducting kidney transplantation is inappropriate, since in diffuse scleroderma, the pathological process affects all organs.

Prevention of

Suspicion of the SSD requires an immediate detailed examination of qualified narrow specialists. Primary prevention of persons at risk includes:

1. Prophylactic examinations once a year or half a year, dispensary observation( special attention to children of puberty age with risk factors)
2. Conducting necessary blood tests, urine
3. Observation with a cardiologist with the performance and analysis of an electrocardiogram, neurologist, psychotherapist
4. Radiographic and ultrasound examination of internal organs
5. Safe employment of SSD( work without the risk of cooling, injuries, overstrain and vaccinations), early career guidance of adolescents withthreat of development of the SSA.
6. Rational nutrition, increasing the body's natural resistance, eliminating smoking as a negative factor, destroying blood vessels.

All specialist doctors, when examining and analyzing the patient's condition, are aimed at detecting primary, often mild, manifestations of the disease. Secondary prevention - prevention of exacerbations and spreading of the process - includes early diagnosis and detection of STI, active treatment in the hospital, later - outpatient, following the rules:

• immediate medical attention if the general condition changes or if there are any strange symptoms;
• strict adherence to diet in scleroderma systemic and prescribed treatment;
• prevention of dose adjustment of medicinal products without doctor's permission;
• elimination of hypothermia, overwork, stress loads and provoking factors;
• in the event of an upcoming surgery or development of infection, supplement therapy with antibacterial agents while maintaining a dosage of corticosteroids.

Complications of

Systemic scleroderma causes inflammation of small vessels, proliferation of fibrous tissue around them, thickening and hardening of the walls. A damage to blood vessels leads to direct disturbances in the functions of internal organs. The main threat lies in severe blood flow disorders that feed cells of tissues and organs with oxygen, leading to their pathological destruction.

In most cases, without early diagnosis and subsequent active treatment, scleroderma leads to serious consequences. Among the complications:

1. Necrosis( necrosis of cells) of tissues.
2. Pulmonary fibrosis( lesion of the alveoli with proliferation around the fibrous tissue, leading to destruction of the lungs).
3. Pulmonary hypertension( closure of the lumen of the lung vessels, causing abnormal pressure with impaired myocardial function).
4. Proteinuria( active excretion of large amounts of protein in the urine, leading to serious illnesses and kidney dysfunction).
5. Arrhythmias and heart failure.
6. Gangrene of the extremities.

In the absence of intensive treatment, the processes of destruction of blood vessels, tissues, joints, and organs are dangerously irreversible. About the life expectancy of patients who have systemic sclerosis, let's talk below.

Forecast

• The prognosis of the juvenile form of systemic scleroderma is often favorable with the gradual fading of many signs of scleroderma.
• Chronic form of SDS when it starts at a young age, often occurs in good quality and with a rather favorable prognosis. Survival of patients with a chronic form for more than 10 years - more than 84%, with subacute - about 62%, with an acute course - by the end of the decade, virtually no one remains. This clearly points to the fact that the nature of the course of scleroderma is a very serious indicator for further prognosis.
• In patients with systemic scleroderma having a high frequency of abnormalities in chromosomes, the prognosis is more negative.

D-penicillamine - today is the only means of active treatment in the acute form of the disease, which can slow its development and significantly improve the prognosis. For 6 years, 9 out of 10 patients survived from penicillamine-treated patients, and only 5 out of 10 survived without therapy. The studies found the effectiveness of prolonged therapy in high doses( daily amount 1.5 g) in an aggressive course of the disease with a significant reduction in fatalities.

An important factor for the survival and recovery of STD patients is the rational employment of patients who

• for severe, acute systemic scleroderma transfer to disability;
• and in chronic form - completely free from heavy physical labor, excluding also the possibility of supercooling and contact with chemicals.

The competent scheme of therapy and adequate work positively influence the prediction of the SSS and enable to maintain the normal working capacity and the rather active standard of living of patients with STI.

Even more useful information about therapy and prognosis for systemic scleroderma is contained in this video: