Edema of pads of fingers

Systemic scleroderma - part III. Symptoms of systemic scleroderma and prognosis

Symptoms of systemic scleroderma are changes in the skin, joints, gastrointestinal tract, lungs, heart, kidneys. Reynaud's syndrome. Life expectancy of patients with systemic scleroderma

Inclusion in the treatment of scleroderma Extracorporal hemocorrection technologies give a chance:
  • To shortly suppress the activity of autoimmune processes
  • sanitize the foci of chronic infections and thereby interrupt the pathological stimulation of the immune system
  • significantly improve blood flow in the microcirculation system, reduce the manifestation of Raynaud's syndrome and prevent its complications
  • to significantly reduce the severity of clinical manifestations of scleroderma
  • increase sensitivity to traditional medicines
  • reduce doses of immunosuppressive drugs or completely abolish these drugs
This is achieved through the use of:
  • of technologies Cryomodification of autoplasm . allowing to remove from the body mediators of inflammation, circulating immune complexes, autoaggressive antibodies, coarse-dispersed proteins
  • of technologies Extracorporeal Immunocorrection . The ability to suppress the activity of autoimmune processes without reducing the immunological defense potential of the organism as a whole
  • of technologies Extracorporeal Pharmacotherapy . giving the possibility to deliver drugs directly to the focus of the pathological process

Reynaud's syndrome. As a rule, systemic scleroderma begins gradually; Its first symptoms are often Raynaud's syndrome and swelling of the fingers.

Reynaud's syndrome develops in 95% of patients with systemicscleroderma; it is characterized by a transient spasm of small arteries and arterioles of the fingers and toes, sometimes - the tip of the nose and ear lobes. During an attack that can be triggered by cold, vibration, or excitement, the skin first pale, then acquires a cyanotic shade, and when warmed, it turns red. Pimple and blue skin are accompanied by a feeling of cold and numbness of fingers, and redness - with pain and tingling. Since the three-phase change in skin color is not always observed, the most reliable sign of Raynaud's syndrome is blurred fingers.

In the acrosclerotic form of the systemicscleroderma skin manifestations may appear months or even years after the development of Raynaud's syndrome, in a diffuse form - usually within a year. Occasionally, Raynaud's syndrome remains the only manifestation of the disease for two years or more.

Leather. In the initial stages of systemic sclerodermadevelops swelling of the fingers and hands. Sometimes, with systemic scleroderma, it extends to the forearms, face, feet and legs, although the legs usually do not swell so much. The edema stage can last several weeks or months, and sometimes more. Edema can be dense or leave a hole when pressed; sometimes it is accompanied by erythema. Initially, the lesion captures only the distal, and subsequently - and proximal parts of the limbs.

Gradually the skin with systemic scleroderma thickens, thickens and eventually coalesces closely with the underlying tissues (stage of induction).

In the diffuse formsystemic scleroderma the defeat of the skin first captures the limbs and inFor several months or years, it spreads to the face and body. Rapidly progressive (within 2 to 3 years) skin lesion is associated with a high risk of damage to internal organs, especially lungs, heart and kidneys. However, usually skin manifestations peak at 3 to 5 years after the onset of systemic scleroderma, and then gradually decrease.

Acrosclerotic form of systemic scleroderma progresses more slowly, capturing onlydistal parts of the limbs and face or only the fingers. However, even with this form of systemic scleroderma, the severity of skin manifestations may increase with time.

With both forms of systemic scleroderma strongerthe skin of the distal parts of the extremities thickens. Years after the onset of the disease, the condition of the skin can be normalized, although sometimes it becomes atrophic and thinned.

Tightly stretched skin of fingers gradually makes it difficulttheir extension, leading to the development of flexural contractures. Sometimes on the finger pads and over the bony protuberances (on the elbows, ankles, the back surface of the proximal interphalangeal joints) ulcers occur, which can later become infected. Subcutaneous tissue of the finger pads atrophy, and they appear depressed scars. Sometimes resorption of the distal phalanges occurs.

With systemic scleroderma, it is possible hyperpigmentation skin of limbs, face and trunk, even in the absence of insolation. The areas of hyperpigmentation can be located along the surface vessels and tendons. There may be areas hypopigmentation .

Hair follicles, sweat and sebaceous glands at systemic scleroderma atrophy, because of what the skin becomes dry and rough.

Sometimes with systemic scleroderma, dryness of the vagina is noted, which can cause pain during sexual intercourse.

Skin of the face with systemic scleroderma tighttense, because of what it becomes a mask. Oral slit narrows, so patients with systemic scleroderma find it difficult to eat and brush their teeth. Radial wrinkles appear around the mouth, the nose is sharpened and becomes like a beak.

A few years after the onset of the disease, the fingers, face, lips, tongue and mucous cheeks may appear small telangiectasia . Most often they are found in the acrosclerotic form of systemic scleroderma, although in later periods they can also appear in a diffuse form.

To reveal the lesion of the microcirculatory bed in systemic scleroderma, capillaroscopy nail rollers or inspect them usingquality magnifier ophthalmoscope. In the acrosclerotic form of systemic scleroderma, the capillaries of the nail ridges widen, their number is usually normal or slightly reduced. In the diffuse form of the disease, the capillaries are also expanded, but their number is significantly reduced. With only Raynaud's syndrome, the capillaries are not changed.

Sometimes, especially with the acrosclerotic form of systemic scleroderma, calcification of the dermis and subcutaneous tissue. most often - in the area of ​​joints, padsfingers, elbow and prenadikolennikovoy hypodermic bags and on the extensor surface of the forearm. Sometimes the centers of calcification are opened, and a white, friable or liquid mass is released.

Musculoskeletal system. More than half of patients with systemic scleroderma have swelling, tenderness and stiffness of knee joints and fingers.

Sometimes with systemic scleroderma develops symmetrical polyarthritis. resembling rheumatoid arthritis. In the late stages of the disease when moving in the joints (especially the knee), there is a crepitation, and a squeak can be heard over the tendons.

Severe fibrosis and thickening of the fascia Forearm leads to carpal tunnel syndrome.

Muscle weakness. which occurs with systemic scleroderma, usually with severe skin damage, in most cases due to atrophy from inactivity.

Myopathy with systemic scleroderma has characteristic histological signs and is not accompanied by an increase in the activity of muscle enzymes in the serum.

In some patients, systemic scleroderma develops myositis. which like polymyositis manifests weakness of proximal muscles and increased activity of muscle enzymes (cross syndrome).

Resorption can be not only distal phalanges of the fingers, but also ribs, clavicle and angle of the lower jaw.

GIT. Both forms of systemic scleroderma usually occurwith a lesion of the digestive tract. More than half of the patients have symptoms of esophageal damage, such as a feeling of bursting or burning in the epigastrium and behind the breastbone and belching. These symptoms increase in the prone position and when tilted forward. Their causes are a decrease in the tone of the lower esophageal sphincter and an expansion of the middle and lower thirds of the esophagus.

In many patients with systemic scleroderma develops reflux esophagitis. which often leads to stricture of the lower thirdesophagus and occasionally to bleeding from the esophagus. In addition, reflux esophagitis may cause a cell-cell metaplasia of the esophageal epithelium, but adenocarcinoma develops rarely.

Dysphagia (especially when swallowing solid foods) canoccur in the absence of other symptoms of esophageal injury. Its cause is a decrease in esophageal motility, caused by neuromuscular disorders. Esophageal manometry and roentgenography can detect a decrease in the amplitude or complete absence of peristalsis in the middle and lower thirds of the esophagus. Disturbance of esophageal motility is observed even in patients suffering only from Raynaud's syndrome.

Extension and atony of the lower parts of the esophagus and reflux esophagitis usually occur in advanced stages of systemic scleroderma.

Stomach lesion with systemic scleroderma, radiologically manifested by its expansion, atony and delayed emptying.

Decreased motility of the small intestine is manifested by flatulence and abdominal pain, sometimes so severe that a patient with systemic scleroderma is diagnosed with mechanical or paralytic intestinal obstruction.

With systemic scleroderma it is possible development of the syndrome of impaired absorption. which is manifested by weight loss, diarrhea and anemia. The reasons for it may be excessive growth of bacteria in the atopic intestine or fibrosis and obstruction of the lymphatic vessels.

In radiopaque studies,expansion of the descending and horizontal parts of the duodenum and jejunum, smoothing of the circular folds and slowing down of the passage of the barium suspension. In some patients, systemic scleroderma develops cystic pneumatosis of the intestine; while on the radiographs in the wall of the small intestine round or linear enlightenments are visible. With the rupture of the cysts, pneumoperitoneum can develop without signs of peritonitis.

Dislocation of the large intestine with systemic scleroderma can manifest itselfchronic constipation, a fatal blockage and even an intestinal obstruction. The atony of any part of the intestine can lead to intussusception. With radiopaque examination of the large intestine, sometimes its expansion and atony are revealed, as well as diverticula with a wide mouth.

Decreased tone of the internal sphincter anus can lead to incontinence of the stool and occasionally to the prolapse of the rectum.

Teleangiectasia of the stomach or intestinal mucosa can become a source of gastrointestinal bleeding.

In the visceral form of systemic scleroderma, the lesion of the skin and other organs is insignificant or absent.

Lungs. Currently, lung damage servesthe main cause of death in systemic scleroderma. It is detected in at least two-thirds of patients; in most cases, it manifests itself as dyspnea during exercise, often in combination with a dry cough. Sometimes these symptoms are observed in the absence of pneumosclerosis, and pneumosclerosis, in contrast, can occur almost asymptomatically.

In some patients with systemic scleroderma, bilateral rales in the lower parts of the lungs are heard. Occasionally there is a restriction of respiratory movements due to severe damage to the skin of the trunk.

Gastroesophageal reflux arising from atony of the lower esophagus can cause aspiration pneumonia.

One of the serious complications of pneumosclerosis in systemic scleroderma - viral or bacterial pneumonia. In addition, pneumosclerosis increases the risk of bronchioloalveolar, squamous and oat cell lung cancer. When examining the function of external respiration, Reduction of the Ligament and Reduction of Flexibility of the Lung. Reduction of the diffusivity of the lungs and low PaO2 at physical exertion testify to the violation of gas exchange. At the same time, there can be no x-ray signs of pulmonary involvement.

On chest radiographs (especially inmiddle and lower parts of pulmonary fields), with systemic scleroderma, linear or multiple shallow-shadow shadows and a picture of the cellular lung can be detected. To detect early changes in the lungs, high-resolution CT and bronchoalveolar lavage are used. In the alveolitis in the fluid obtained from bronchoalveolar lavage, a large number of alveolar macrophages, neutrophils and eosinophils are detected.

Less than 10% of patients with acrosclerotic form of systemic scleroderma after years after the onset of the disease develops pulmonary hypertension in the absence of severe pneumosclerosis. Its cause is obliteration of the pulmonary arteries and arterioles, caused by intimal fibrosis and hypertrophy of the media. The development of pulmonary hypertension in systemic scleroderma is usually accompanied by changes in the ECG. Clinically, it manifests itself with increasing dyspnea and subsequent development of right ventricular failure. The prognosis for pulmonary hypertension is extremely unfavorable, with an average life expectancy of about 2 years.

A heart. Systemic scleroderma can lead to the development of pericarditis (sometimes with pericardial effusion), heart failure, arrhythmias and AV blockade of varying degrees.

The defeat of the heart is observed in the majority of patients with a diffuse form of systemic scleroderma.

Less than 10% of patients (mainly with a diffuse form of systemic scleroderma) cardiosclerosis leads to restrictive cardiomyopathy. In patients with cardiosclerosis in isotopicVentriculography reveals a violation of left ventricular function. In patients with Raynaud's syndrome, scintigraphy with 201 TI, performed during seizures, reveals a violation of myocardial perfusion.

Spasm of the coronary vessels can lead todamage to the myocardium, histologically manifested by contracture damage to myofibrils. In some patients with systemic scleroderma, despite the absence of changes in coronary angiograms, angina pectoris .

Arterial hypertension can lead to left ventricular failure, and pulmonary hypertension - to the development of pulmonary heart.

The kidneys. Before the advent of modern methods of treating renal failure, it was the main cause of death in systemic scleroderma.

Severe renal disease is observed mainly in the diffuse formsystemic scleroderma. Risk renal scleroderma crisis above all with a vast rapidly progressingdefeat of the skin. Renal scleroderma crisis is almost always accompanied by malignant arterial hypertension, which can quickly lead to kidney failure. Such patients have hypertensive encephalopathy with severe headache and epileptic seizures, retinopathy and left ventricular failure. The beginning of the crisis is marked by hematuria and proteinuria, and subsequently develops oliguria and renal insufficiency.

The pathogenesis of this complication is systemicscleroderma is the activation of the renin-angiotensin system. Before the introduction of new antihypertensive drugs, most patients who developed a renal scleroderma crisis died within 6 months. In patients with mild or moderate arterial hypertension and insignificant proteinuria, renal dysfunction progresses less rapidly, and renal insufficiency develops only in the late stages of systemic scleroderma. However, heart failure or hypovolemia with an overdose of diuretics can lead to a decrease in renal blood flow and, as a consequence, to the development of a renal scleroderma crisis, even if there were no signs of kidney damage before.

Harbinger renal failure - microangiopathic hemolytic anemia, which can occur in the absence of arterial hypertension, and a long-lasting pericardial effusion.

Other organs with systemic scleroderma. Frequently marked xerostomia and xerophthalmia. At a biopsy of a lip reveal a lymphocyticinfiltration of small salivary glands, characteristic of Sjogren's syndrome, or fibrosis of salivary glands and surrounding tissues. Antibodies to Ro / SS-A or La / SS-B are found only in patients with lymphocytic infiltration of the salivary glands.

With systemic scleroderma often develops hypothyroidism. which can be accompanied by a sharp increase in the level of antithyroid antibodies in the serum. The cause of hypothyroidism can be not only chronic lymphocytic thyroiditis, but also thyroid fibrosis.

Sometimes, with systemic scleroderma, trigeminal neuralgia. Some patients develop impotence. The level of testosterone and gonadotropic hormones in them is normal; probably, the cause of impotence is vascular and autonomic disorders.

In the acrosclerotic form of systemic scleroderma, the primary biliary cirrhosis of the liver.

Table 1. Symptoms of systemic scleroderma

and Less than 2% of cases the disease occurs without severe skin damage (visceral form of systemic scleroderma).

The course and prognosis of systemic scleroderma

The course of systemic scleroderma is very diverse. Estimate the prognosis in the early stages of the disease, when the differences between its two forms are not yet expressed, is difficult.

With an acrosclerotic formsystemic scleroderma. especially when detecting antibodies to centromeres, the forecast is generally favorable. but less than 10% of patients 10 to 20 years after the onset of systemic scleroderma (and sometimes later) develops pulmonary hypertension. Sometimes there is also a syndrome of impaired absorption and primary biliary cirrhosis of the liver.

In the diffuse formsystemic sclerodermathe forecast is much worse. especially in men and people who became ill in the elderlyage. In patients with rapidly progressive extensive skin lesions, early kidneys and other internal organs may be affected early in the course of the disease.

The main causes of death with systemic scleroderma - defeat of the lungs, kidneys and heart.

After the introduction of new methods of treatment of renal scleroderma crises, as well as hemodialysis in renal failure Ten-year survival rate with systemic scleroderma increased to 65%. In general Ten-year survival rate in the diffuse form of systemic scleroderma is 55%. a with acrosclerotic form of systemic scleroderma - 75% .

Years after the onset of systemic sclerodermathe skin tightening can decrease, and if the skin lesion first captures the distal, then the proximal parts of the limbs and subsequently spreads to the trunk and other parts of the body, then it disappears in the reverse order. Sclerodactyly and flexural contractures can still be preserved. The thickness of the skin with time can also be normalized, although sometimes the skin atrophies.