Diagnosis of systemic scleroderma

Diagnosis of systemic scleroderma, which is based on data from instrumental and laboratory studies, allows assessing the degree of involvement of internal organs and the severity of pulmonary hypertension.

To this end, chest radiography, electro- and phonocardiography, echocardiography (EchoCG), a 6-minute walk test to determine the FC of circulatory insufficiency and pulmonary hypertension, an evaluation of the function of external respiration, ventilation-perfusion scintigraphy) of lungs, angiopulmonography, catheterization of the right of the heart, multispiral computed tomography of the chest, as well as blood tests (clinical, biochemical, immunological, analysis for hemostasis and rheological evaluation of blood).

ECG examination in systemic scleroderma most often reveals a decrease in voltage, heart rhythm disturbance (67%) - supraventricular and ventricular tachyarrhythmias, extrasstoles, intra-atrial (42%) and intraventricular (32%) conduction up to a complete blockade requiring pacemaker implantation. There are described "infarct-like" ECG changes in SDS.

Echocardiography is one of the most informative non-invasive methods for assessing pulmonary artery pressure, in addition, the study allows estimating chamber sizes and wall thickness, contractile and pumping function of the myocardium, dynamics and shape of intracardiac flows. The dilatation of the right ventricle is best judged by an increase in the ratio of the area of the right ventricle to the area of the left ventricle (preferably from the apical 4-chamber position), with a value of this ratio of 0.6-1.0 suggest a slight dilatation of the right ventricle, with a value of more than 1, 0 - about pronounced dilatation. Two-dimensional echocardiography allows us to evaluate the kinetics of the interventricular septum-a paradoxical systolic movement with pronounced pulmonary hypertension, which, along with a decrease in the pulmonary venous influx, leads to a breakdown in the isometric relaxation of the left ventricle. In most patients with systemic scleroderma, even with minor signs of myocardial damage in echocardiography, diastolic dysfunction of the left ventricle (50-80%) is detected. When symptoms of systolic dysfunction (reduction of the left ventricular ejection fraction less than 55%) occur, the risk of a lethal outcome with systemic scleroderma increases manyfold.

With the help of pulse-wave Doppler, it is possible to measure the pressure in the pulmonary artery. Systolic pressure in the pulmonary artery is equivalent to systolic pressure in the right ventricle in the absence of an obstruction to the flow of blood from the ventricle. The systolic pressure in the right ventricle is assessed by measuring the flow rate of systolic regurgitation on the tricuspid valve (V) and the right atrial pressure estimation (BPD) used in the formula:

Systolic pressure in the right ventricle = 4v2 + DPP.

DFT is either a standard value, or it is measured by the characteristics of the inferior vena cava or the expansion of the jugular veins. The tricuspid regurgitation can be evaluated in the majority (74%) of patients with pulmonary hypertension,

Based on the values of systolic pressure in the pulmonary artery, the following degrees of pulmonary hypertension are distinguished: •

• light - from 30 to 50 mm Hg;
• average - from 51 to 80 mm Hg;
• Heavy - from 81 mm Hg. And higher.

Despite all the unconditional advantages of EchoCG, there are limitations of the method in terms of diagnosis of right ventricular dysfunction, taking into account the difficulty of visualization and features of the anatomical structure of the right ventricle (the presence of trabeculae and moderator strand). The study of the parameters of its functional activity using standard EchoCG techniques is not entirely correct. Thus, the problem of non-invasive assessment of the functional capabilities of the right heart is evident. Now in the literature there are data on the possibility of using tissue Doppler echocardiography (DEHOKG), the technique of which is to determine the speed of movement of tissue structures and is intended for in-depth study of myocardial function. This method will provide objective information about the state of global and segmental longitudinal function of the myocardium. The peculiarity of the technique consists in the possibility of this use for determining the systolic and diastolic function of the right myocardium of the heart.

The catheterization of the right heart and pulmonary arteries is the "gold standard" method for the diagnosis of pulmonary hypertension. The "direct" method allows you to measure with the greatest accuracy the pressure in the right atrium and right ventricle, pulmonary artery, pulmonary artery wedge pressure (PZL), calculate cardiac output (more often apply the method of thermodilution, less often - Fick's method), determine the level of oxygenation of mixed venous blood (PvG, and SvC)). This method helps to assess the severity of pulmonary hypertension and right ventricular dysfunction, it is also used to assess the effectiveness of vasodilators (usually acute samples),

Magnetic resonance imaging (MRI) is a relatively new method for diagnosing pulmonary hypertension. MRI will provide an opportunity to accurately estimate the wall thickness and volume of the right ventricular cavity, as well as the right ventricular ejection fraction.

[1], [2], [3]

Diagnostic criteria for systemic scleroderma

The American Association of Rheumatologists proposed the following diagnostic criteria for STDs.

A large criterion is proximal scleroderma: a symmetrical thickening and induration of the skin of the fingers, extending proximally from the metacarpophalangeal and metatarsophalangeal joints. Changes can affect the face, neck, chest and stomach.

Small criteria.

• Sclerodactyly; the above skin changes are limited to the fingers.
• Digital scars: areas of skin twisting at the fingertips or loss of the substance of the pads of the fingers.
• Two-sided basal pneumofibrosis: mesh or linear-nodal shadows, most expressed in the basal regions of the lung with a standard X-ray examination, may be manifestations of the type of the cellular lung.

In order to establish a diagnosis of DSS, one or two small criteria must be present. Using these criteria to recognize the early stages of the disease is impossible.

To assess the activity of the SSA, indices developed by the European troupe for the study of systemic scleroderma are currently used. Points sum up. The maximum possible score is 10, with an activity of 3 points or higher, the disease is considered active, less than 3 - inactive.

Assessment of the activity of systemic scleroderma

Parameter	Score	Characteristic
Skin Score> 14	1	Use a modified skin count, estimated in scores from 0 to 3 in 17 areas of the body
Scleremedia	0.5	Thickening of soft tissues, mainly on the fingers due to induration / skin
Leather	2	Deterioration of cutaneous manifestations in the last month, according to the patient
Digital necrosis	0 5	Active digigal ulcers or necrosis
Vessels	0.5	Deterioration of vascular manifestations in the last month, according to the patient
Arthritis	0 5	Symmetrical swelling of the joints
Heart / lungs	2	Deterioration of cardiopulmonary manifestations in the last month, according to the patient
ESR> 30 mm / h	1.5	Determined by the method of Westergren
Hypocomplementation	1	Reduction of NW- or C4-com-
Reduction of RLCO *	0.5	RLCO <80% of the normal level

[4], [5], [6], [7]

Examples of the formulation of the diagnosis

Systemic scleroderma, limited form, chronic course, active. Reynaud's syndrome, esophagitis, sclerodactyly, pulmonary arterial hypertension of the II degree, II FC.

Systemic scleroderma, diffuse form, rapidly progressive course, active, polyarthritis, functional class (FC) II, interstitial myositis, glomerulonephritis, chronic renal failure I, recurrent pneumonia, basal pneumosclerosis, respiratory failure I, myocarditis, frequent ventricular extrasystole, circulatory insufficiency ) II A, III FC.

[8], [9], [10], [11], [12],