JUVENILE DERMATOMYOSITIS

Yuvenilngy dermatomyositis - a disease of the groups of diffuse diseases of connective tissue with a primary lesion
of the proximal skeletal muscles, the development of muscle weakness, as well as purple erythema on the
skin. Because the etiology of the disease is unclear, juvenile dermatomyositis include the composition of a
heterogeneous group of idiopathic inflammatory myopathy with leading clinical manifestation - the defeat of the
skeletal muscle of inflammatory genesis. In accordance with the classifications RL Woltman (1994), except for
juvenile dermatomyositis in this group also includes other myopathies (Table 13-10).

Table 13.10. Classification of inflammatory myopathy

I. Idiopathic inflammatory myopathies

Primary polymyositis;

'Primary dermatomyositis;

juvenile dermatomyositis;

myositis, associated with other diffuse diseases of connective tissue;

myositis, associated with tumors;

myositis "inclusions";

myositis, associated with eosinophilia;

cavalry;

Giant cell myositis;

localized, or focal, myositis

P. Mionatii caused by infection

III. Myopathy, caused by drugs and toxins

In children, other types of myopathy than juvenile dermatomyositis observed very rarely.

As the incidence of juvenile dermatomyositis is ranked third among the diffuse diseases of connective tissue, it is
1,9:1 OOO OOO children under the age of 16 years.Incidence of children an average of 1/5-1/8 of the number of
affected adults. Juvenile dermatomyositis are more common in girls than in boys (ratio 2:1). Notes two peak
incidence - in the 3-5 and 7-9 years.

Etiology and pathogenesis

Among the possible causes of dermatomyositis discuss the role of viral infection (Coxsackie virus A and B,
Picornavirus) to cause disease in genetically predisposed people. Discuss three possible sources of exposure to the
virus in the body.

Direct damage of muscle tissue.

 Synthesis of AT to viral Ag, located on the surface of muscle fibers.

Synthesis of antibodies to viral Ag, cross-reactive autoantigen-us (antigenic mimicry).

On the role of genetic factors indicate the presence of family cases of dermatomyositis, the development of disease
in twins, the association of dermatomyositis with HLA-B8,-B14 and-DR3. As with other diffuse connective tissue
disease, juvenile dermatomyositis developed with the participation of Environment and endocrine factors.

Immunological abnormalities in juvenile dermatomyositis identify both the cellular and humoral level in. Affected
muscles are infiltrated with activated T-lymphocytes and B-lymphocytes and macrophages, and, as studies have
shown that T-cells have tsitotok-ture activity against myofibrils. In dermatomyositis developing humoral immune
response, leading to complement activation that is accompanied by a vascular lesion microvasculature. The
relevance of a broad spectrum of myositis-specific AT: to the amino-atsilsintetazam tRNA, signal recognition
particles, protein-nuclear complex Mi-2, etc.

Pathomorphology

In the morphological study of patients with dermatomyositis skin reveal productive and productive-destructive
vasculitis all layers of the dermis, perivaskulyar-ing lymphocytic infiltration, thinning of the epidermis, sclerosis of
the dermis in the affected areas. In muscle biopsy, except for vasculitis reveal cellular infiltrates in perimizii and
around blood vessels and veins in microvasculature, consisting mainly of lymphocytes and macrophages, histiocytes
and plasma cells. Diagnostic significance is the discovery of large macrophages, infiltrated the nekrotieirovannye
muscle, with signs of excessive phagocytosis. Necro-biotic processes in the myofibrils are combined with their
pronounced regeneration. In the long course of the disease reveal atrophy of muscle fibers, increase in fibrosis and
sclerosis of the endo-and perimiziya.

Clinical picture

Children dermatomyositis often begins acute or subacute in onset of the disease often occur fever, weakness,
malaise, weight loss, myalgia, arthralgia, progressive decline in muscle strength. The clinical picture of
dermatomyositis usually polisindromna, but the most characteristic changes of the skin and muscles.

Skin lesion

The skin lesion - a characteristic feature of dermatomyositis. By the cutaneous manifestations of dermatomyositis
include erythematous rash with a purple tinge on the face of paraorbitalnoy (symptom "dermatomiozitnyh points;
Fig. 13/10 inset), in the neck, above the metacarpophalangeal and proximal interphalangeal joints hands (a sign
Gottrona) and over the large limb joints, especially elbow and knee. In the acute phase patients often note a
superficial skin necrosis in the field of defeat, and later developed atrophy, with areas of depigmentation. In some
patients, redness, peeling and cracking of the skin of the palms (hand mechanics ").

In children, patients with dermatomyositis usually occur bright livedo, especially in the shoulder and pelvic girdle,
the capillaries of the palms and feet, those-leangiektazii.Generalized vascular lesions especially true for children of
preschool age.

In acute and subacute course there is a pronounced trophic disturbances in the form dermatoxerasia, brittle nails,
alopecia.

The defeat of the subcutaneous tissue

Over the affected muscles of the limbs and face often appears testovaty or dense edema. Perhaps the development of
partial lipodistro-IFE face and limbs, usually associated with muscle atrophy.
The defeat of the muscles

Usually at the beginning of the disease dermatomyositis patients complain of fatigue during physical exertion,
muscle pain, occurring spontaneously and growing at a palpation and movements. For dermatomyositis is
characterized by a symmetrical lesion primarily proximal limb muscles, thus children can not wear a briefcase in
hand, it is difficult to lift up their hands and hold them in position, they can not own your hair (a symptom of a comb
"), dress (" symptom shirts "), quickly tire of walking, often falling, can not climb stairs, rise from a chair, lift and
hold his legs. In severe lesions of the neck muscles and spine patients are unable to tear his head from the pillow,
turn and get out of bed. In the most severe cases, a generalized muscle weakness with an emphasis on the proximal
group, so that patients can be almost completely immobilized.

Any damage to the muscles of the larynx and pharynx appears twang and hoarseness and swallowing difficulties,
which can lead to aspiration

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food and saliva. Any damage to facial muscles celebrate maskoobraznost persons with lesions of the oculomotor
muscles - diplopia and ptosis of the eyelids. The heavy defeat of the diaphragm and intercostal muscles leads to a
violation of breath. At the end polimiozita develops hypotrophy of muscles.

In children, unlike adults, are often formed persistent, sometimes painful tendon-muscle contractures, sharply
limiting the amount of movement.

The defeat of the joints

The defeat of the joints observed in more than 75% of patients. Developing arthralgia or polyarthritis. Most
commonly affects the small joints of hands (mostly proximal interphalangeal), knee and elbow. Articular changes
are characterized by moderate defiguratsiey and tenderness to palpation and movement. In most cases, articular
syndrome quickly docked at the background of treatment, only 25% of patients noted the formation of contractures,
deformities and subluxations at interphalangeal joints, with some limited functionality.

Calcinosis

Calcinosis in dermatomyositis in children occurs 3-4 times more frequently than in adults. It occurs in up to 40% of
patients mostly in terms of 1 to 5 years after onset. Calcinates may be limited in separate pockets or plates and
localized subcutaneous or connective tissue around the muscle fibers, they can also be located in areas most
traumatic -- around the knee or elbow joints, along the Achilles tendon, on the thighs, buttocks, shoulders. Patients
with dermatomyositis continuous-flow recurrent calcinosis usually has a diffuse character.

The defeat of the internal organs

In dermatomyositis the most often develops myocarditis, manifested primarily disorders of rhythm and conduction,
reduced the contractile ability of cardiac muscle. In 25% of patients developed pericarditis with mild symptoms,
rapidly disappearing after the start of treatment with glucocorticoids.

Pulmonary disease (pneumonia) associated with the vascular-interstitial changes and manifested clinically
unproductive cough, shortness of breath, wheezing at auscultation erratic. Prognostically unfavorable development
of diffuse alveolitis with the formation of alveolar-capillary unit, the rapid development of pulmonary failure and
death.Pulmonary disease in dermatomyositis may also be due to the development of aspiration and banal hypostatic
pneumonia due to injury of muscles involved in swallowing and breathing. Often children find pleurisy, with a high
degree of activity of the process is sometimes accompanied by the formation fluid.
Renal disease is rarely reveal. Renal syndrome is presented transient urinary syndrome, in some cases accompanied
by impaired renal function until the development of acute renal failure due to massive myoglobinuria.

Often in children with high activity of the process of having esophagitis, gastroduodenitis, enterocolitis, perhaps the
development of erosive and ulcerative process, complicating perforation and hemorrhage. Occasionally observed
psevdoabdominalny syndrome resulting from the defeat of the anterior abdominal wall muscles, with swelling,
condensation, and a sharp breath and morbidity at a palpation.

LABORATORY STUDIES

In a laboratory study of patients in the active period of the disease usually reveal increased ESR, mild anemia, some
patients - moderate leukocytosis, hypergammaglobulinemia.

Among the biochemical parameters to characteristic changes, reflecting the failure of skeletal muscle, should be to
increase the activity of CPK and aldolase. In addition, patients often reveal an increase in the concentration of
lactate dehydrogenase and aminotransferases in serum. A number of patients there myoglobinuria.

Identification of myositis-specific AT is important primarily for the classification, ie clarify the clinical and
immunological subtype of dermatomyositis and polimiozita. In some patients to identify AT amino atsilsintetazam
tRNA, primarily to AT gistidil-tRNA syn-tetaze (Jo-1). In the presence of these AT blood develops antisinte-tazny
syndrome characterized by acute onset myositis, interstitial lung, fever, symmetrical arthritis, Raynaud's syndrome,
lesion of the skin brush in an "arm mechanics, incomplete response to the use of glucocorticoids, and part of the
development of exacerbations against the background of reducing their dose, the debut of the disease mainly in the
spring.

DIAGNOSIS

Developed the following criteria for diagnosis of dermatomyositis (Tanimoto etal, 1995).

1. Skin lesion.

Yes. Heliotropic rash - reddish-purple erythematous rash on the eyelids.

b.. Symptom Gottrona - red-purple scaly atrophic erythema or spots on the extensor surfaces of
metacarpophalangeal wrist-tether-doping and proximal interphalangeal joints.

into. Erythema on the extensor surface of extremities, over the elbow and knee joints.

2. The weakness of the muscles proximal extremities and trunk.

3. Increased activity of creatine and / or aldolase in the serum.

4. Myalgia or muscle pain on palpation.

5. Changes on EMG (short polyphasic motor unit potentials with spontaneous fibrillation potentials).

6. Detection of AT Jo-1.

7. No destructive arthritis or arthralgia.

8. Signs of systemic inflammation (fever over 37 C, increasing the concentration of C-reactive protein or increased
erythrocyte sedimentation rate 20 mm / h).
9. Histological changes: inflammatory infiltrates in skeletal muscle with degeneration or necrosis of muscle fibers,
active phagocytosis, or signs of active regeneration.

In the presence of the first and all four of the following criteria for diagnosis of dermatomyositis are considered
reliable. Diagnosis of dermatomyositis is difficult in cases where the disease debut single syndrome, especially when
the syndrome is the leading myopathy, and pale skin changes.

In the presence of typical skin and miopaticheskogo diagnosis of dermatomyositis syndromes usually causes fewer
problems, but in infants, early diagnosis is difficult due to the complexity of the identification of muscle weakness
and correct interpretation of symptoms.

DIFFERENTIAL DIAGNOSIS

The differential diagnosis of dermatomyositis should be conducted with polyneuropathy, diseases of the defeat
neuromuscular junction (myasthenia gravis), progressive muscular dystrophy (Erb, miodistrofiya Landuzi-dejerine),
endocrine myopathy, infectious myositis. Muscle weakness should be distinguished from the general weakness and
muscle fatigue, resulting in many diseases, in particular with anemia in young children with rickets, malignant
tumors, etc.

TREATMENT

Drug of choice for treatment of dermatomyositis are glyukokortikoi-ing short-acting - prednisolone, and
methylprednisolone. They determine the dose according to the severity of the patient, disease activity and
effectiveness of previous therapy. With high activity during the initial dose of prednisone for oral administration of
1-1,5 mg / kg / day (not more than 60 mg / day), with moderate activity - 0,5-0,9 mg / kg / day. Clinical effect in the
treatment of patients with dermatomyositis develops more slowly than in SLE, so the maximum treatment dose
glucocorticoids spend long (up to 2 months). In a subsequent decrease to the extent of disease activity and improve
the patients' doses slow down to maintain (at least 10-15 mg / day). Treatment doses support should continue for
many years, which will help prevent relapses.

For patients with more severe forms of dermatomyositis spend pulse methylprednisolone therapy: the drug is
administered intravenously at a dose of 10-20 mg / kg / day (1000 mg / day) for three consecutive days or more,
allowing for a shorter time to suppress activity of the pathological process. For patients with a crisis of
miopaticheskim and torpid to ongoing standard therapy used plazmafe-cut, synchronizing it with the holding of
pulse therapy with glucocorticoids, which allows to achieve positive results including patients resistant to steroid
therapy.

In recent years, for the treatment of patients with dermatomyositis already in the early period after onset of the
disease in combination with glucocorticoids is widely used methotrexate. The drug is administered orally in a dose
10-12,5 mg / m 2 / week (up to 20 mg / week given tolerance), treatment is carried out for a long time. Combination
therapy makes it easier to achieve clinical effect and to start reducing the dose glucocorticoids, which reduces the
severity of their side effects, prevents the progression of calcification.

For the treatment of calcification apply etidronovuyu acid (ksidifon), 2 EDTA.

FORECAST

Weather in juvenile dermatomyositis is less favorable than in dermatomyositis in adults. Deaths occur
predominantly in the first years after the onset of the disease on the background of high activity process and
krizovogo flow. The survival rate of patients after 5 years after diagnosis on average more than 90%. At the same
early diagnosis and effective long-term treatment, most patients can achieve prolonged remission for many
years. The worst prognosis in children who had taken ill at an early age, as well as in patients with severe
gastrointestinal tract, lungs.