SYSTEMIC VASCULITIS

Vasculitis (angiitis) - primary or secondary inflammation of the walls of blood vessels. Primary systemic vasculitis -
a group of diseases characterized by lesions in vascular wall of different caliber (from the microvasculature to the
aorta and its branches) by the type of focal inflammation and necrosis with subsequent involvement in the
pathological process of organs and tissues in the zone of vascular injury. Secondary vasculitis developed in
infectious, autoimmune, rheumatic, onkogematologiches-cal and other diseases.

Prevalence of diseases of this group of children is unknown. Shenlyayna-Schnlein's disease (hemorrhagic

vasculitis), juvenile polyarteritis, Kawasaki disease, Takayasu's arteritis (nonspecific aortoarteriit) occurs mainly in
childhood. In contrast to adults, systemic vasculitis in children although less developed, but are distinguished by the
exigencies of the flow, vivid manifest symptoms at the same time - a more optimistic prognosis if early and
adequate therapy.

Etiology and pathogenesis

The etiology of the majority of primary vasculitis is not known. It is believed that their children may have can
contribute to frequent acute infectious diseases, chronic foci of infection, drug allergy, genetic predisposition to
vascular or rheumatic diseases. Bacterial or viral infections (streptococcal, ger-pesvirusnaya, parvovirus, hepatitis B
or C) on the one hand, allergy, or weighed down allergologic history on the other hand, according to experts, are the
equal of the two factors forming the backdrop for gipersensibilizatsii organism or acting as a trigger, provoking
moments.

Of great importance in the development of vasculitis are the formation, circulation and deposition in vascular walls
of immune complexes, antineytrofil-tion of cytoplasmic AT, immune proliferative-destructive inflammation of the
walls of arteries of different caliber, vascular endothelial damage, increased vascular permeability, hypercoagulation
in violation of blood flow, ischemia in the zone of injury vessels.

Pathomorphology

Destructive-proliferative vasculitis is a systemic nature. If the fibrinoidnogo necrotic arterial wall is a gap elastic
frame, the first formed aneurysm. In predominantly segmental proliferative inflammation develop stenosis and
occlusion of the lumen of the artery. Changes in the damaged part of the vessel are diverse - from ischemia to
hemorrhage, necrosis, infarction. The degree of vascular disorders depends on the caliber of the affected arteries and
the state of collateral circulation.

Clinical picture

In the initial period of vasculitis observed common manifestations of nonspecific inflammatory syndrome: low-
grade febrile, or fever, arthralgia, weight loss, symptoms of peripheral and visceral vascular disorders, mild anemia,
leukocytosis in the peripheral blood, increased erythrocyte sedimentation rate, signs of hypercoagulability,
Dysproteinemia, increased C-reactive protein, IgA, CEC and cryoglobulins. However, along with the general
features of each disease peculiar to their own clinical syndromes (Table 13-12). The peculiarity of the clinical
manifestations of vasculitis due to localization, caliber of the affected vessels, the prevalence of pathological
process, the peculiarities of the morphological changes (the predominance of destructive or proliferative processes),
the degree of disorders of hemodynamics and ischemic organs and tissues.
Table 13.12. The clinical syndromes of juvenile forms of systemic vasculitis

Name Characteristic clinical syndromes

disease
Disease Cutaneous bleeding, joint, abdominal,
Shenlyayna -
Schnlein Kidney
Kawasaki Feverish, mucous membrane lesions (conjunctivitis, pharyngitis), cutaneous, limfonodulyarny,
disease heart with a possible formation of aneurysms of the coronary arteries

Nodular Abdominal, renal, arterial hypertension,

polyarteritis Multiple asymmetric mononevritichesky, skin, trombangiitichesky

Takayasu Asymmetry, or lack of pulse, fatigue, intermittent claudication, cerebrovascular, abdominal, and
disease pulmonary arterial hypertension

Diagnosis

The diagnosis of each of the systemic vasculitis based on the characteristic clinical syndromes. Laboratory findings
reflect only the inflammatory activity. According to testimony from conducting research tool, allowing to identify
the level of vascular lesion and the nature of deformation of blood vessels, signs of extra-and intraorganic
circulation (ultrasound, Doppler, CT, MRI, REG, rheovasography, aorta and koronarogra-raphy), and diagnostic
biopsy.

General principles of treatment

Choice of methods of treatment of disease involves the impact on the possible causes and basic mechanisms of
disease development.

The suppression of immune inflammation by prescribing anti-inflammatory and immunosuppressive action:

glucocorticoids (prednisolone, methylprednisolone), cytostatics (cyclophosphamide, techniques treksat).

Removal Ar, CEC: intravenous Ig, plasmapheresis synchronized with pulse therapy with glucocorticoids and / or
cytostatics.

Correction of hemostasis: the appointment of anticoagulants, antiplatelet agents.

Symptomatic therapy.

The treatment prescribed based nosological diagnosis, phase of the disease and its clinical features. The effect of
treatment was evaluated by dynamics of clinical syndromes and laboratory parameters. Treatment in the acute phase
of illness spend in hospital, then continue with the mandatory outpatient dispensary observation and control.

Clinical supervision

Children suffering from systemic vasculitis, put on dispensary observation in rheumatology. If necessary for the
inspection attract neurologist, ophthalmologist, dentist, otolaryngologist and surgeon. It is recommended to conduct
monthly inspections during the year after discharge from the hospital every 3 months, then - 1 times in six
months. Objectives of clinical examination: addressing the issue of disability, development of individual treatment,
to the systematic clinical and laboratory examination, monitoring of treatment, prevention of medical complications,
readjustment of foci of infection. Preventive vaccination can be performed during remission, and only killed
(inactivated-doped) vaccines.

DISEASE SHENLYAYNA-Schnlein

Shenlyayna-Schnlein's disease (hemorrhagic vasculitis) - widespread systemic disease with a primary lesion
mikrotsirku-lyatornogo channel. The disease usually occurs between the ages 7-12 years. Frequency of 13,5:100
OOO children.

Clinical picture

The disease usually begins acutely with subfebrile, less febrile fever (sometimes without the temperature of the
reaction), with one or several specific syndromes, depending on what distinguish simple and mixed forms of the
disease.

Cutaneous syndrome. The defeat of the skin as purpura occurs in all patients, usually early in the disease,
sometimes - after abdominal or other characteristic syndrome.The skin appears melkopyatnistaya or maculopapular
haemorrhagic rash. The rash is symmetric, localized mainly in the skin of the lower extremities, buttocks, around the
large joints, at least - on the upper extremities, trunk, face. The intensity of the rash varies - from single items to an
abundant discharge, sometimes in combination with angioedema. Eruptions wavy recur, are gathering in the distal (
"sock disease"). Hemorrhagic elements leave behind pigmentation, passing without a trace.

Articular syndrome. This is the second most common sign of illness. Severity of joint damage varies from
arthralgias to reversible arthritis predominantly large joints.

Abdominal syndrome. It occurs in almost 70% of children as a result of edema and hemorrhage in the wall of the
intestine, mesentery, or peritoneum. The most common symptom - cramping abdominal pain. Painful attacks can be
repeated many times during the day and accompanied dispeptiches-Kimi disorders such as nausea, vomiting, watery
stools mixed with blood.

Renal syndrome. Renal disease occurs less frequently and is manifested hematuria varying degrees of severity,
sometimes - the development glomerulonef Rita. Nephrotic syndrome and renal insufficiency occur rarely. It is the
development of glomerulonephritis may deprive the child a chance to recover. The course of acute illness often
cyclical, with recovery within 2 months, but may be protracted, recurrent and may last 6 months, rarely a year or
more. Chronic characteristic variations with the development of glomerulonephritis or with isolated, continuously
recurrent cutaneous hemorrhagic syndrome.

Complications. In the development of abdominal syndrome possible surgical complications (intussusception,

intestinal obstruction, perforation of the colon with peritonitis).Glomerulonephritis may be complicated by acute
renal failure or CRF.

Diagnosis

The diagnosis is established by the nature of acute cutaneous syndrome emerged, primarily by the presence of
symmetrically located melkopyatnistoy hemorrhagic rash on my legs. Difficulties arise if the first manifestation of
the disease are joint pain, abdominal pain or changes in the urine. In these cases, the diagnosis may be set before the
subsequent appearance of a typical rash.

Treatment

In the acute period of compulsory hospitalization, bed rest, hypo-allergens diet. Shown basic heparin therapy and
antiagreganty-mi. The doses of heparin are selected individually depending on the severity of the disease (average of
300-500 U / kg, at least - higher). Treatment is carried out in a 4-6-8 week with the gradual removal of the
drug. From dezagre-Ganta appoint dipyridamole (3-5 mg / kg / day), pentoxifylline, from antiag-regantov tretgo
generation - indobufen (ibustrin), ticlopidine. In order to activate fibrinolysis use of niacin, ksantinola no-kotinat. In
severe disease course in the acute phase of applying pre-nizolon a daily dose of 0,5-1 mg / kg (short course),
hepatitis-permanent rinizatsiyu, intravenous reopolyglukine, glucose -novocaine mixture (ratio 3:1), on the
testimony of conduct sessions plasmapheresis synchronized with pulse methylprednisolone therapy. Antibiotics are
prescribed only in case of local infection.

Forecast

60-65% of patients recover, but probably relapsing course. In the case of development of glomerulonephritis child
needs to monitor a nephrologist, as the outcome may be CRF.

Kawasaki disease

Kawasaki disease (mucous-cutaneous-lymphatic syndrome) - an acute systemic necrotizing vasculitis with damage
to large, medium and small arteries, combined with skin-mucous-glandular syndrome.

Sick children under 8 years (80%), mostly boys. The prevalence of the disease far exceeds the frequency of all other
forms of vasculitis, and rheumatic fever. According to experts, namely rheumatic fever and Kawasaki disease - the
leading cause of formation of acquired diseases of the heart and blood vessels. Frequency of Kawasaki disease in
Japan, China and Korea is 100-110:100 000 children under 5 years in the U.S. - 10-22:100 000, in Germany - 9:100
000, the incidence in Russia is unknown. The presence of the seasonal and cyclical variability of the disease
suggests its infectious nature, but so far to confirm this assumption failed.

Clinical picture

Disease begins acutely, increased body temperature, appear conjunctivas congestion, dryness and hyperemia of the
lips, oral mucosa. In 50-70% of patients with increased cervical lymph nodes on one or both sides. In the days align
intense erythema of the fingers and toes, polymorphic, or scarlet fever-like rash on the trunk, limbs and inguinal
areas, dense edema of hands and feet. May also appear arthralgia, cardiovascular megalia, muffled heart tones,
systolic hum, an increase in the size of the liver, diarrhea.Perhaps the formation of aneurysms of the coronary
vessels. High fever lasts from 12 to 36 days. At the 2 nd week, rash, conjunctivitis, swollen lymph nodes
disappeared, the language becomes a "raspberry", appears lamellated peeling fingers and toes.

Complications. Myocardial infarction, coronary artery rupture.

Laboratory research

During lab tests reveal leukocytosis and platelet-inos in the peripheral blood, increased ESR, anemia. Any damage
to the coronary vessels in the ECG to detect signs of myocardial ischemia, and with the help of Doppler and
coronary angiography - an aneurysm. By 6-10-th week, all clinical and laboratory symptoms disappear, there comes
healing. In the same time possible and sudden death.

Diagnosis

The diagnosis of Kawasaki disease determine the presence of five of the six major clinical criteria or four major and
koronarita (see below). The main criteria for Kawasaki disease follows.

Increased body temperature, lasts at least 5 days.

Hyperaemia conjunctivas.
 Inflammatory changes in the mucous membrane of the lips and mouth.

Palmar and plantar erythema with edema and subsequent peeling fingers.

Polymorphic rash.

Nonsuppurative increase of one or more lymph nodes (more than 1,5 cm in diameter).

Treatment

Treatment with antibiotics and corticosteroids is ineffective. Prevent the formation of coronary aneurysms and their
complications only helps treatment of acetylsalicylic acid and high doses of intravenously injected Ig. Ig prescribed
in a course dose of 1-2 g / kg body weight of the child (1 per day for 5 days) in combination with acetylsalicylic acid
(30 mg / kg / day for the duration of febrile period). Subsequently within 3 months prescribed maintenance dose of
acetylsalicylic acid (2-5 mg / kg / day). Aneurysms detected in 6% of cases in the treatment of Ig during the first 10
days of onset of fever and in 29% - with a later start of treatment. Left untreated, aneurysms of the coronary arteries
are formed by more than 60% of patients.

Forecast

Forecast more favorable. The majority of patients recover. In case of delay or lack of treatment the highest risk of
developing aneurysms noted in children under 1 year.Mortality is 0,1-0,5%. Death usually is caused by rupture of
aneurysm of coronary artery or myocardial infarction.

Uzelkov polyarteritis

Polyarteritis nodosa - acute, subacute or chronic disease, which is based on the defeat of the peripheral and visceral
arteries, mainly small and medium caliber. The ICD-10 identified three options for nodular poliarteriita.

Polyarteritis nodosa (classic).

Polyarteritis with lung (Churg-Strauss syndrome).

Juvenile polyarteritis.

The disease is now rarely reveal, especially in its classical variant, associated with HBV prevalence is unknown. The
disease occurs in children of all ages, juvenile polyarteritis noted more often in girls.

Clinical picture

The disease begins in most cases, acute. Appear remittent high fever, profuse sweats, severe pain in muscles, large
joints, stomach, exhaustion. During lab tests reveal neutrophilic hyperskeocytosis, a significant increase in ESR,
hypergammaglobulinemia, markers of hepatitis B virus (in the absence of symptoms of hepatitis). In a few weeks
(and at the beginning of a gradual - months) appears several characteristic clinical features: nodules (aneurysmal
changes of the arteries), livedo arborescens (blue-violet vascular network in the skin in the form of tree branches),
multiple mononevrit, cerebral vascular crises, abdominal pain , crown-rhythmic, arterial hypertension. In children,
eight times more frequently than in adults, develops trombangiitichesky syndrome - necrosis of skin, mucous
membranes, distal gangrene. Feature poliarteriita with lung - always preceded and accompanied the system Vasco
litas syndrome gipereozinofilnoy bronchial asthma.

In children, classic polyarteritis nodosa (with a primary lesion of the internal organs) and polyarteritis with lung
reveal rare, and the flow of these "baby" versions do not differ from the "adult". Mainly watching juvenile
polyarteritis (with a primary lesion of the peripheral vessels). The clinical picture varies depending on the variant,
the predominant localization and severity of vasculitis (Table 13-13).

Complications. Stroke, cerebral edema, myocardial infarction, renal failure, peritonitis, respiratory failure, infection
of necrosis.

Table 13-13. Typical symptoms different options nodular poliarteriita

Clinical Symptoms of acute phase

version
All versions Fever, pain in joints, muscles, hyperesthesia, weight loss

Classic Syndrome of hypertension renal origin,

Multiple mononevrit, cerebral vascular crises,
coronaritis, necrosis of the intestine
Juvenile Nodules, livedo, local edema, mononevrit, foci of necrosis
skin, mucous membranes, gangrene of the distal
Syndrome of Churg - Bronchial asthma or recurrent obstructive
Strauss syndrome in the history, gipereozinofilny syndrome in combination
with any of the symptoms mentioned above active
poliarteriita

Diagnosis

The diagnosis is established if the patient's typical clinical symptoms. Of the laboratory parameters take into account
the high degree of neu-trofilnogo leukocytosis and increased erythrocyte sedimentation rate, characteristic of
juvenile poliarteriitu, positive markers of hepatitis B virus, characteristic of the classical poliarteriita, and increased
eosinophils in the peripheral blood of above 20-30% for Churg-Strauss syndrome. In difficult cases, produce a
biopsy of skin and subcutaneous tissue, rarely - the kidneys, and possibly conduct aortography (renography).

Treatment

In the active period of treatment is carried out in hospital, in severe cases - in intensive care. All patients prescribed
prednizo-Lon in a daily dose of 1-2 mg / kg. At the high activity of the process and expressed vistseritah prescribe a
daily dose of cyclophosphamide 2-3 mg / kg daily or as pulse therapy (10-15 mg / kg 1 time per month for at least a
year). Patients with marked trombangiiticheskim, neurological, abdominal syndromes shows plasmapheresis
synchronized with pulse methylprednisolone therapy and / or cyclophosphamide. At high arterial hypertension used
only cyclophosphamide or combination with short course of prednisolone in low doses (less than 0.5 mg / kg). To
enhance anti-inflammatory effect prescribed phenylbutazone (butadion).

After a month of treatment (with positive outcomes) dose glucocorticoids are beginning to reduce
maintenance. Hormone treatment continued for at least 2 years. To improve the blood circulation used
anticoagulant, antiagreganty, angioprotektory. When expressed pain prescribe painkillers.

Forecast

Juvenile polyarteritis characterized by chronic recurrent course, but as they grow older the risk of formation of
gangrene is minimal. In the classic version of the possible long-term remission, but in the acute period (usually
within the first year of illness) is at high risk (40%) of death from complications of malignant hypertension. When
polyarteritis with lung can achieve stable remission of vasculitis, but bronchial asthma can be hormone-dependent.
NONSPECIFIC AORTOARTERIIT

Nonspecific aortoarteriit (Takayasu's disease) - a destructive-productive segmental aortitis and subaortal panarteriit,
characterized by the formation of aneurysms and / or stenosis of the aorta and its branches up to the segmental
arterial occlusion, which clinically manifested ischemic disorders and the syndrome of asymmetry and pulseless. In
those women sex are detected in 5-7 times more frequently than men. In most cases the disease begins in 10-20
years, in preschool children is rarely diagnosed. Incidence of 1,2-6,3 cases per 1 million population.

Clinical picture

The disease has two phases of development: an acute (from several weeks to several months or years), and chronic,
with exacerbation of waves or without them.

The beginning of the disease in most children acute. The first symptoms are nonspecific - fever, anorexia, arthralgia,
and myalgia, hemorrhagic or nodoznye rashes. As the disease progresses against a background of persistently
increased erythrocyte sedimentation rate (up to 40-60 mm / h) there are pains in the muscles of the hands (feet), or
fatigue during physical stress, a syndrome of intermittent claudication, numbness of fingers, tachycardia, expanding
the boundaries of the heart, circulatory system noise while auskulta - tion. frequent headaches, dizziness, blurred
vision and other symptoms of a local deficiency of blood circulation, depending on the level of damage
arteries.There are four options (type) of localization process (Table 13-14).

Table 13-14. Types nonspecific aortoarteriita *

Type Localization

I Aortic arch and the arteries branching off from her

II Descending, the abdominal aorta, celiac, renal, femoral
other arteries
III Mixed variant (common vascular lesions of
arch and other parts of the aorta)
IV The defeat of the pulmonary arteries, combined with any of the three types
disease

* By E. Lupi-Herrero et ah, \ 911

Characteristic syndrome of asymmetry and lack of pulse in the peripheral arteries, the difference of blood pressure
over 10 mmHg on symmetric limbs appear in 1-5 years from the onset of the disease, which to some extent creates
diagnostic difficulties. Dynamics of clinical manifestations of acute phase and some syndromes that persist in the
chronic phase, in no small part due to the place of injury and the degree of deficiency of blood circulation (Table 13-
15).

Table 13-15. Communication clinical syndromes with localization aortoarteriita

Arterial Manifestations

Subclavian, shoulder, thigh, The syndrome of intermittent claudication,

popliteal syndrome asymmetry or pulseless

Sleepy Headache, blurred vision,
 cerebrovascular accident
Pulmonary Pulmonary hypertension
Kidney Systemic hypertension in renal
origin
Celiac, mesenteric Abdominal pain, vomiting, diarrhea

The active period for the disease can last for months or even years, despite the clinical improvement during therapy.
Laboratory and instrumental investigations help to identify signs of activity of the process. The blood analysis
determined significantly increased ESR, anemia, increased concentration of C-reactive protein, IgA. C using
Doppler, duplex scanning, MRI, aortography can identify and trace the dynamics of deformation of the vascular bed,
change the linear velocity of blood flow, thickening of the walls of the aorta or narrowing of the mouths of major
arteries, arterial occlusion.

Complications are common lesions in the aorta, renal, carotid arteries. There may be separation wall aneurysm of
the aorta, rupture of the aneurysm, stroke, chronic renal failure.

Diagnosis

Diagnosis usually established only in identifying the syndrome of asymmetry or lack of pulse, usually at the end of
2-year illness. Possible an earlier diagnosis with careful search for causes local reduction of blood circulation in the
patient (mostly teenage girls) with elevated ESR of unknown etiology.

A careful inspection, palpation of pulse and blood pressure measurement on both arms and legs, auscultation of the
carotid, subclavian, femoral artery, the aorta to identify abnormal vascular noise, holding Doppler graph allows
diagnosis of nonspecific aortoarteriita.

Treatment

In the acute phase of prescribed prednisolone 0,5-1 mg / kg / day with dose reduction after 1-2 months to
maintenance, methotrexate for at least 10 mg / m 2 body surface 1 time per week, drugs that enhance collateral
circulation (pentoxifylline, Vinpocetine, etc.). With the onset of chronic phase within 1-2 years, continues to support
treatment prednisolone and methotrexate. According to the testimony produced surgery (vascular plasty, excision of
the aneurysm, etc.).

Forecast

The majority of patients after the acute phase of nonspecific aortoarteriita or more exacerbations, long time remain
fit for work. Any damage to the renal arteries and hypertension may develop chronic renal failure.