Symptoms of systemic lupus erythematosus

Symptoms of systemic lupus erythematosus are characterized by pronounced polymorphism, but almost monoclonal variants of the debut of the disease are observed in almost 20% of children. The course of systemic lupus erythematosus usually wavy, with alternating periods of exacerbations and remissions. In general, systemic lupus erythematosus in children is characterized by a more acute onset and course of the disease, earlier and rapid generalization and less favorable outcome than in adults.

[1], [2], [3], [4], [5], [6]

Common symptoms of systemic lupus erythematosus

The first symptoms of the onset of systemic lupus erythematosus or its aggravation in most children are fever (usually intermittent), increasing weakness, malaise, decreased appetite, weight loss, increased hair loss.

[7], [8], [9]

Lesion of the skin and its appendages in systemic lupus erythematosus

Skin syndrome, noted in children with systemic lupus erythematosus, is very diverse.

The lupus "butterfly" is the most characteristic manifestation of systemic lupus erythematosus, which is noted in 80% of patients, 40% of them in the debut of the disease. "Butterfly" is a symmetrical erythematous rash on the skin of the face, located in the zygomatic area and the region of the nose, in shape resembling the figure of a butterfly with open wings; rashes can spread beyond the zygomatic area to the skin of the forehead, chin, free edge of the ear and its lobes.

Lupus "butterfly" can be in the form of:

• erythema, manifested by flushing of the skin with clearly delineated boundaries, infiltration, follicular hyperkeratosis followed by cicatricial atrophy;
• bright erysipelatous inflammation with infiltration, hyperemia, small necrosis, covered with crusts, and edema of the face;
• centrifugal erythema of persistent erythematous-edematous spots with blurred follicular hyperkeratosis, located in the center of the face;
• vasculitic "butterfly" - unstable diffuse reddening with a cyanotic shade in the middle zone of the face, which increases with excitement, exposure to insolation, etc.

Erythematous eruptions can also occur in open areas of the skin: the upper third of the chest and back (the "decollete" area), above the elbow and knee joints.

Discoid foci are erythematous eruptions with hyperemic margins and depigmentation in the center, infiltration, follicular hyperkeratosis and subsequent cicatricial atrophy. Localized mainly on the skin of the scalp, face, neck, upper limbs. In children, such rashes are observed usually in chronic course of systemic lupus erythematosus.

Photosensitivity - increasing the sensitivity of the skin to the effects of solar radiation, typical for patients with systemic lupus erythematosus. Typical erythematous skin rashes usually appear in children in the spring-summer period, their brightness increases after exposure to the sun or treatment of UFOs.

Capillaryitis - edematous erythema with telangiectasia and atrophy on the finger pads, palms and plantar surface of the foot, observed in most children in the acute period of the disease.

Hemorrhagic eruptions in the form of petechial or purpurous elements, usually located symmetrically on the skin of the distal parts of the extremities, especially the lower ones, are often noted in children with systemic lupus erythematosus as cutaneous vasculitis.

Mesh liveno (bluish-purple spots forming a net on the skin of the lower, less often upper limbs and trunk) and subungual microinfarctions (thrombovascular of the capillaries of the nail bed).

Nonspecific skin rashes are often observed in patients with high activity of systemic lupus erythematosus; they can be represented by all the basic morphological types of skin elements: from patchy-papular to bullous.

Reynaud's syndrome (periodically developing ischemia of the fingers, due to vasospasm and structural vascular lesions) is observed in children much less often than in adults.

Alopecia is very characteristic of patients with systemic lupus erythematosus. In the active period of the disease, patients notice thinning and increased hair loss, which leads to nodular or diffuse alopecia.

Lesion of mucous membranes with systemic lupus erythematosus

The defeat of the oral mucosa, which is noted in more than 30% of children mainly in the active period of the disease, includes:

• lupus enanthema (erythematous-edematous spots with clear boundaries and sometimes with an erosive center, located in the region of a solid palate);
• aphthous stomatitis (painless erosive or, more rarely, deeper ulcerous foci with keratotic rim and intense erythema);
• cheilitis - the defeat of the red border of the lips, often the lower one (underlined rim along the lip of the lip, puffiness, hyperemia, the formation of cracks, in some cases, erosion and sores, followed by the development of cicatricial atrophy).

Lesion of joints with systemic lupus erythematosus

Articular syndrome is observed in practically all children with systemic lupus erythematosus, and in 80% of them already in the initial period of the disease. This syndrome has a migratory nature of lesions, rarely leads to the formation of persistent deformities, with the exception of symmetrical spindle-shaped deformations of the proximal interphalangeal joints of the II-IV fingers of the hands without affecting their function.

Arthralgia is characteristic of the active period of the disease. They are localized in the large and small joints of the extremities, most often the knee, ankle, elbow and proximal interphalangeal joints of the fingers of the hands, rarely in the shoulder, elbow, wrist, sometimes in the hip joints and the cervical spine.

Arthritis. Acute arthritis in systemic lupus erythematosus usually occurs with multiple, more often symmetrical lesions of joints (primarily proximal interphalangeal fingers, knee, ankle, elbow), accompanied by marked periarticular reactions, painful contractures, but quickly disappears after the start of treatment with glucocorticosteroids. Subacute and chronic polyarthritis is characterized by a longer, wavy and often progressive course, tenderness, mild exudation, accompanied by restriction of the function of affected joints, complaints of short morning stiffness. Radiologic examination reveals moderate changes in the form of epiphyseal osteoporosis (stage I according to Steinbroke).

Bony changes in systemic lupus erythematosus

Aseptic necrosis, characterized by bone-cartilaginous sequestration with secondary osteosclerosis, is much less common in children than in adults. Usually they are localized in the region of the epiphysis of the femoral head (rarely in other bones), leading to a disruption of the function of the affected limb and disability of the patient.

[10], [11], [12], [13], [14], [15], [16], [17], [18]

Muscle damage in systemic lupus erythematosus

Muscle damage is observed in 30-40% of children in the active period of systemic lupus erythematosus. It is expressed by myalgia or polymyositis involving symmetrically located, more often proximal limb muscles.

In polymyositis, except pain in the muscles, they note their soreness in palpation, a slight decrease in muscle strength, a moderate increase in the activity of enzymes of muscle decay (creatine phosphokinase - CKK, aldolase). The outcome of polymyositis can be the development of moderate hypotrophy. Lupus polyneositis should be differentiated from steroid myopathy, which develops in patients on the background of treatment with glucocorticosteroids.

The defeat of serous membranes

The defeat of serous membranes (polyserositis) - a characteristic manifestation of systemic lupus erythematosus, is observed in 30-50% of children.

Pleurisy is usually symmetrical, dry, less often effusive, rarely has a vivid clinical manifestation. Clinically, the development of pleurisy can be manifested by coughing, the appearance of pain in the chest, increasing with deep breathing, noise of friction of the pleura during auscultation. On radiographs, the thickening of the costal, interlobar or mediastinal pleura is noted, as well as pleuropericardial adhesions. In some cases, a noticeably massive accumulation of exudate in the pleural cavities.

Pericarditis in children is observed more often than in adults. Typical clinical signs of pericarditis include tachycardia, dyspnoea, friction noise, but in most cases pericarditis is clinically small-symptomatic, it is only detected with EchoCG: a thickening and separation of epi- and pericardial sheets are noticeable in the picture. With high activity of the disease, pericarditis is usually accompanied by accumulation of exudate. Rarely, when massive swelling occurs, there may be a risk of cardiac tamponade. With rarely occurring constrictive pericarditis, spicules form in the pericardial cavity until it is obliterated.

In some cases, patients see a picture of aseptic peritonitis.

The defeat of the respiratory system in systemic lupus erythematosus

Observe in 10-30% of children with systemic lupus erythematosus at different stages of the disease.

Acute lupus erythematosus is occasionally observed with high activity of the disease, manifested by a symptomatic complex characteristic of pneumonia (cough, dyspnea, acrocyanosis, evidence of a weakening of breathing and wheezing in the lungs during auscultation, etc.). On the roentgenogram in these cases usually find symmetrically located infiltrative shadows in the lungs, discoid atelectasis.

Chronic diffuse interstitial lung disease can be formed with a relatively prolonged course of systemic lupus erythematosus. Physical signs of lung damage are scarce or absent. With the help of methods of functional diagnostics, a decrease in the function of the lungs, a violation of pulmonary blood flow, on the roentgenogram note the strengthening and deformation of the vascular interstitial pattern with the loss of the sharpness of its outlines and the widening of the lumen of the vessels.

Pulmonary (alveolar) hemorrhages, which are very rare in children, can lead to death. Patients with this note signs of acute respiratory distress syndrome with a rapid decrease in hemoglobin and hematocrit and the development of severe hypoxemia.

Pulmonary hypertension in children is very rare, it is usually formed with antiphospholipid syndrome (AFS).

Characteristically high diaphragm position due to diaphragmatis, pleurodiaphragmatic adhesions and adhesions, reduction in muscle tone of the diaphragm.

Heart failure with systemic lupus erythematosus

The defeat of the heart in children with systemic lupus erythematosus is observed in 50% of cases.

Myocarditis in severe course is characterized by the widening of the borders of the heart, changes in the sonority of the tones, disturbances in the heart rate and conduction, a decrease in the contractility of the myocardium, and the appearance of signs of cardiac insufficiency. With high activity of the disease, myocarditis is usually combined with pericarditis. In most cases, myocarditis has poor clinical signs, it is diagnosed only with a comprehensive instrumental study.

Probably formation in patients with myocardial dystrophy.

Endocarditis. In systemic lupus erythematosus, the valvular or parietal endocardium can be affected. In most cases, valvulitis of mitral, less often aortic or tricuspid valves is observed or its consequences in the form of densification of the valves that do not cause hemodynamic disturbances and do not create conditions for the occurrence of organic noises. The formation of heart defects due to endocarditis in systemic lupus erythematosus is not typical and is extremely rare.

Systemic lupus erythematosus is characterized by atypical warty endocarditis of Liebman-Saksa with the formation of warty overlays with a diameter of 1-4 mm in the sites of small ulcers of the endocardium and possible appearance of small perforations of valve flaps and rupture of chords.

Coronary (coronary artery vasculitis), which causes violation of myocardial perfusion, may be accompanied by pain behind the sternum or in the heart, but usually proceeds clinically asymptomatically. Single cases of development in adolescents of myocardial infarction were noted .

Kidney damage in systemic lupus erythematosus

Nephritis is clinically diagnosed in 70-75% of children with systemic lupus erythematosus, most of them develop within the first 2 years from the onset of the disease, and about a third - already in the debut. The prognosis and outcome of the disease as a whole largely depend on the nature of the kidney damage.

In the morphological study of the kidneys, signs of immunocomplex glomerulonephritis of various types are noted.

Classification of the World Health Organization for kidney damage in systemic lupus erythematosus

A type	Description	Clinical and laboratory signs
I	No change in light, immunofluorescence and electron microscopy data	None
IIA	Mesangial glomerulonephritis with minimal changes (absence of light-optical changes in the biopsy specimen in the presence of deposits of immune complexes in the mesangium according to immunofluorescence and electron microscopy)	None
IIb	Mesangial glomerulonephritis (varying degrees of mesangial hypercellularity with the presence of immune deposits in mesangium)	Proteinuria <1 g / day, erythrocytes 5-15 per p / sp
III	Focal proliferative glomerulonephritis (active or chronic, segmental or total, endo- or extra-capillary injury involving less than 50% of the glomeruli)	Proteinuria <2 g / day, erythrocytes 5-15 per p / sp
IV	Diffuse proliferative glomerulonephritis (the same changes as in class III involving more than 50% of the glomeruli)	Proteinuria> 2 g / day, erythrocytes> 20 in p / zr, arterial hypertension, renal failure
V	Membranous glomerulonephritis (uniform thickening of the glial basement membrane due to subepithelial and intramembrane deposition of immune complexes)	Proteinuria> 3.5 g / day, lean urine sediment
VI	Chronic glomerulosclerosis (diffuse and segmental glomerulosclerosis, canal atrophy, interstitial fibrosis, arteriolosclerosis)	Arterial hypertension, renal failure

The classification of lupus nephritis in children, based on clinical data (VI Kartasheva, 1982), includes:

• severe nephritis with nephrotic syndrome (NS) (characterized by diffuse edema, massive proteinuria, hypoproteinemia, hypercholesterolemia, severe hematuria in most cases with persistent arterial hypertension and hyperaemia);
• nephritis of severe form without nephrotic syndrome (characterized proteinuria with protein loss in the range of 1.5-3 g / day, significant erythrocytic activity, often macrohematuria, moderate arterial hypertension and azotemia);
• nephritis of latent form (characterized by a mildly expressed urinary syndrome: proteinuria <1.3 g / day, hematuria <20 erythrocytes per p / sp).

The most unfavorable prognosis is likely with rapidly progressing lupus nephritis, characterized by the presence of a nephrotic syndrome, severe (sometimes malignant) arterial hypertension and the rapid development of renal failure leading to an unfavorable outcome within a few weeks or months.

In addition to glomerulonephritis, the spectrum of renal pathology in systemic lupus erythematosus includes tubulointerstitial lesions, as well as thrombotic lesions of vessels of different calibrations within the framework of APS.

Lesion of the gastrointestinal tract with systemic lupus erythematosus

Gastrointestinal disorders are noted in systemic lupus erythematosus in 30-40% of patients. In an acute period, usually a decrease in appetite, dyspeptic disorders. When endoscopic examination is often diagnosed inflammatory lesions of the mucosa of the esophagus, stomach and duodenum, sometimes with the formation of erosions and even ulcers.

Bowel involvement is relatively rare and is mainly caused by mesentery vascular lesions. Vasculitis of the mesenteric arteries with the subsequent development of thrombosis can lead to hemorrhage, infarctions and necrosis followed by perforation and development of intestinal bleeding or fibrinous purulent peritonitis. A symptomatic complex of malignant current Crohn's disease (terminal ileitis) is possible.

Lesion of the liver. Hepatomegaly of different degree, more often reactive character, is diagnosed in most patients with systemic lupus erythematosus. In 10-12% of patients, along with hepatomegaly, there is a moderate increase in the level of liver enzymes (usually 2-3 times), caused by thrombotic microangiopathy.

In some cases, it is possible to develop hepatitis, liver infarctions, thrombosis of the hepatic veins (Badd-Chiari syndrome).

The defeat of the pancreas (pancreatitis) may be a consequence of the pathological process within the systemic lupus erythematosus or due to the effects of high doses of glucocorticosteroids.

The defeat of the nervous system in systemic lupus erythematosus

Psychoneurological symptoms in systemic lupus erythematosus are extremely diverse, since it is possible to damage any parts of the nervous system. Characteristic for the clinic of systemic lupus erythematosus manifestations are observed in 30-50% of children.

Organic brain syndrome, the development of which is caused by thrombotic vasculopathy or diffuse damage mediated by antineuronal antibodies, is accompanied by a worsening of cognitive functions (memory, attention, thinking), which can lead to a marked decrease in intelligence, and the emergence of emotional-personality disorders (emotional lability, irritability, apathy , depression).

Mental disorders in systemic lupus erythematosus in children differ in clinical polymorphism, a tendency to recur, their severity usually correlates with the severity of somatic disorders. With high activity, it is possible to develop acute psychosis with the emergence of productive symptoms in the form of visual and auditory hallucinations, schizophreniform disorders, affective syndromes (manic and depressive), motor anxiety, sleep disturbances, etc.

Headaches, including migraine, usually intense, are observed in the active period of the disease, usually in patients with systemic lupus erythematosus with antiphospholipid syndrome.

The convulsive syndrome, usually manifested by generalized epileptiform seizures, is characteristic of highly active systemic lupus erythematosus.

Chorea, similar to a small chorea with rheumatism, can be unilateral or generalized.

Transitory disorders of cerebral circulation characterized by cerebral, focal or mixed symptoms lasting no more than 24 hours, and ischemic stroke are observed in children much less often than in adults. Their occurrence is due to thrombosis or thromboembolism of the intracerebral arteries in the presence of antiphospholipid antibodies.

Intracerebral hemorrhages can be caused by arterial hypertension or thrombocytopenia, subarachnoidal hemorrhage and subdural hematoma - cerebrovascular infection.

Spinal cord injury due to ischemic necrosis and demyelination of the fibers is rarely observed in children. It can be accompanied by a symmetrical lesion of the thoracic spinal cord. In this case, diagnosed lower paraparesis, a violation of sensitivity in the lower half of the trunk, pelvic disorders, severe back pain. The prognosis for "transverse myelitis" is unfavorable.

The defeat of the cranial nerves (oculomotor, trigeminal, facial or visual) can be isolated or combined with other stem symptoms.

The defeat of the peripheral nervous system proceeds according to the type of symmetrical distal, mainly sensory polyneuropathy, rarely - multiple mononeuropathy. In some cases, the Hyenna-Barre syndrome develops (acute inflammatory polyradiculoneuropathy).

The defeat of the nervous system in systemic lupus erythematosus can have a secondary character and be due to arterial hypertension, uremia, hypoxemia, infectious diseases, the intake of glucocorticosteroids (lead to steroid psychoses), etc. Specification of the genesis of lesion of the nervous system is necessary for pathogenetically substantiated treatment.

Cumulative assessment of lesions of various organs in systemic lupus erythematosus

To determine the severity of organ damage in systemic lupus erythematosus, the damage index of the SLICC / ACR Damage Index is determined. The score includes all types of damage from the onset of the disease due to systemic lupus erythematosus and developed as a result of ongoing therapy and persisting for 6 months or more.

Index of damage in systemic lupus erythematosus SLICC / ACR Damage Index

Symptom	Score, points
Vision (each eye) in clinical evaluation
Any cataract	1
Changes in the retina or atrophy of the optic nerve	1
Nervous system
Cognitive impairment (memory loss, difficulty with counting, poor concentration, difficulties in speaking or writing, violation of the level of performance) or great psychosis	?
Seizures that require treatment more than 6 months	1
Strokes ever (score 2 points, if more than one episode) or cerebral resection, not associated with neoplasm	1-2
Cranial-cerebral or peripheral neuropathy (with the exception of visual)	1
Transverse myelitis	1
Kidneys
Glomerular filtration <50 ml / min	1
Proteinuria> 3.5 g / day	1
The final stage of kidney disease (regardless of dialysis or transplantation)	3
Lungs
Pulmonary hypertension (right ventricular bulging or sonorous tone II over the pulmonary artery)	1
Fibrosis of the lung (physically and radiologically)	1
Wrinkled lung (radiologically)	1
Pleural fibrosis (radiologically)	1
Infarction of the lung (radiological)	1
The cardiovascular system
Aortocoronary bypass surgery	1
Myocardial infarction ever (score 2 points, if> 1)	1-2
Cardiomyopathy (ventricular dysfunction)	1
Damage to valves (diastolic or systolic murmur> 3/6)	1
Pericarditis within 6 months (or pericardectomy)	1
Peripheral vessels
Intermittent claudication within 6 months	1
A small loss of tissue ("pads" of fingers)	1
Significant loss of tissue ever (loss of a finger or limb) (score 2 points, if more than one place)	1-2
Vein thrombosis with edema, ulceration, or venous stasis	1
Gastrointestinal tract
Infarction / resection of the intestine (below the duodenum), spleen, liver or gall bladder ever for any reason (score 1 point if more than one place)	1-2
Mesenteric insufficiency	1
Chronic peritonitis	1
Strictures or surgeries on the upper gastrointestinal tract	1
Musculoskeletal system
Muscle atrophy or weakness	1
Deforming or erosive arthritis (including deformable deformities, excluding vascular necrosis)	1
Osteoporosis with fractures or vertebral collapse (excluding avascular necrosis)	1
Avascular necrosis (score 2 points, if> 1)	1-2
Osteomyelitis	1
Rupture of tendons	1
Leather
Cicatricial chronic alopecia	1
Extensive scarring or panniculitis (except the scalp and pads)	1
Ulceration of the skin (excluding thrombosis) for more than 6 months	1
Sexual system
Premature failure of the gonads	1
Endocrine system
Diabetes mellitus (regardless of therapy)	1
Malignization
Excluding dysplasia (score 2 points, if more than one location)	1-2

[19], [20], [21], [22], [23], [24], [25]