Diagnosis of juvenile systemic scleroderma

For the diagnosis of systemic scleroderma, preliminary diagnostic criteria for juvenile systemic scleroderma developed by European rheumatologists (Pediatric Rheumatology European Society, 2004) are proposed . To establish the diagnosis, 2 large and at least one small criterion are needed.

"Big" criteria

• Sclerosis / Induction.
• Sclerodactyly (symmetrical thickening, consolidation and induction of the skin of the fingers).
• Reynaud's syndrome.

"Small" criteria

• Vascular:
  • changes in the capillaries of the nail bed according to capillaroscopy data;
  • digital ulcers.
• Gastrointestinal:
  • dysphagia;
  • gastroesophageal reflux.
• Kidney:
  • kidney crisis;
  • the appearance of hypertension.
• Cardiac:
  • arrhythmia;
  • heart failure.
• Pulmonary:
  • pulmonary fibrosis (according to CT and radiography);
  • Diffusion of the lungs;
  • pulmonary hypertension.
• Musculoskeletal:
  • flexor tendon contractures;
  • arthritis;
  • myositis.
• Neurological:
  • Neuropathy;
  • syndrome of the carpal canal.
• Serological:
  • ANF;
  • specific antibodies (Scl-70, anti-centromeric, PM-Scl).

Laboratory research

Laboratory tests have a relative diagnostic value, but help to assess the degree of activity, the functional state of some internal organs.

• Clinical blood test. The increase in ESR, moderate transient leukocytosis and / or eosinophilia is noted only in 20-30% of patients, therefore their changes do not always correlate with the activity of the disease.
• The general analysis of urine, the analysis of urine according to Zimnitsky, the Reberg test is performed with suspicion of kidney damage - reveal a moderate urinary syndrome, a decrease in the filtration and concentration functions of the kidneys.
• Blood chemistry. Hyperproteinemia, mainly due to an increase in gamma-globulin fraction, is noted in 10% of patients.

Immunological research

The content of serum immunoglobulin G was increased in 30%, C-reactive protein - in 13% of patients with juvenile systemic scleroderma; rheumatoid factor is revealed in 20% of patients with systemic scleroderma, ANF (more homogeneous, mottled glow) - in 80% of patients, which indicates the activity of the disease and often determines the choice of more aggressive therapy.

Specific scleroderma antibodies - Scl-70 (antitopoisomerase) detect in 20-30% of children with systemic scleroderma, more often in the diffuse form of the disease, anticenteric antibodies - in about 7% of children with a limited form of systemic scleroderma.

Instrumental Research Methods

• Musculoskeletal system:
  • radiography of joints;
  • EMG for assessing the degree of muscle damage.
• Respiratory system:
  • examination of the function of external respiration;
  • Chest X-ray;
  • CT scan of high resolution (according to indications).
• The cardiovascular system:
  • ECG;
  • Echocardiography;
  • monitoring of ECG by Holter (according to indications).
• Gastrointestinal tract:
  • coprogramme;
  • Ultrasound of the abdominal cavity;
  • X-ray of the esophagus with barium;
  • esophagogastroduodenoscopy;
  • recto- and colonoscopy (according to indications).
• Nervous system:
  • electroencephalography;
  • MRI of the brain (according to indications).

Wide-field capillaroscopy of the nail bed reveals characteristic for systemic scleroderma signs - dilated capillaries, their reduction with the formation of avascular fields, the appearance of bush capillaries.

Differential diagnosis of systemic scleroderma

Differential diagnosis of systemic scleroderma should be carried out with other diseases of the scleroderma group: limited by sporoderma, mixed connective tissue disease, Busch scleroderma, diffuse eosinophilic fasciitis, as well as juvenile rheumatoid arthritis, juvenile dermatomyositis.

Scleroderm-like skin changes can also occur in some non-rheumatic diseases: phenylketonuria, progeria, cutaneous porphyria, diabetes, etc.

[1], [2], [3], [4], [5], [6], [7]