Diagnostic complications in the debut of systemic lupus erythematosus in children

Abstract

Systemic lupus erythematosus (SLE) is an autoimmune multisystem disease that is more severe in children than in adults. The mortality of patients with SLE without treatment reaches 95.3% during the first 5 years from the onset of the disease. SLE develops more often in women. The ratio between girls and boys affected by SLE in prepubertal age is approximately 4.5:1 - 5:1, and in pubertal age - 9:1 - 10:1.

Purpose - to show, on the basis of clinical observation, the difficulties of diagnosis and treatment in the debut of SLE in children.

Clinical case. A 16-year-old child was treated in the children’s department of Road Hospital No. 1 with a diagnosis of «acute glomerulonephritis with leading incomplete nephrotic syndrome, hematuria, hypertension, tubulointerstitial component; a period of extensive clinical manifestations, with impaired kidney function. Postazotemic anemia. Secondary myelosuppression». She was admitted with complaints of swelling of the legs and face, changes in urine tests (proteinuria 1.65 g/day, leukocyturia 45-50 in the field of vision, erythrocyturia 35-40-50 in the field of vision). She considered herself sick when she noticed swelling of the ankle joints. Three weeks before admission to the hospital, she suffered acute pharyngitis (negative antistreptolysin O titer). Allergological history is not burdensome. At 6 years old - chicken pox. The child was prescribed corticosteroid therapy (prednisolone 50 mg/day), against the background of which, in the 4^th week, the creatinine level normalized, but hypoproteinemia, elevated cholesterol, and proteinuria persisted, which indicated the lack of effect from CS therapy. Cytostatic therapy was started (leukeran - 8 mg/day (3 weeks); 4 mg/day - 3 weeks). Despite complex therapy, the child developed complete nephrotic syndrome, kidney failure six months later. During the examination of the patient, antinuclear antibodies with a titer >80 were detected, which gave rise to the calculation of additional criteria points (17 points) in accordance with the diagnostic criteria for SLE proposed by the European Antirheumatic League (EULAR) and the American College of Rheumatology (ACR), 2019. A diagnosis was made: SLE. Lupus nephritis, nephrotic syndrome, period of extensive clinical manifestations. Arterial hypertension; anemia I st. severity; azotemia; 2-3 degree of activity of the pathological process.

Conclusions. SLE in children is difficult to diagnose, especially in the absence of clinical polymorphism and systemic lesions. It is advisable to use the criteria proposed by EULAR and ACR, 2019, which are the most sensitive and specific in diagnosis. Treatment of patients with LN is individualized; the use of different treatment schemes depends on the severity of the pathological process. Early diagnosis of kidney damage in SLE using the criteria proposed by EULAR and ACR, 2019, and biopsy will contribute to the timely determination of the tactics and strategy of the child’s management, and improve the prognosis of Lupus nephritis.

The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies

No conflict of interests was declared by the authors.