PEDIATRIC ONSET SYSTEMIC LUPUS ERYTHEMATOSUS
Introduction: Systemic lupus erythematosus (SLE) is connective tissue disease, which can occur in children. It is said that in 15 ? 17% of all patients with SLE, the disease onset is before the age of 16 years. Although it can affect both sexes, it is five to six times commoner in girls. Africans & Asians are said to have a more aggressive disease than the white population.
Clinical manifestations: The common clinical manifestations are fever, hair loss, oral ulcers and rash on the face. The fever is often high grade and persistent. It may not have a diurnal variation. It is often associated with significant hair fall. This hair fall is spontaneous and is not related to combing. The oral ulcers are recalcitrant and characteristically occur in the hard palate. Rash on the cheeks is typical. This rash worsens on exposure to sunlight. Arthritis can be troublesome.
Although most of the clinical features listed above are important, involvement of the kidney, nervous system, hematological system and cardiac system all carry serious connotations.
When kidney involvement occurs, expert care is needed. Renal involvement often presents with edema and high blood pressure. Examination of the urine and blood confirm that there is inflammation of the kidneys. Ultrasound examination in the early stages reveals enlarged kidneys. Since there are various types of kidney involvement there often is a need to do a kidney biopsy before proceeding with aggressive therapy. Apart from treatment with appropriate drugs for the disease, optimal control of blood pressure and lipid level is essential.
Nervous system involvement can present with confusion, meningitis, encephalitis, convulsion or stroke. Such children are very sick and need urgent admission. The child may need various investigations including scans of the brain and spinal CSF analysis.
Although anemia is a common manifestation of SLE, severe and rapidly progressive anemia, low white cell count and low platelets all point towards aggressive disease and need aggressive therapy. When these events occur again the child needs investigations, which may include a bone marrow examination.
Inflammation of the different layers of the heart can occur. At times fluid collection in the outer layer called pericardial effusion can occur. Rarely increase in pressure of the circulation in the lungs leads to a condition called pulmonary hypertension. When these complications occur, the child needs assessment by an experienced cardiologist.
Diagnosis: The diagnosis is made in the presence of at least 2 appropriate clinical features and strongly positive antinuclear antibody test (ANA) and a positive dsDNA test. The point to note is that ANA should be done by immunofluorescence method and a titre of more than 1: 80 is significant.
Differential diagnosis: Infections are common in our setting and specific infections like tuberculosis should be ruled out. In children, malignancies of the blood and lymph nodes have to be carefully eliminated.
Patient and parent education: This forms the most vital part of management. It should be made clear that this disorder is chronic and needs careful and continued follow-up. As yet there is no cure but like diabetes or hypertension, good control is possible. Worry regarding the side effects of the medications is inevitable. However, the consequence of untreated disease could be very serious, and hence the need for regular medications. Every effort will be made by the treating physician to control the disease and at the same time minimize the side effects.
As the skin lesions and at times the disease can worsen with sun exposure, it should be avoided especially when the disease is active.
Medications: For mild to moderate disease, painkillers, chloroquine and small doses of steroid may be sufficient. Those with serious involvement of the kidneys, nervous system, hematological system or cardiovascular system need appropriate aggressive therapy. This often includes high doses of steroids along with drugs like cyclophosphamide.
Regular tests: It is important to realize that since the disease is chronic, regular tests may be needed to monitor both the disease and the drug side effects. Urine examination should be done on monthly basis to detect early renal involvement. CBC & ESR can be done on 2-3 monthly basis.
Physical growth: Both the disease and the drugs like steroids can lead to stunted growth. This fact should be kept in mind and regular checks of the child?s height and comparison with the average normal for that particular age must be made. Every effort must be made to keep the steroid dose at a minimum. Calcium and Vitamin D supplements are to be taken with steroid therapy.
Absence from school: As the disease is often characterized by remission and exacerbations, the child may lose days in school. The parents and the treating rheumatologist should make it a point to inform and counsel the teachers regarding this aspect of the disease.
Emotional problems: A child afflicted by a chronic disease often has complex emotional problems. These may need the assessment by an expert in child psychology.
Last updated on 07-09-2002