An 11-year-old girl was admitted at our institute with anasarca, maculopapular rash over the face and hematuria (urine RBC plenty, urine protein–creatinine-ratio 1:7). Her blood pressure was normal for age, sex and height as per centile charts.2 Investigations revealed mildly deranged renal function tests (BUN 88mg/dl, creatinine 1.2mg/dl) along with low C3 levels (35mg/dl) and a positive ANA (titer >1:160), but complete blood count was unremarkable. She was also shown positive for anti-double-stranded DNA (dsDNA) (3+) and anti-nucleosome (3+) antibodies by immunofluorescence assay. Renal biopsy specimen confirmed changes of lupus nephritis stage II. Her lipid profile at presentation was serum triglyceride 172mg/dl and serum cholesterol 233mg/dl (HDL 44mg/dl, LDL 106mg/dl, VLDL 38mg/dl). She was started on oral prednisolone (2mg/kg/day), along with hydroxychloroquine (7mg/kg/day) and rosuvastatin (5mg/day) after obtaining due approval from her parents.

We repeated investigations after one week of oral prednisolone and rosuvastatin usage. Surprisingly, lipid profile levels were found to be markedly elevated (triglyceride 1786mg/dl, cholesterol 350mg/dl, LDL 275mg/dl, HDL 30mg/dl and VLDL 88mg/dl). She had also developed mild cushingoid features. Considering the possibility of an autoimmune phenomena, anti-lipoprotein-lipase (LPL) antibody (reactivity of anti-LPL immunoglobulin G was determined by enzyme-linked immunosorbent assay) estimation was done to find out the cause of hyperlipidemia. The report turned out to be negative. Hence, we were left with the possibility of steroid-induced hypertryglyceridemia. Prednisolone dose was rapidly tapered to 0.5mg/kg/day. On serial follow-up, triglyceride levels of this child gradually reduced with decreasing doses of prednisolone (Fig. 1). At 4 weeks, her triglyceride level was 356mg/dl and cholesterol was 222mg/dl (HDL 38mg/dl, LDL 146mg/dl, VLDL 48mg/dl). Mycophenolate mofetil (MMF) at a dose of 1000mg/m2/day was added to the schedule and prednisolone was continued at 0.5mg/kg/day.

Fig. 1.

Serum triglyceride levels over time.

(0.11MB).

A 10-year-old girl was admitted to our pediatric emergency with hypertensive encephalopathy. She had also been suffering from low-grade intermittent fever, joint pains and a non-pruritic rash over exposed parts of the body, which worsened on exposure to sunlight. Strikingly, routine blood samples drawn in the emergency room were lipemic. Samples sent for determining lipid-profile revealed marked hypertriglyceridemia (serum triglyceride 4696mg/dl) with hypercholesterolemia (serum cholesterol 514mg/dl, HDL 24mg/dl, LDL 357mg/dl, VLDL 133mg/dl). Complete blood counts revealed anemia (hemoglobin 8.7mg/dl) with thrombocytopenia (1.2lakh/cumm). Anti-nuclear (ANA, titer >1:160) and anti-ds DNA (3+) antibodies were positive. An MRI of the brain showed changes suggestive of acute disseminated encephalomyelilitis (ADEM). The child became fully conscious after successful control of hypertension over 48h at our pediatric intensive care unit. In view of SLE with ADEM, pulse methyl-prednisolone (30mg/kg/dose) was given for five days followed by oral prednisolone (2mg/kg/day).

Hydroxychloroquine (7mg/kg/day), antihypertensives namely labetalol (1mg/kg/dose) and amlodipine (10mg/day), along with hypolipidemic agent rosuvastatin (5mg/day) were also started along with fat-free diet. Due approval was obtained from the parents. Renal biopsy specimen confirmed changes of lupus nephritis stage II.

In this case, repeat investigations after one week of prednisolone and rosuvastatin therapy revealed a mild improvement in lipid profile levels. Serum triglyceride reduced to 3851mg/dl, cholesterol to 514mg/dl, HDL to 24mg/dl, LDL to 357mg/dl and VLDL to 133mg/dl. Another hypolipidemic agent, namely fenofibrate (45mg/day) was added. Plasmapheresis was done to rapidly reduce the levels of circulating triglyceride levels to prevent inflammation of the pancreas. Anti-LPL antibody turned out to be positive (samples were considered positive if optical density was ≥3 standard deviations above the mean optical density obtained for the 20 control samples from healthy individuals included in each assay) this time. The dose of oral prednisolone was hiked to 3mg/kg/day and MMF (800mg/m2/day) was also added. The child showed significant improvement in her lipid profile levels at the end of the second week (serum triglyceride 1435mg/dl, cholesterol 234mg/dl, HDL 54mg/dl, LDL 237mg/dl, VLDL 93mg/dl) (Fig. 1). However, there was worsening of hypertension along some cushingoid-features due to the high-dose of prednisolone. So, we tapered the dose of prednisolone and increased the dose of MMF to 1200mg/m2/day. At 4-week follow-up, the child was normotensive on two antihypertensives and her lipid profiles significantly improved. Her serum triglyceride was 457mg/dl and serum cholesterol was 184mg/dl (HDL 74mg/dl, LDL 137mg/dl, VLDL 83mg/dl) with decrease in the titer of anti-LPL antibody. Prednisolone was continued at a maintenance dose of 0.8mg/kg/day.

The authors declare that the procedures followed were in accordance with the regulations of the relevant clinical research ethics committee and with those of the Code of Ethics of the World Medical Association (Declaration of Helsinki).

The authors declare that they have followed the protocols of their work center on the publication of patient data.

The authors have obtained the written informed consent of the patients or subjects mentioned in the article. The corresponding author is in possession of this document.