Clinical Brief
Familial Systemic Lupus Erythematosus With Hypercalcemia Utkarsh Kohli, Rakesh Lodha and Arvind Bagga Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
ABSTRACT An 8-yr-old girl with familial systemic lupus erythematosus and several severe manifestations, including persistent thrombocytopenia, rapidly progressive renal failure and hepatic failure is described. The course was complicated by the occurrence of hypercalcemia, hypophosphatemia and elevated levels of parathormone, an association not previously reported in children. [Indian J Pediatr 2008; 75 (8) : 855-857] E-mail:
[email protected] Key words : Hyperparathyroidism, Hypercalcemia; Lupus nephritis; Thrombocytopenia
Systemic lupus erythematosus (SLE) is a multi-system disorder with variable presentation. While involvement of the skin, joints and kidneys is the chief cause of morbidity in children, involvement of other organ systems may occur. We report an 8-yr-old girl with SLE, severe hepatitis, nephritis, sustained hypercalcemia, hypophosphatemia and elevated levels of parathormone (PTH). The association of hypercalcemia with SLE is rare and hitherto limited to case reports involving adult subjects.1-5
Coomb's test was negative and prothrombin time was normal (14 seconds). The blood urea level was 77 mg/dL, creatinine 1 mg/dL, calcium 8.3 mg/dL, phosphate 3.7 mg/dL, bilirubin 15 (conjugated 10.5) mg/dL, albumin 2.5 g/dL, oxaloacetate transaminase 360 U/L, pyruvate transaminase 120 U/L, alkaline phosphatase 270 U/L, γglutamyltranspeptidase 40 U/L (normal 1-90), ammonia 55 mmol/L (normal 14-50) and ceruloplasmin 30 mg/dL (normal 20-45). Blood and urine cultures were sterile and C-reactive protein was negative. Urine analysis showed 3+ proteinuria, 10-15 red cells/hpf and red cell and granular casts.
CASE REPORT
Serum antinuclear antibody was positive (titer 1:160), C3 was 35 mg/dL (normal 77-190) and anti-double stranded deoxyribonucleic-acid antibody 85 IU/mL (normal 0-55). Antineutrophil-cytoplasmic antibody, antiliver kidney microsomal antibody and anticardiolipin antibodies were negative. Serologies for hepatitis A, B, C and E viruses were negative. Echocardiogram showed left ventricular ejection fraction of 60% and mild pericardial effusion. MRI of the brain was normal. Kidney biopsy showed diffuse proliferative glomerulonephritis with high activity index; immunofluorescence showed capillary wall deposits of IgG, IgM, IgA, C3 and C4.
This 8-yr-old girl presented with complaints of headache, intermittent fever, hematuria and facial erythema for 2 months, and jaundice for 10 days. Her mother, diagnosed to have SLE with class IV nephritis and thrombocytopenia 2-yr back, was receiving treatment with prednisolone and azathioprine. She was the only child of a nonconsanguineous marriage. On examination, she had icterus, petechiae, anasarca and blood pressure of 100/80 mm Hg; the liver was palpable 6 cm below the costal margin (span 13 cm). She was irritable and drowsy; deep tendon reflexes were exaggerated and plantar reflexes were extensor. The hemoglobin level was 7.2 g/dL, leukocytes 3200/ mm 3 , platelets 70,000/mm 3 and reticulocytes
