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Rajagopalan, Arul
- Hyper IgE Syndrome With Systemic Lupus Erythematosus as Complication
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1 Institute of Child Health, Chennai, IN
1 Institute of Child Health, Chennai, IN
Source
Journal of Clinical Pediatric Nephrology, Vol 1, No 1 (2013), Pagination:Abstract
Hyper IgE syndrome (HIES) is a rare syndrome of primary immunodeficiency associated with recurrent skin infections and pneumonia, eczema, eosinophilia and elevated levels of serum IgE. Patients with autosomal recessive form of HIES may be at increased risk of developing autoimmune diseases like systemic lupus erythematosus (SLE). A young girl presented with recurrent staphylococcal infections, eczema and oral candidiasis. She developed acute renal failure following skin infections. Evaluation suggested positive antinuclear antibody and renal biopsy showed diffuse proliferative glomerulonephritis with positive staining for immunoglobulins and complement on immunofluorescence, confirming the diagnosis of lupus nephritis (WHO class IV). We discuss the dilemma of treating recurrent infections during Hyper IgE syndrome along with SLE, an autoimmune disease.References
- Buckley Rh, Wray BB, Belmaker EZ. Extreme hyperimmunoglobulinemia E and undue susceptibility to infection. Pediatrics 1972; 49: 59-70.
- North J, Kotecha S, Houtman P, Whaley K. Systemic lupus erythematosus complicating hyper IgE syndrome. Br J Rheumatol 1997; 36: 297–298.
- Grimbacher B, Scaffer AA, Holland SM. Genetic linkage of hyper IgE syndrome to chromosome 4. Am J Hum Gen 1999; 65: 735-744.
- Ito R, Mori M, Katakura S, Kobayashi N, Naruto T, Osamura Y, et al. Selective insufficiency of IFN-g secretion in patients with hyper-IgE syndrome. Allergy 2003; 58: 329-336.
- Geha RS, Reinherz E, Leung D, McKee KT Jr, Schlossman S, Rosen FS. Deficiency of suppressor T cells in the hyperimmunoglobulin E syndrome. J Clin Invest 1981; 68: 783- 91.
- Engelhardt KR, Chatila TA. Large deletions and point mutations involving the dedicator of cytokinesis 8 (DOCK8) in the autosomal-recessive form of hyper-IgE syndrome. J Allergy Clin Immunol 2009; 124: 1289-302.
- Bong CN, Huang SC, Wang CL, Liu PM, Chen HH, Yang KD. Cyclosporin A therapy in a case with hyperimmunoglobulin E and nephrotic syndrome. J Microbiol Immunol Infect 2005; 1: 60-64.
- Borges Wg, Augustine NH, Hill HR. Defective IL-2 IFN Y pathway in patients with hyper IgE syndrome. J. Pediatr 2000; 136:176.
- Parks CG, Biagini RE, Cooper GS, Gilkeson GS, Dooley MA. Total serum IgE levels in systemic lupus erythematosus and associations with childhood onset allergies. Lupus 2010; 19: 1614