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Job's Syndrome (Hyper-IgE Syndrome)

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Primary immunodeficiency syndrome characterized by recurrent infections and hyperimmunoglobulinemia E. Most cases are sporadic. Of the rare familial forms, the dominantly inherited subtype has additional connective tissue, dental and skeletal involvement that the recessive type does not share.

Also Known As:

Hyper-IgE Syndrome; Job Syndrome; Syndrome, Hyper-IgE; HIES, Autosomal Dominant; Hyper-IgE Syndrome, Autosomal Dominant; Hyper-Immunoglobulin E Syndrome, Autosomal Dominant; Hyperimmunoglobulin E, Recurrent Infection Syndrome; Hyperimmunoglobulinemia E Syndrome; Autosomal Dominant HIES; Autosomal Dominant HIESs; Autosomal Recessive HIES; Autosomal Recessive HIESs; HIESs, Autosomal Dominant; HIESs, Autosomal Recessive; Hyper IgE Syndrome; Hyper IgE Syndrome, Autosomal Dominant; Hyper IgE Syndrome, Autosomal Recessive; Hyper Immunoglobulin E Syndrome, Autosomal Dominant; Hyper Immunoglobulin E Syndrome, Autosomal Recessive; Hyper-IgE Syndromes; Hyperimmunoglobulin E Recurrent Infection Syndrome; Hyperimmunoglobulinemia E Syndromes; Jobs Syndrome; Syndrome, Hyperimmunoglobulinemia E; Syndrome, Job's; Syndromes, Hyperimmunoglobulinemia E; HIES, Autosomal Recessive; Hyper-IgE Syndrome, Autosomal Recessive; Hyper-Immunoglobulin E Syndrome, Autosomal Recessive; Hyperimmunoglobulin E-Recurrent Infection Syndrome

Networked: 295 relevant articles (11 outcomes, 17 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Infection
2.	Pneumonia (Pneumonitis)
3.	Atopic Dermatitis (Atopic Eczema)
4.	Abscess
5.	Hypergammaglobulinemia (Hyperimmunoglobulinemia)

Experts

1.	Freeman, Alexandra F: 26 articles (11/2015 - 11/2005)
2.	Holland, Steven M: 25 articles (11/2015 - 12/2004)
3.	Grimbacher, Bodo: 9 articles (12/2014 - 10/2007)
4.	Puck, Jennifer M: 7 articles (06/2013 - 05/2007)
5.	Minegishi, Yoshiyuki: 6 articles (09/2015 - 08/2007)
6.	Davis, Joie: 6 articles (07/2013 - 05/2007)
7.	Hsu, Amy P: 6 articles (07/2013 - 10/2007)
8.	Renner, Ellen D: 5 articles (07/2013 - 07/2008)
9.	Ochs, Hans D: 5 articles (02/2012 - 07/2008)
10.	Woellner, Cristina: 5 articles (10/2011 - 10/2007)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Job's Syndrome:

1.	Immunoglobulin E (IgE)IBA 12/01/2011 - "The discovery that loss-of-function mutations in the gene DOCK8 are responsible for most forms of autosomal recessive hyper-IgE syndrome and some forms of combined immunodeficiency without elevated serum IgE has led to studies into the immunopathogenesis of this disease. " 11/01/1994 - "Our objective in this study was to compare the regulation of in vitro IgE synthesis in this patient with results reported for in vitro IgE production by patients with the hyper-IgE syndrome. " 05/01/1983 - "The aim of this study was to investigate the PMN chemotactic function in 18 children affected with AD and high levels of serum IgE, and in 4 children with Hyper IgE Syndrome (HIES). " 11/07/2015 - "Optimized conditions were then used to screen 113 serum samples simultaneously for hyper IgE syndrome (hIgE), a rare primary immunodeficiency characterized by elevated levels of IgE. " 01/01/2015 - "Laboratory workup for immune deficiency showed high eosinophil count and high level of immunoglobulin-E, due to some diagnostic criteria (NIH sores: 41 in 9-year-olds), he was suggestive of hyper IgE syndrome. "
2.	Anti-Bacterial Agents (Antibiotics)IBA 01/01/1999 - "A diagnosis of hyperimmunoglobulin E-recurrent infection syndrome (HIE) was made and she was successfully treated with surgical drainage and antibiotics. " 04/01/2004 - "Traditional prophylactic antistaphylococcal antibiotics, in combination with Neocate formula, were effective in treating the early skin manifestations of hyper-IgE syndrome and FTT in this infant. " 07/01/1990 - "We present three pediatric patients with life threatening infections in whom the fluoroquinolones were used when other antibiotics failed: A seven-year-old boy with meningitis due to multiresistant Acinetobacter calcoaceticus, a three-year-old boy with Job's syndrome with line sepsis due to Staphylococcus epidermidis and a four month-old boy with agammaglobulinemia with mixed infection due to Escherichia coli, Pseudomonas aeruginosa, and Acinetobacter spp. All three children were cured of their infections."
3.	Cyclosporine (Ciclosporin)FDA LinkGeneric 04/01/1997 - "A small dose of cyclosporin A (3 to 5 mg/kg/d) is beneficial in patients with hyperimmunoglobulinemia E syndrome. " 09/01/1989 - "Renal disease, symptoms of hyper IgE syndrome and level of serum IgE were improved by a long-term cyclosporine treatment (3/5 mg/kg/day) with a follow-up of 4 years. "
4.	Intravenous Immunoglobulins (IVIG)FDA Link 12/01/1999 - "We recommend that IVIG be considered as one of the first choices in the treatment of hyper IgE syndrome." 05/01/2000 - "Intravenous immunoglobulins represent a useful treatment for acute pneumonia in Job's syndrome." 08/01/1998 - "To determine if high-dose IVIG would be of benefit to patients with severe atopic dermatitis and/or hyper-IgE syndrome using serial clinical, pulmonary, and laboratory studies for evaluation. " 05/01/2000 - "To describe the effects of IVIG therapy in a 37-year-old woman with hyper IgE syndrome and pneumonia. " 08/01/1998 - "This was an open label study in which we gave patients with hyper-IgE syndrome (n = 1) or severe atopic dermatitis (n = 9) IVIG as a 10% solution (Venoglobulin I-Alpha Therapeutic Corporation, Los Angeles, CA) at a dose of 2 g/kg every 30 days for seven infusions. "
5.	anti-IgEIBA 05/01/2006 - "Clinical pearls and pitfalls include the following: (1) deep-seeded Staphylococcus aureus infections occur rarely in AD and should raise the possibility of immunodeficiency syndromes such as hyper-IgE syndrome (HIES); (2) HIES is characterized by a clinical triad consisting of elevated serum IgE levels, recurrent staphylococcal skin abscesses, and pneumonia with pneumatocele formation; (3) although serum IgE levels in AD have been noted to be as high as 10,000 IU/mL, severe cases of AD such as that presented here can exceed this range; (4) the efficacy of anti-IgE therapy in AD or HIES is unknown and may be limited by dosing requirements." 11/01/1986 - "Our data indicate that complexes made of IgE and IgG anti-IgE are present mainly in patients with chronic allergic symptoms and most frequent in cases of hyper-IgE syndrome." 11/01/1986 - "IgG anti-IgE in circulating immune complexes in the hyper-IgE syndrome."
6.	FluoroquinolonesIBA 07/01/1990 - "We present three pediatric patients with life threatening infections in whom the fluoroquinolones were used when other antibiotics failed: A seven-year-old boy with meningitis due to multiresistant Acinetobacter calcoaceticus, a three-year-old boy with Job's syndrome with line sepsis due to Staphylococcus epidermidis and a four month-old boy with agammaglobulinemia with mixed infection due to Escherichia coli, Pseudomonas aeruginosa, and Acinetobacter spp. All three children were cured of their infections."
7.	Fluconazole (Zonal)FDA LinkGeneric 11/01/1996 - "Based on our experience, we think that fluconazole may be useful in the treatment of onychomycosis in patients with hyper-IgE syndrome as well as in those with other primary immunodeficiency disorders."
8.	ExotoxinsIBA 05/01/2007 - "Since significant increase of serum IgE suggested hyper-IgE syndrome, IgE antibody specific to Staphylococcal enterotoxin A and B, and the exotoxins of Staphylococcus aureus were measured with positive results. "
9.	NeocateIBA 04/01/2004 - "Traditional prophylactic antistaphylococcal antibiotics, in combination with Neocate formula, were effective in treating the early skin manifestations of hyper-IgE syndrome and FTT in this infant. "
10.	Staphylococcal enterotoxin AIBA 05/01/2007 - "Since significant increase of serum IgE suggested hyper-IgE syndrome, IgE antibody specific to Staphylococcal enterotoxin A and B, and the exotoxins of Staphylococcus aureus were measured with positive results. "

Therapies and Procedures

1.	Plasmapheresis 01/01/1988 - "Remission of hyper-IgE syndrome treated with plasmapheresis and cytotoxic immunosuppression." 01/01/1988 - "A patient with a hyper-IgE syndrome was treated with 60 plasmaphereses over a period of 2 years in conjunction with cytotoxic immunosuppressive drug therapy. " 04/01/1988 - "A 19-year-old woman with hyperimmunoglobulinemia E (the hyper-IgE syndrome [HIE]) and a 62-year-old man with atopic dermatitis experienced dramatic improvement in their chronic ocular symptoms and signs of atopic keratoconjunctivitis after plasmapheresis was instituted. "
2.	Drug Therapy (Chemotherapy) 01/01/1988 - "A patient with a hyper-IgE syndrome was treated with 60 plasmaphereses over a period of 2 years in conjunction with cytotoxic immunosuppressive drug therapy. " 12/01/2004 - "With appropriate chemotherapy and hematological support, lymphoma associated with Job's syndrome can achieve complete remission."
3.	Transplants (Transplant) 07/01/2015 - "Successful haploidentical donor hematopoietic stem cell transplant and restoration of STAT3 function in an adolescent with autosomal dominant hyper-IgE syndrome." 01/01/2015 - "WU polyomavirus in respiratory epithelial cells from lung transplant patient with Job syndrome." 05/26/2001 - "[Loss of an eye due to hyper-IgE syndrome after a corneal transplant]." 01/01/2014 - "We describe clinical and laboratory features and response to therapy in 7 patients, three of them with HIV infection and four non-HIV-three organ transplant recipients and one with hyper-IgE syndrome-in Valencia, Spain, in a ten years period. "
4.	Hematopoietic Stem Cell Transplantation 11/01/2010 - "Successful long-term correction of autosomal recessive hyper-IgE syndrome due to DOCK8 deficiency by hematopoietic stem cell transplantation." 08/01/2010 - "Successful long-term immunologic reconstitution by allogeneic hematopoietic stem cell transplantation cures patients with autosomal dominant hyper-IgE syndrome."
5.	Cell Transplantation 04/01/2011 - "Curative treatment of autosomal-recessive hyper-IgE syndrome by hematopoietic cell transplantation." 12/01/2010 - "Successful engraftment of donor marrow after allogeneic hematopoietic cell transplantation in autosomal-recessive hyper-IgE syndrome caused by dedicator of cytokinesis 8 deficiency."