Treatment and immunisation

Treatment of Hyper IgE syndromes depends on preventing and management of infections to reduce the threat of overwhelming infection and limit damage to the lungs. It is really important to find out what particular bug is causing the infection so that can specific treatment can be given.

  • Long-term treatment with antibiotics and antifungal therapy to prevent and treat infections. These are especially important to treat infections caused by Staphylococcus aureus, Haemophilus influenzae, Streptococcus pneumoniae and Candida albicans. The development of resistance in the course of long-term therapy outweighs the risk of severe infections and lung damage when it is discontinued.
  • Skin abscesses may need to be cut and drained, but these can largely be prevented with continuous oral antibiotics.
  • Lung abscesses may require drainage or resection, but surgery is difficult in Hyper IgE patients because the remaining lung tissue often fails to expand to fill the chest cavity. Prolonged chest tube drainage and intensive intravenous antibiotic treatment is sometimes needed. Pulmonary surgery (surgery on the lungs) therefore requires specialist advice and should not be undertaken lightly, and ideally should be done at centres with experience with the disease.
  • Super infections caused by Pseudomonas aeruginosa, Aspergillus and other fungal species following the resolution of acute pneumonias. Treatment strategies include continuous treatment with antifungal drugs and/or, aerosolised antibiotics.
  • Immunoglobulin therapy may also help in the case of severe infections.
    Infections caused by viruses are treated using anti-viral medicines, for example acyclovir to treat chicken pox.
  • Good skin care and prompt treatment of skin infections is really important. Topical (meaning applied on the skin) antibacterial and oral antibiotics are often effective at preventing infection. Attention to skin care is essential when plaster casts are used to treat fractures or scoliosis.
  • Using moisturising creams helps treat the eczema and your doctor may give you on occasion steroid creams to use. Antiseptic treatments of the skin can help to reduce the bacterial burden in your skin without leading to emergence of antibiotic resistant bacteria. Your doctor may also recommend something to help relieve itching so that areas of eczema don’t become infected.

Role of bone marrow transplantation

There has been some success over the last few years in treating both the autosomal dominant and the autosomal recessive DOCK8 form of Hyper IgE using bone marrow transplantation (BMT).

Although the number of people treated by BMT remains small and the long-term outcomes are as yet unclear clinical outcomes were the disappearance of skin problems and severe infections, improvement of lung function and a drop in IgE levels. The recommendation is that BMT should be considered early before life-threatening complications develop.

Immunisation

There are no contra-indications to the use of killed or inactivated vaccines. The use of live vaccines is however best avoided because of the risk of vaccine-induced disease as seen in some patients with TYK2 deficiency.

This page was reviewed by the Medical Advisory Panel April 2013.