A UK national audit of hereditary and acquired angioedema.

Hereditary angioedema (HAE) and acquired angioedema (AAE) are rare life-threatening conditions caused by deficiency of C1 inhibitor (C1INH). Both are characterized by recurrent unpredictable episodes of mucosal swelling involving three main areas: the skin, gastrointestinal tract and larynx. Swelling in the gastrointestinal tract results in abdominal pain and vomiting, while swelling in the larynx may be fatal. There are limited UK data on these patients to help improve practice and understand more clearly the burden of disease. An audit tool was designed, informed by the published UK consensus document and clinical practice, and sent to clinicians involved in the care of HAE patients through a number of national organizations. Data sets on 376 patients were received from 14 centres in England, Scotland and Wales. There were 55 deaths from HAE in 33 families, emphasizing the potentially lethal nature of this disease. These data also show that there is a significant diagnostic delay of on average 10 years for type I HAE, 18 years for type II HAE and 5 years for AAE. For HAE the average annual frequency of swellings per patient affecting the periphery was eight, abdomen 5 and airway 0·5, with wide individual variation. The impact on quality of life was rated as moderate or severe by 37% of adult patients. The audit has helped to define the burden of disease in the UK and has aided planning new treatments for UK patients.

The treatment of atopic dermatitis with adjunctive high-dose intravenous immunoglobulin: a report of three patients and review of the literature.

There are few reports of the use of high-dose intravenous immunoglobulin (hdIVIg) in the treatment of atopic dermatitis (AD). We describe our experience using this therapy in three patients with severe AD, all of whom had steroid-related side-effects. These patients received either Alphaglobin(R) or Sandoglobulin(R) 2 g/kg monthly: all had improved skin scores, allowing reduction of their steroid dose. Total IgE fell in one of three patients. We discuss the side-effects of hdIVIg and their management, and detail the differences between the available immunoglobulin products available in the U.K. There are several proposed mechanisms of action of this therapy which may be operative, and those most important in AD are discussed. In view of the time and expense involved in the treatment of patients with hdIVIg, careful patient assessment is vital. We describe dose reduction strategies and methods for cost containment. In addition, one of the patients has embarked on IVIg home therapy training. This will be the first time this has been attempted for a dermatological indication. Training of this type may be available through an immunotherapy service such as exists for patients with primary immunodeficiencies.

Dermatological uses of high-dose intravenous immunoglobulin.

High-dose intravenous immunoglobulin (hdIVIg) is increasingly used to treat a range of inflammatory and autoimmune diseases. The current dermatological uses of hdIVIg include the treatment of dermatomyositis and the autoimmune bullous disorders, epidermolysis bullosa acquisita, pemphigoid, and pemphigus. Numerous immunomodulatory mechanisms for hdIVIg have been proposed, and they are discussed alongside treatment protocols and adverse effects. Increasing use of this therapy has helped to establish its excellent safety record, without the many adverse effects of steroids and other immunosuppressive agents. This safety record makes hdIVIg an attractive therapeutic option; however, in view of the time required to administer the infusions, the cost, and the urgent need for controlled trials of hdIVIg in patients with specific dermatological disorders such as pemphigus, patients must be carefully selected. Unfortunately, current dermatological uses of hdIVIg have been limited to either uncontrolled trials or anecdotal case reports, except for a single controlled trial of hdIVIg as adjunctive therapy in patients with dermatomyositis, which documented a significant benefit. Further trials in dermatomyositis should be established to confirm these data and to clarify the dose and frequency of therapy required for patients with dermatomyositis. When using hdIVIg, liaison between the dermatologist and the immunologist is helpful because it allows the use of both the nursing and the medical expertise of an existing immunotherapy unit. If appropriate, the patient may be entered into an hdIVIg home therapy training program, such as the one that exists for primary immunodeficiency and some neurologic indications, with clear benefits in quality of life and inpatient costs.