Amin Kanani

Canadian hereditary angioedema guideline

Hereditary angioedema (HAE) is a disease which is associated with random and often unpredictable attacks of painful swelling typically affecting the extremities, bowel mucosa, genitals, face and upper airway. Attacks are associated with significant functional impairment, decreased Health Related Quality of Life, and mortality in the case of laryngeal attacks. Caring for patients with HAE can be challenging due to the complexity of this disease. The care of patients with HAE in Canada is neither optimal nor uniform across the country. It lags behind other countries where there are more organized models for HAE management, and where additional therapeutic options are licensed and available for use. The objective of this guideline is to provide graded recommendations for the management of patients in Canada with HAE. This includes the treatment of attacks, short-term prophylaxis, long-term prophylaxis, and recommendations for self-administration, individualized therapy, quality of life, and comprehensive care. It is anticipated that by providing this guideline to caregivers, policy makers, patients and their advocates, that there will be an improved understanding of the current recommendations regarding management of HAE and the factors that need to be considered when choosing therapies and treatment plans for individual patients. The primary target users of this guideline are healthcare providers who are managing patients with HAE. Other healthcare providers who may use this guideline are emergency physicians, gastroenterologists, dentists and otolaryngologists, who will encounter patients with HAE and need to be aware of this condition. Hospital administrators, insurers and policy makers may also find this guideline helpful.

Urticaria and angioedema

Urticaria (hives) is a common disorder that often presents with angioedema (swelling that occurs beneath the skin). It is generally classified as acute, chronic or physical. Second-generation, non-sedating H1-receptor antihistamines represent the mainstay of therapy for both acute and chronic urticaria. Angioedema can occur in the absence of urticaria, with angiotensin-converting enzyme (ACE) inhibitor-induced angioedema and idiopathic angioedema being the more common causes. Rarer causes are hereditary angioedema (HAE) or acquired angioedema (AAE). Although the angioedema associated with these disorders is often self-limited, laryngeal involvement can lead to fatal asphyxiation in some cases. The management of HAE and AAE involves both prophylactic strategies to prevent attacks of angioedema (i.e., trigger avoidance, attenuated androgens, tranexamic acid, and plasma-derived C1 inhibitor replacement therapy) as well as pharmacological interventions for the treatment of acute attacks (i.e., C1 inhibitor replacement therapy, ecallantide and icatibant). In this article, the authors review the causes, diagnosis and management of urticaria (with or without angioedema) as well as the work-up and management of isolated angioedema, which vary considerably from that of angioedema that occurs in the presence of urticaria.

Economic Assessment of Different Modalities of Immunoglobulin Replacement Therapy

The delivery (intravenous or subcutaneous), location (home or hospital), and other factors of immunoglobulin replacement therapy are examined for cost effectiveness. Cost-minimization studies from several countries are reviewed and analyzed. A Canadian cost-minimization study is performed. Although common themes emerge, there are cost differences between the various countries.

Insights and advances in chronic urticaria: a Canadian perspective

In the past few years there have been significant advances which have changed the face of chronic urticaria. In this review, we aim to update physicians about clinically relevant advances in the classification, diagnosis and management of chronic urticaria that have occurred in recent years. These include clarification of the terminology used to describe and classify urticaria. We also detail the development and validation of instruments to assess urticaria and understand the impairment on quality-of-life and the morbidity caused by this disease. Additionally, the approach to management of chronic urticaria now focuses on evidence-based use of non-impairing, non-sedating H1-antihistamines given initially in standard doses and if this is not effective, in up to 4-fold doses. For urticaria refractory to H1-antihistamines, omalizumab treatment has emerged as an effective, safe option.

Oral allergy syndrome and risk of food-related anaphylaxis: a cross-sectional survey analysis

Background Oral Allergy Syndrome (OAS) is an IgE-mediated allergic response to fresh fruits, nuts and vegetables caused by cross-reactivity between pollen allergens and structurally similar food proteins. Alder pollen is a prominent allergen in coastal British Columbia, present at high levels from FebruaryApril. We hypothesized that this exposure may lead to increased prevalence of Alder pollen allergy and OAS. We sought to determine our population-based prevalence, cross-reactivity patterns, and incidence of food-related anaphylaxis.

Canadian Physician Survey on the Medical Management of Hereditary Angioedema.

BACKGROUND Hereditary angioedema (HAE) is a rare disease that has significant morbidity and may be potentially fatal because of airway obstruction. OBJECTIVE To determine practice patterns in physicians treating HAE. METHODS A survey was designed to determine HAE practice patterns among Canadian physicians. These physicians were identified by sending the survey to members of 3 physician organizations (Canadian Hereditary Angioedema Network, Canadian Society of Clinical Immunology and Allergy, and Canadian Hematology Society). RESULTS Thirty-six physicians responded to the survey. Thirty-four physicians were included in the analysis. Most referrals to HAE-treating physicians were from family and emergency department physicians. The most common sites of swelling reported by patients to physicians were facial, peripheral, and abdominal. A mean of 53.9% of patients with type 1 and 2 HAE and 53.4% of patients with HAE with normal C1 esterase inhibitor were undergoing long-term prophylaxis. A mean of 41.9%, 19.4%, and 93.5% of respondents had some patients taking danazol, tranexamic acid, and C1-esterase inhibitor, respectively. Most physicians believed that severity and frequency of attacks were the most important determinants in deciding when to use prophylaxis. A mean of 88.2% of physicians used C1-esterase inhibitor to treat acute attacks and 79.4% used icatibant. All respondents were aware of HAE guidelines. CONCLUSION Physicians are using guidelines to support their practice and using agents suggested by guidelines with confidence. C1 inhibitor is being used widely for prophylaxis and treatment of acute attacks along with icatibant. However, certain special patient populations may require additional focus in future guidelines.

A pilot study of quality of life, mood, sleepiness and fatigue in patients with primary humoral immunodeficiency transitioning to subcutaneous immunoglobulin therapy

Background Immunoglobulin replacement therapy is standard of care for patients with primary humoral immunodeficiency [1]. Compared with intravenous immunoglobulin (IVIG), subcutaneous immunoglobulin (SCIG) offers comparable efficacy, lower costs and reduced systemic reactions [2,3]. However, little is known about effects on quality of life when patients transition from IVIG to SCIG. It was our objective to assess changes in quality of life, mood, sleepiness and fatigue in patients transitioning from IVIG to SCIG.