Abstract
There are estimated to be approximately 1500 people in the United Kingdom with C1 inhibitor (C1INH) deficiency. At BartsHealth National Health Service (NHS) Trust we manage 133 patients with this condition and we believe that this represents one of the largest cohorts in the United Kingdom. C1INH deficiency may be hereditary or acquired. It is characterized by unpredictable episodic swellings, which may affect any part of the body, but are potentially fatal if they involve the larynx and cause significant morbidity if they involve the viscera. The last few years have seen a revolution in the treatment options that are available for C1 inhibitor deficiency. However, this occurs at a time when there are increased spending restraints in the NHS and the commissioning structure is being overhauled. Integrated care pathways (ICP) are a tool for disseminating best practice, for facilitating clinical audit, enabling multi-disciplinary working and for reducing health-care costs. Here we present an ICP for managing C1 inhibitor deficiency.
Highlights
C1 inhibitor (C1INH) deficiency may be inherited [hereditary angioedema (HAE)] or acquired [acquired angioedema (AAE) with C1INH deficiency]
At BartsHealth National Health Service (NHS) trust we oversee the regular management of 124 patients with HAE and nine patients with AAE with C1INH deficiency – we believe this to be the largest cohort in the United Kingdom, which may be skewed to include some of the most severely affected patients
HAE and AAE with C1INH deficiency are chronic conditions characterized by an unpredictable tendency to develop swellings within the deeper layers of the skin or the mucus membranes [3,4]
Summary
C1 inhibitor (C1INH) deficiency may be inherited [hereditary angioedema (HAE)] or acquired [acquired angioedema (AAE) with C1INH deficiency]. Because patients may be required to travel long distances to tertiary centres, care may be shared with more local specialists who have an interest in the condition, such as gastroenterologists or general physicians. HAE and AAE with C1INH deficiency are chronic conditions characterized by an unpredictable tendency to develop swellings within the deeper layers of the skin or the mucus membranes [3,4]. These swellings may cause airway obstruction if they occur in the upper airway and severe, intractable pain associated with significant fluid shifts and hypotension if they occur in the abdominal viscera. In recent years new treatment options have become available for C1INH deficiency, which are based on an improved understanding of the pathophysiology of the condition and robust evidence of efficacy from licensing studies and clinical trials [6,7,8,9]
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
