Eosinophil-associated diseases (EADs) represent a range of heterogeneous conditions in which eosinophils are believed to play a critical pathological role |
Patients with EADs face substantial disease burdens, including debilitating symptoms and associated disease flares, and an arduous path to diagnosis and treatment, often involving repeated visits to multiple clinical subspecialties |
This charter outlines key rights that patients should expect from the management of their condition(s) and describes an ambitious action plan to improve health outcomes for patients with EADs |
Patients and their caretakers, multiple healthcare professionals, and the broader public need greater awareness and education about EADs |
All patients with EADs deserve a timely and accurate diagnosis, access to an appropriate multidisciplinary team, and access to appropriate treatments |
Digital Features
Introduction
Disease information | Estimated prevalence | Guidelines |
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Atopic dermatitis (AD) | ||
Also known as eczema, AD is a common, heterogeneous, and recurrent chronic inflammatory skin disease, which is associated with other known allergic disorders, including asthma. Eosinophils play an active role in AD pathology, with the presence of eosinophil granule proteins in skin lesions. AD is characterised by acute flare-ups of pruritic lesions over dry skin and causes discomfort, which includes intense itching, sleep impairment, and impact on health-related quality of life (including concerns about aesthetics/physical appearance) | Prevalence estimates vary between 3.2–10.2% in the US [50] | |
Bullous pemphigoid (BP) | ||
BP is a rare autoimmune, chronic skin disorder characterised by blistering and/or urticarial lesions (hives) and itching, where patients often show high levels of eosinophils in peripheral blood and/or tissue lesions. It is associated with high morbidity and mortality. It typically affects elderly patients with an average patient age of 80 years old | 7.63 per 100,000 patient-years in England [51] | |
Chronic obstructive pulmonary disease (COPD) | ||
COPD is a progressive disease that can cause obstruction of airflow in the lungs, resulting in debilitating bouts of breathlessness. The COPD subset with eosinophil-driven inflammation phenotypes is characterised by elevated eosinophil counts in the blood or airways and accounts for 20% to 40% of patients with stable COPD. Improving lung function, reducing exacerbations, and managing daily symptoms, such as breathlessness, are important treatment goals in the management of COPD | 384 million people worldwide (20%–40%) [52] | |
Chronic spontaneous urticaria (CSU) | ||
CSU is a dermatological condition in which hives, welts, or subcutaneous swellings occur lasting for > 6 weeks. Eosinophilic infiltration is common in patients with CSU, where signalling between activated eosinophils and mast cells in the skin leads to chronic inflammation. These hives—which range in size from just a few millimetres to several centimetres—are itchy and can occur anywhere on the body, including the face, extremities, chest, or back. CSU has a significant burden on a patient’s health-related quality of life, including sleep impairment and overall functioning | Global prevalence of 0.1–1.4% [53] | |
Eosinophilic asthma (EA) | ||
EA is the most common type of asthma and is associated with high levels of eosinophils in the blood and airways, leading to daily asthma symptoms and the risk of life-threatening asthma attacks | ||
Eosinophilic bronchitis (EB) | ||
Eosinophilic bronchitis (EB) is characterised by eosinophilic airway inflammation (similar to the airway inflammation in asthma) without associated airway hyperresponsiveness or variable airflow obstruction | ||
Eosinophilic colitis | ||
Eosinophilic colitis is a rare disease in the group of EGIDs. It is characterised by the presence of a dense eosinophilic infiltrate into the colonic wall; more often, patients present with abdominal pain or diarrhoea. Diagnosing the condition is challenging due to the lack of diagnostic guidelines and the use of a diagnostic process of exclusion where other causes of colonic eosinophilia are excluded first | Estimated prevalence of 3.3 per 100,000 in the US [36] | |
Eosinophilic cystitis (EC) | ||
EC is a rare inflammatory bladder condition caused by the accumulation of eosinophils in the bladder. In patients with EC, eosinophilic inflammation of the bladder results in urinary frequency, painful urination, blood in the urine, and abdominal or pelvic pain | ||
Eosinophilic duodenitis (EoD) | ||
Eosinophilic duodenitis is a chronic, inflammatory disease characterised by high levels of mast cells and eosinophils in the stomach and/or the duodenum, leading to symptoms that include chronic abdominal pain, nausea, bloating, loss of appetite, early satiety, abdominal cramping, vomiting, and diarrhoea | The estimated prevalence of EG/EoD is approximately 15 per 100,000 people, although this is likely an underestimate as misdiagnosis is common for these conditions [19] | |
Eosinophilic esophagitis (EoE) | ||
A rare chronic, local, immune-mediated oesophageal disease, characterised clinically by symptoms related to oesophageal dysfunction associated with marked inflammation, including eosinophils. Dysregulated immune responses lead to recruitment and activation of eosinophils in the oesophagus, which results in injury, fibrosis, and dysfunction that can potentially lead to dysphagia and food impaction, chronic pain, difficulty swallowing, poor growth in children, malnutrition, and weight loss. The EoE diagnosis can be challenging to make in children, given that it requires invasive endoscopic assessment and biopsy with high-powered imaging | ||
Eosinophilic fasciitis | ||
Eosinophilic fasciitis is a rare condition characterised by inflammation of the band of fibrous tissue beneath the skin and surrounding the muscles (fascia), usually affecting the arms and legs. The inflammation is caused by the abnormal accumulation of certain white blood cells, including eosinophils, in the fascia. The accumulation of eosinophils eventually causes skin swelling and progressive thickening and hardening | ||
Eosinophilic dermatitis | ||
A broad, heterogeneous group of dermatological diseases that are characterised by eosinophil infiltration and/or degranulation in skin lesions with or without associated blood eosinophilia. Conditions classically described as eosinophilic dermatitis include eosinophilic cellulitis, eosinophilic fasciitis, eosinophilic folliculitis, cutaneous eosinophilic vasculitis, and granuloma faciale | ||
Eosinophilic gastritis (EG) | ||
Eosinophilic gastritis is a rare digestive condition characterised by eosinophilic infiltration of the stomach, which may lead to epigastric pain, nausea, and vomiting or, less frequently, signs of upper gastrointestinal bleeding. It can be an isolated condition or, more commonly, be part of eosinophilic gastroenteritis | Estimated prevalence of 6.3 per 100,000 in the US [36] | |
Eosinophilic gastroenteritis (EGE; also known as eosinophilic enteritis) | ||
Eosinophilic gastroenteritis is a rare digestive disease characterised by eosinophilic infiltration into segments of the intestinal tract. Symptoms include abdominal pain, diarrhoea, nausea, vomiting, bloating, or ascites. The aetiology remains unknown, but there is some evidence to support the role of allergens in the pathogenesis of this disorder | Estimated prevalence is 8.4 per 100,000 in the US, although this is likely an underestimate as misdiagnosis is common for these conditions [36] | |
Eosinophilic granulomatosis with polyangiitis (EGPA) | ||
EGPA is a rare, chronic autoimmune multisystem disease that is associated with high levels of eosinophilic inflammation of various tissues, as well as small- to medium-sized blood vessels, resulting in damage to multiple organs, including lungs, skin, heart, gastrointestinal tract, and nerves. Diagnosing the condition is challenging given its rarity and the varied clinical manifestations of the disease, which can be difficult to distinguish from severe eosinophilic asthma, hypereosinophilic syndrome (HES) with asthma, and chronic eosinophilic pneumonia | The estimated global and European prevalences are 15.27 and 12.13 cases per million individuals, respectively [14] | |
Eosinophilic pneumonia | ||
Eosinophilic pneumonia comprises a group of lung diseases in which eosinophils appear in increased numbers in the lungs and usually in the bloodstream, with symptoms that range from mild to life-threatening, including shortness of breath, fever, chest pain, cough, wheeze, and decreased levels of oxygen in the blood. In acute eosinophilic pneumonia (AEP), the number of blood eosinophils may be normal, while chronic eosinophilic pneumonia (CEP) generally shows high numbers | AEP: 9.1 per 100,000 patient-years; CEP 0.23 per 100,000 population [56] | |
Hypereosinophilic syndrome (HES) | ||
A heterogenous group of rare disorders characterised by high numbers of eosinophils in blood and tissues, which can cause progressive damage to any organ and, if left untreated, be fatal. HES most commonly impacts the skin, heart, lungs, gastrointestinal tract, and nervous system | 0.15–0.89 per 100,000 persons in the UK [57] | |
Nasal polyposis (NP) | ||
Nasal polyps are growths on the lining of the sinuses and nasal passages. In patients with nasal polyps, elevated levels of eosinophils accumulate in the upper respiratory tract, which can lead to breathing problems, frequent sinus infections, or loss of the ability to smell. Nasal polyps are also associated with EA | Estimated prevalence of 10.9% in Europe and 11.9% in the US [58] |