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Eosinophilic granulomatosis with polyangiitis EGPA , formerly known as allergic granulomatosis, [3] [4] is an extremely rare autoimmune condition that causes inflammation of small and medium-sized blood vessels vasculitis in persons with a history of airway allergic hypersensitivity atopy. It usually manifests in three stages. The second stage is characterized by abnormally high numbers of eosinophils hypereosinophilia , which causes tissue damage, most commonly to the lungs and the digestive tract. This condition is now called "eosinophilic granulomatosis with polyangiitis" to remove all eponyms from the vasculitides. To facilitate the transition, it was referred to as "eosinophilic granulomatosis with polyangiitis Churg—Strauss " for a period of time starting in Jacob Churg and Lotte Strauss who, in , first published about the syndrome using the term "allergic granulomatosis" to describe it.

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Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed. Eosinophilic granulomatosis with polyangiitis EGPA , previously known as Churg-Strauss syndrome, is a systemic vasculitis of small-to medium vessels, characterized by asthma, transient pulmonary infiltrates, and hypereosinophilia. Onset usually occurs between 15 and 70 years of age.

EGPA may involve multiple organ systems and patients often have an atopic background. The onset of EGPA is often associated with worsening of the asthma. Allergy and angiitis are the two hallmarks of the disease.

EGPA has been divided into three distinct phases, which may or may not be sequential. The prodromal phase is characterized by asthma with or without allergic rhinitis. The second phase is marked by peripheral blood eosinophilia and eosinophilic tissue infiltration producing a picture similar to those of simple pulmonary eosinophilia Loefflers syndrome , chronic eosinophilic pneumonia, or eosinophilic gastroenteritis see these terms. Skin involvement nodules, utricarial rash occurs in two thirds of patients.

Fever, flu like symptoms, and weight loss are also observed. Although the exact etiology of EGPA is unknown, the prominence of allergic features and the presence of immune complexes, heightened T-cell immunity, and altered humoral immunity indicate that the syndrome may represent an autoimmune disorder. Leukotriene receptor antagonists have been associated with the onset of EGPA, but a causative role in the induction of the disease has not yet been clearly demonstrated.

EGPA can be diagnosed if in addition to the signs of vasculitis, four of the following six features are present: asthma, eosinophilia, neuropathy, pulmonary infiltrates, paranasal sinus abnormalities and eosinophilic vasculitis. Tissue biopsies show eosinophilia, necrotizing vasculitis of small-to medium vessels and sometimes small necrotizing granulomatous inflammation. Differential diagnoses of EGPA include granulomatosis with polyangiitis formerly Wegener granulomatosis , hypereosinophilic syndrome, microscopic polyangiitis, cryoglobulinemia see these terms , drug reactions, bronchocentric granulomatosis, fungal and parasitic infections, and malignancy.

The treatment of patients with mild disease involves glucocorticoid GC monotherapy. Other immunosuppressive regimens, such as those using cyclophosphamide CP , are used for patients with an aggressive disease.

Treatment with interferon-alpha has been effective in patients refractory to GC plus CP. Infliximab is used for steroid dependent patients.

Treatment with the anti-interleukin-5 antibody mepolizumab might be effective in treating refractory EGPA and is under trial. Cardiac involvement is the leading cause of death related to EGPA, followed by cerebral hemorrhage and stroke. Despite treatment, neurological sequelae rarely resolve completely. Other search option s Alphabetical list. Suggest an update. Summary and related texts. Related genes.

Clinical signs. Check this box if you wish to receive a copy of your message. Disease definition Eosinophilic granulomatosis with polyangiitis EGPA , previously known as Churg-Strauss syndrome, is a systemic vasculitis of small-to medium vessels, characterized by asthma, transient pulmonary infiltrates, and hypereosinophilia. Clinical description Onset usually occurs between 15 and 70 years of age.

Etiology Although the exact etiology of EGPA is unknown, the prominence of allergic features and the presence of immune complexes, heightened T-cell immunity, and altered humoral immunity indicate that the syndrome may represent an autoimmune disorder. Diagnostic methods EGPA can be diagnosed if in addition to the signs of vasculitis, four of the following six features are present: asthma, eosinophilia, neuropathy, pulmonary infiltrates, paranasal sinus abnormalities and eosinophilic vasculitis.

Differential diagnosis Differential diagnoses of EGPA include granulomatosis with polyangiitis formerly Wegener granulomatosis , hypereosinophilic syndrome, microscopic polyangiitis, cryoglobulinemia see these terms , drug reactions, bronchocentric granulomatosis, fungal and parasitic infections, and malignancy. Management and treatment The treatment of patients with mild disease involves glucocorticoid GC monotherapy.

Prognosis Cardiac involvement is the leading cause of death related to EGPA, followed by cerebral hemorrhage and stroke. Additional information Further information on this disease Classification s 8 Gene s 0 Disability Clinical signs and symptoms Publications in PubMed Other website s Health care resources for this disease Expert centres Diagnostic tests 7 Patient organisations 34 Orphan designation s and orphan drug s 4.

Specialised Social Services Eurordis directory. The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.

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Eosinophilic granulomatosis with polyangiitis

Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed. Eosinophilic granulomatosis with polyangiitis EGPA , previously known as Churg-Strauss syndrome, is a systemic vasculitis of small-to medium vessels, characterized by asthma, transient pulmonary infiltrates, and hypereosinophilia.

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L'atteinte cardiaque, souvent silencieuse, domine le pronostic. Churg-Strauss syndrome is a vasculitis associated with asthma and eosinophilia. Respiratory involvement is marked by generally severe and often steroid-dependent late-onset asthma associated with allergic rhinitis and sometimes nasal polyposis and recurrent sinusitis. Asthma generally precedes the systemic vasculitis by a few years. General signs, eosinophilic gastroenteritis, peripheral multiplex neuropathy, cutaneous vasculitis, nephropathy, or arthromyalgia, predominate. Cardiac involvement is often silent but of severe prognosis. The chest X-ray usually shows irregularly delimited and sometimes labile infiltrates.

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