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Eosinophilic granulomatosis with polyangiitis
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Eosinophilic granulomatosis with polyangiitis

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Contributors: Edward Li PhD, Trilochan Hiremath MD, Paritosh Prasad MD, Susan Burgin MD
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Synopsis

Eosinophilic granulomatosis with polyangiitis (EGPA), also known as allergic granulomatosis angiitis and, formerly, Churg-Strauss syndrome, is a disorder affecting multiple body systems that is characterized by asthma, chronic rhinosinusitis, peripheral eosinophilia, and systemic small to medium vessel vasculitis.

EGPA has an estimated incidence of 0.1-3 cases per million and a prevalence of 11-18 cases per million worldwide. It does not have a clear sex predominance. The mean age at diagnosis is 48 years. Disease onset in childhood or adolescence is possible but extremely rare. Those affected in younger age groups tend to have a more aggressive disease course with prominent pulmonary and cardiovascular complications.

The clinical course of EGPA typically develops in 3 sequential but overlapping phases: a prodromal atopic phase, an eosinophilic phase, and finally a vasculitic phase.
  • The prodromal phase frequently occurs in the second to third decade of life and may last months to years, with asthma as the main manifestation (96%-100% of patients). The asthma of EGPA is typically difficult to control with conventional treatments. Also common in this phase are allergic rhinosinusitis and nasal polyposis.
  • The eosinophilic phase is marked by an elevated eosinophil count and eosinophil infiltration into multiple organs including the heart, lungs, and gastrointestinal tract. 
  • The vasculitic phase, typically occurring a decade or more after the onset of the prodromal phase, is characterized by a small to medium vessel necrotizing vasculitis with granuloma formation.
    Complications arising from eosinophil infiltration into multiple organs include pleural effusions and alveolar hemorrhage (lungs), pericarditis and myocardial conduction defects (heart), eosinophilic gastroenteritis and abdominal pain (gastrointestinal), myalgias and arthralgias (musculoskeletal), and a wide spectrum of renal complications ranging from microscopic proteinuria and hematuria to necrotizing glomerulonephritis. Cardiac involvement remains the primary cause of death in EGPA.

    Cutaneous findings are common, especially during the vasculitic phase of EGPA, and can be polymorphic, including erythema, urticaria, purpura, tender nodules, and necrosis.

    The exact mechanism of pathogenesis for EGPA is currently unknown. However, it is generally thought to be due to a dysregulation of immune function. Research indicates that eosinophil infiltration and antineutrophil cytoplasmic antibody (ANCA)-induced endothelial damage may be involved in the underlying disease mechanism. About half of patients with EGPA have positive ANCA. Recently, it has been suggested that 2 distinct phenotypes of EGPA are present and depend on the presence or absence of ANCA. Several medications (eg, leukotriene modifying agents, omalizumab) have also been found to be associated with the apparent onset of EGPA; however, causal relationships have not been established, and it is likely that these medications only served to unmask the underlying disease.

    Codes

    ICD10CM:
    M30.1 – Polyarteritis with lung involvement [Churg-Strauss]

    SNOMEDCT:
    82275008 – Churg-Strauss syndrome

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    Diagnostic Pearls

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    Differential Diagnosis & Pitfalls

    • Chronic eosinophilic pneumonia – This condition should be first considered because it is more common than EGPA. The degree of eosinophilia is not as great as EGPA, and necrotizing vasculitis and granulomas are not present.
    • Hypereosinophilic syndrome – This condition has many features that overlap with EGPA. However, the key findings that distinguish it from EGPA are the absence of asthma, vasculitis, and granulomas.
    • Bronchocentric granulomatosis – A rare condition associated with allergic bronchopulmonary aspergillosis (ABPA). Patients may have asthma, necrotizing granulomas, blood eosinophilia, and eosinophilic pneumonia. However, the granulomatous inflammation is limited to bronchocentric locations with mucoid impaction of bronchi. Necrotizing vasculitis is almost always absent.
    • Granulomatosis with polyangiitis (GPA; formerly Wegener granulomatosis) – While rare cases can present with eosinophilia, patients do not have asthma or the characteristic lung findings of EGPA.
    Other conditions to rule out that present with tissue eosinophilia and granulomatous inflammation include Löffler syndrome, endemic fungal pneumonias, and parasitic lung infections.

    Best Tests

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    Management Pearls

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    Therapy

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    Drug Reaction Data

    Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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    References

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    Last Reviewed: 07/25/2019
    Last Updated: 07/26/2019
    Copyright © 2019 VisualDx®. All rights reserved.
    Eosinophilic granulomatosis with polyangiitis
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    Eosinophilic granulomatosis with polyangiitis (Allergic Stage) : Cough, Headache, Nasal obstruction, Nasal polyps, Rhinorrhea, Dyspnea
    Clinical image of Eosinophilic granulomatosis with polyangiitis
    Several tiny, punched-out, crusted ulcers around the ankle and some larger ulcers with overlying eschars and surrounding erythema on the dorsal foot and lower shin.
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