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      Drugs & Diseases > Rheumatology

      Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) Guidelines

      Updated: Aug 06, 2022
      • Author: Spencer T Lowe, MD; Chief Editor: Herbert S Diamond, MD  more...
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      Guidelines

      Guidelines Summary

      European guidelines from the EGPA Consensus Task Force recommend the following for the workup of eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss syndrome) [2] :

      • Serologic testing for toxocariasis and HIV
      • Specific IgE and IgG for  Aspergillus species
      • Testing for  Aspergillus spp. in sputum, bronchoalveolar lavage fluid, or both
      • Tryptase and vitamin B12 levels
      • Peripheral blood smear (looking for dysplastic eosinophils or blasts)
      • Antineutrophil cytoplasmic antibody (ANCA) with indirect immunofluorescence and enzyme-linked immunosorbent assay (ELISA)
      • Chest computed tomography (CT) scan

      Additional investigations should be guided by patient-specific clinical findings and an extensive search for causes of hypereosinophilia. [2]

      The EGPA Consensus Task Force encourages obtaining biopsies from patients with suspected EGPA. In the correct clinical context (asthma with eosinophilia or systemic manifestations, or even eosinophilia with extrapulmonary disease), a biopsy showing small- or medium-vessel vasculitis, a strong clinical surrogate of vasculitis, or both strongly supports a diagnosis of EGPA. [2]

      The American College of Rheumatology and the European Alliance of Associations for Rheumatology have published classification criteria for EGPA. [9] Diagnostic criteria and assigned points are as follows:

      • Maximum eosinophil count ≥1×10 9/L: +5
      • Obstructive airway disease: +3
      • Nasal polyps: +3
      • Cytoplasmic ANCA or anti-proteinase 3 ANCA positivity: -3
      • Extravascular eosinophilic-predominant inflammation: +2
      • Mononeuritis multiplex/motor neuropathy not due to radiculopathy: +1
      • Hematuria: -1

      Once mimics of vasculitis have been excluded, patients with a diagnosis of small- or medium-vessel vasculitis could be classified as having EGPA if their cumulative score was 6 points or higher. [9]

      Treatment

      The American College of Rheumatology/Vasculitis Foundation has published a guideline for the management of ANCA-associated vasculitis, including EGPA. The guideline contains recommendations and ungraded position statements; all the recommendations are conditional, as the level of supporting evidence ranges from very low to low. [57]

      Remission induction for active, severe EGPA

      • Position statement: Either IV pulse glucocorticoids or high-dose oral glucocorticoids may be prescribed as initial therapy.
      • Position statement: Either cyclophosphamide or rituximab may be prescribed for remission induction.
      • Treatment with cyclophosphamide or rituximab is recommended over mepolizumab for remission induction.

      Remission induction for active, nonsevere EGPA

      • Initiate treatment with mepolizumab and glucocorticoids rather than methotrexate, azathioprine, or mycophenolate mofetil and glucocorticoids.
      • Initiate treatment with methotrexate, azathioprine, or mycophenolate mofetil and glucocorticoids rather than glucocorticoids alone, cyclophosphamide, or rituximab and glucocorticoids.

      Remission maintenance

      • For patients with severe EGPA whose disease has entered remission with cyclophosphamide therapy, use methotrexate, azathioprine, or mycophenolate mofetil in preference to rituximab for remission maintenance.
      • For patients with severe EGPA whose disease has entered remission, use methotrexate, azathioprine, or mycophenolate mofetil in prefernce to mepolizumab for remission maintenance.
      • Position statement: The duration of glucocorticoid therapy in EGPA should be guided by the patient’s clinical condition, values, and preferences.

      Treatment of relapse

      • For patients with EGPA who have experienced relapse with severe disease manifestations after prior successful remission induction with cyclophosphamide, use rituximab rather than cyclophosphamide for remission re-induction.
      • For patients with EGPA who have experienced relapse with severe disease manifestations after prior successful remission induction with rituximab, use rituximab instead of switching to cyclophosphamide for remission re-induction.
      • For patients with EGPA who have experienced relapse with nonsevere disease manifestations (asthma and/or sinonasal disease) while receiving methotrexate, azathioprine, or mycophenolate mofetil, add mepolizumab instead of switching to another agent.
      • For patients with EGPA who have experienced relapse with nonsevere disease manifestations (asthma and/or sinonasal disease) while receiving low-dose glucocorticoids and no other therapy, add mepolizumab rather than adding methotrexate, azathioprine, or mycophenolate mofetil.
      • For patients with EGPA and high serum IgE levels who have experienced relapse with nonsevere disease manifestations (asthma and/or sinonasal disease) while receiving methotrexate, azathioprine, or mycophenolate mofetil, add mepolizumab rather than adding omalizumab.

      Other considerations

      • For patients with newly diagnosed EGPA who are receiving leukotriene inhibitors, continue leukotriene inhibitors.
      • Use of leukotriene inhibitors is not contraindicated for patients with EGPA with active asthma and/or sinonasal disease.
      • Obtain an echocardiogram at the time of EGPA diagnosis.
      • Use the Five-Factor Score to guide EGPA therapy.
      • Position statement: In patients with sinonasal involvement in EGPA, treatment with nasal rinses and topical therapies (eg, antibiotics, lubricants, and glucocorticoids) may be considered.
      • For patients with EGPA who are receiving cyclophosphamide or rituximab, prescribe medications for prophylaxis of Pneumocystis jirovecii pneumonia.
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