Eosinophilic granulomatosis with polyangitis(EGPA; Churg-Strauss syndrome): Treatments
Differential diagnosis
The most common diseases are aspirin-exacerbated respiratory disease (AERD), hypereosinophilic syndrome, granulomatosis with polyangiitis (Wegener's), microscopic polyangiitis, eosinophilic pneumonia and allergic bronchopulmonary aspergillosis. In AERD, asthma, chronic rhinosinusitis with nasal polyposis, and hypersensitivity to aspirin or NSAID, which are bronchoconstriction, rhinorrhea and nasal congestion. Serum eosinophilia may also increase, but eosinophilic pneumonia and other organ involvement are not seen. AERD can evolve into EGPA, so proper management is essential. Chronic eosinophilic pneumonia may be difficult to differentiate because of its clinical features similar to those of EGPA. However, there is no characteristic granuloma on histologic examination and there is no other organ penetration except the lung. Patients with hypereosinophilic syndrome (HES) may have cough and pulmonary infiltration, but asthma is rare. In order to differentiate, we need to find the FIP1L1 / PDGFR alpha mutation characteristic of HES by molecular testing. Asthma and eosinophilia are not characteristic of other vasculitis (Wegener's, microscopic polyangiitis) and are significantly less common than EGPA. In addition, EGPA is predominantly p-ANCA with MPO, whereas other granulomatosis with polyangiitis AP-3 c-ANCA is more prevalent.
Treatments
To treat EGPA, the severity of vasculitis must first be assessed. The five-factor score established the initial treatment plan based on the presence or absence of five clinical features including cardiac involvement, GI involvement (bleeding), renal failure (creatinine> 1.58 mg / dL), proteinuria> 1 g / day and central nervous system Involvement. In 2009, it was reported that all of the age-related items (> 65 years old) and absence of otorhinolaryngology symptoms (if the symptoms of Ent had a better therapeutic result), they were all associated with prognosis. Initial therapy is systemic glucocorticoids. If the disease is late, recurrent, or flare occurs at the time of steroid tapering, use an immunosuppressive agent. Basically, treatment is prednisone 0.5-1.5 mg / kg per day PO and high dose or IV for severe patients. In cases of severe and multiorgan involvement, cyclophosphamide is mainly used with systemic steroids. Initial therapy usually takes 6 to 12 months to remission, and if successful, it is replaced with an immunosuppressive agent that is less toxic and goes to maintenance therapy. The most commonly used drugs are azathioprine (increasing 0.5 mg up to 25-50 mg / day), methotrexate (20-25 mg / week with folic acid 1 mg / day), and leflunomide (10-30 mg / day). Other therapies include rituximab, interferon-alpha, IVIG, and anti-IgE therapy.
The most common diseases are aspirin-exacerbated respiratory disease (AERD), hypereosinophilic syndrome, granulomatosis with polyangiitis (Wegener's), microscopic polyangiitis, eosinophilic pneumonia and allergic bronchopulmonary aspergillosis. In AERD, asthma, chronic rhinosinusitis with nasal polyposis, and hypersensitivity to aspirin or NSAID, which are bronchoconstriction, rhinorrhea and nasal congestion. Serum eosinophilia may also increase, but eosinophilic pneumonia and other organ involvement are not seen. AERD can evolve into EGPA, so proper management is essential. Chronic eosinophilic pneumonia may be difficult to differentiate because of its clinical features similar to those of EGPA. However, there is no characteristic granuloma on histologic examination and there is no other organ penetration except the lung. Patients with hypereosinophilic syndrome (HES) may have cough and pulmonary infiltration, but asthma is rare. In order to differentiate, we need to find the FIP1L1 / PDGFR alpha mutation characteristic of HES by molecular testing. Asthma and eosinophilia are not characteristic of other vasculitis (Wegener's, microscopic polyangiitis) and are significantly less common than EGPA. In addition, EGPA is predominantly p-ANCA with MPO, whereas other granulomatosis with polyangiitis AP-3 c-ANCA is more prevalent.
Treatments
To treat EGPA, the severity of vasculitis must first be assessed. The five-factor score established the initial treatment plan based on the presence or absence of five clinical features including cardiac involvement, GI involvement (bleeding), renal failure (creatinine> 1.58 mg / dL), proteinuria> 1 g / day and central nervous system Involvement. In 2009, it was reported that all of the age-related items (> 65 years old) and absence of otorhinolaryngology symptoms (if the symptoms of Ent had a better therapeutic result), they were all associated with prognosis. Initial therapy is systemic glucocorticoids. If the disease is late, recurrent, or flare occurs at the time of steroid tapering, use an immunosuppressive agent. Basically, treatment is prednisone 0.5-1.5 mg / kg per day PO and high dose or IV for severe patients. In cases of severe and multiorgan involvement, cyclophosphamide is mainly used with systemic steroids. Initial therapy usually takes 6 to 12 months to remission, and if successful, it is replaced with an immunosuppressive agent that is less toxic and goes to maintenance therapy. The most commonly used drugs are azathioprine (increasing 0.5 mg up to 25-50 mg / day), methotrexate (20-25 mg / week with folic acid 1 mg / day), and leflunomide (10-30 mg / day). Other therapies include rituximab, interferon-alpha, IVIG, and anti-IgE therapy.
