Materiały źródłowe

• #1 Churg-Strauss syndrome – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/churg-strauss-syndrome/diagnosis-treatment/drc-20353765

  There’s no cure for Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA). But medications can help manage your symptoms. […] Prednisone, which reduces inflammation, is the most commonly prescribed drug for Churg-Strauss syndrome. Your doctor might prescribe a high dose of corticosteroids or a boost in your current dose of corticosteroids to get your symptoms under control quickly. […] High doses of corticosteroids can cause serious side effects, so your doctor will decrease the dose gradually until you’re taking the smallest amount that will keep your disease under control. Even lower doses taken for extended periods can cause side effects. […] Mepolizumab (Nucala) is currently the only drug approved by the U.S. Food and Drug Administration for treatment of Churg-Strauss syndrome. However, depending on the severity of disease and the organs involved, other medications may be required. Examples include: Azathioprine (Azasan, Imuran), Benralizumab (Fasenra), Cyclophosphamide, Methotrexate (Trexall), Rituximab (Rituxan). […] Because these drugs impair your body’s ability to fight infection and can cause other serious side effects, your condition will be closely monitored while you’re taking them.

• #2 Churg-Strauss syndrome – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/churg-strauss-syndrome/symptoms-causes/syc-20353760

  Churg-Strauss syndrome is rare and has no cure. Symptoms can usually be controlled with steroids and other powerful immunosuppressant drugs. […] Early diagnosis and treatment improve the chances of a good outcome. […] Without treatment, the disease can be fatal.

• #3 Churg-Strauss syndrome – Hancock Health

  https://www.hancockhealth.org/mayo-health-library/churg-strauss-syndrome/

  Because these drugs impair your bodys ability to fight infection and can cause other serious side effects, your condition will be closely monitored while youre taking them. […] Long-term treatment with corticosteroids can cause a number of side effects. You can minimize these problems by taking the following steps: Protect your bones. Ask your doctor how much vitamin D and calcium you need in your diet, and discuss whether you should take supplements. Exercise. Exercise can help you maintain a healthy weight, which is important when youre taking corticosteroid medications that can cause weight gain. Strength training and weight-bearing exercises such as walking and jogging also help improve bone health. Eat a healthy diet. Steroids can cause high blood sugar levels and, eventually, type 2 diabetes. Eat foods that help keep blood sugar stable, such as fruits, vegetables and whole grains. […] If you have signs and symptoms common to Churg-Strauss syndrome, make an appointment with your doctor. Early diagnosis and treatment significantly improve the outlook of this condition.

• #4 Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/942

  The cornerstone of treatment is corticosteroids. Severe disease is treated in a similar manner to other anti-neutrophil cytoplasmic antibody associated vasculitides, with additional immunosuppressive agents. […] Treatment algorithm

• #5 Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): Treatment and prognosis – UpToDate

  https://www.uptodate.com/contents/eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-treatment-and-prognosis

  Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): Treatment and prognosis […] The goals of therapy are to achieve rapid onset of remission (particularly organ-threatening manifestations), maintain a long remission, and minimize complications of therapy. […] REMISSION INDUCTION […] Systemic glucocorticoids […] Severe EGPA […] – Choice of agent […] – Cyclophosphamide […] – Rituximab […] Nonsevere EGPA […] – Anti-IL-5 or anti-IL-5R agents […] – Methotrexate, azathioprine, mycophenolate as alternative agents […] REMISSION MAINTENANCE […] Glucocorticoid dosing and taper […] Choice of maintenance agent […] Azathioprine […] Methotrexate […] Mycophenolate mofetil […] Anti-IL-5 and anti-IL-5 receptor agents […] Rituximab […] Adjunctive topical therapy for asthma and chronic rhinosinusitis […] LESS COMMON THERAPIES […] Leflunomide […] Anti-IgE therapy […] Hydroxyurea […] Interferon-alpha […] Intravenous immune globulin […] Plasma exchange

• #6 Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK537099/

  Corticosteroids help reduce the burden of eosinophils in blood and tissues and inhibit the prolonged survival of eosinophils in extravascular tissues. For non-severe disease, the initial dose of therapy typically involves 1 mg/kg of oral prednisone. However, remission induction in severe cases is often more effectively achieved with pulse doses of methylprednisolone, with or without cyclophosphamide. […] The 2022 EULAR Recommendations for the Treatment of EGPA and Results of the French Vasculitis Study […] For new-onset or relapsing EGPA with organ/life-threatening manifestations, high-dose glucocorticoids plus either cyclosporine or rituximab is recommended. […] For patients with FFS 1, corticosteroids with IV cyclophosphamide is preferred. […] For new-onset or relapsing EGPA without organ/life-threatening disease, treatment with glucocorticoids alone is recommended. Glucocorticoids alone achieve remission in over 90% of cases.

• #6 Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK537099/

  The ACR-EULAR organizations are currently developing treatment criteria for AAV. Based on new clinical trial data, recent recommendations distinguish between GPA/MPA as one treatment group and EGPA as a separate group. Early recognition and the effective use of corticosteroids and immunosuppressants have significantly altered the natural history of EGPA, leading to improved prognosis and overall survival. Treatment involves 2 stages: induction of remission, defined as the absence of active disease symptoms, and maintenance therapy. […] Current treatment choices are based on classifying patients by the extent of involvement in limited or severe disease. Severe disease constitutes a life-threatening or organ-threatening disease, which includes active glomerulonephritis, pulmonary hemorrhage, cerebral vasculitis, progressive peripheral or cranial neuropathy, gastrointestinal bleeding, pericarditis, orbital pseudotumor, or myocarditis. Non-organ or life-threatening disease includes manifestations such as asthma, rhinosinusitis, cutaneous disease, or mild arthritis.

• #6 Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK537099/

  For induction in relapsing or refractory EGPA without organ/life-threatening disease, mepolizumab (an IL-5 inhibitor) is recommended by the 2022 EULAR guidelines. […] For maintaining remission in relapsing EGPA after induction for non-organ-threatening or non-life-threatening disease, mepolizumab is recommended. […] Treatment strategies for frequent relapses or severe refractory disease often depend on end-organ involvement. Plasmapheresis is effective and preferred in rapidly progressive glomerulonephritis or alveolar hemorrhage. Intravenous immunoglobulin (IVIG), on the other hand, is considered for neuropathy or cardiomyopathy, which is refractory to conventional therapy. […] Recent reports highlight the successful use of mepolizumab (an anti-IL-5 monoclonal antibody) and omalizumab (a recombinant humanized monoclonal anti-IgE antibody) in refractory EGPA.

• #7 Treatment of Churg-Strauss Syndrome

  https://www.webmd.com/lung/churg-strauss-treatment

  Churg-Strauss syndrome is also called eosinophilic granulomatosis with polyangiitis, or EGPA. There’s no cure, but steroids and other medications can help control symptoms. […] Glucocorticoids — a type of „steroid” — are man-made versions of hormones your body makes. Prednisone and other steroid drugs bring down inflammation in your body quickly. […] Your doctor will likely start you on a high-dose prednisone pill — 40 to 60 milligrams a day. This medicine can put your disease into remission, which is when you no longer have any symptoms or signs of Churg-Strauss syndrome. […] If a steroid alone doesn’t control your symptoms, you may need to take medications known as „glucocorticoid sparing therapy” along with it. […] In 2017, the FDA approved the first drug specifically to treat Churg-Strauss syndrome. Mepolizumab (Nucala) is a biologic drug.

• #8 Churg–Strauss syndrome: Case series | Pulmonology

  https://www.journalpulmonology.org/en-churgstrauss-syndrome-case-series-articulo-S2173511512000061

  ChurgStrauss syndrome (CSS) is a systemic necrotizing vasculitis of the small and medium vessels, associated with extravascular eosinophilic granulomas, peripheral eosinophilia and asthma. […] CSS patients usually respond well to steroid treatment, although relapses are common after it ends. Timely diagnosis and treatment generally lead to a good prognosis with a 90% survival rate at one year. […] The treatment of CSS is similar to various other small-vessel vasculitis. High dose steroids are the mainstay of treatment and generally begin with a dose of 1mg/Kg/day (max 60mg/day) of prednisolone, maintained until symptoms are controlled and then slowly tapered (for one year). In patients with factors which have a poor prognosis (central nervous system, renal, cardiac or gastro-intestinal involvement), the combination of steroids and cyclophosphamide produced better control and sustained remission rates, compared to steroids alone and is therefore included for remission induction in the European League Against Rheumatism (EULAR) recommendations for the management of small and medium vessel vasculitis.

• #8 Churg–Strauss syndrome: Case series | Pulmonology

  https://www.journalpulmonology.org/en-churgstrauss-syndrome-case-series-articulo-S2173511512000061

  For remission maintenance, the combination of low-dose steroids and azathioprine, leflunomide or methotrexate is used. […] Patients not responding or who relapse despite appropriate therapy are managed with different therapies, including mycophenolate mofetil, human intravenous immunoglobulin, and biological agents including rituximab, interferon-alpha, and mepolizumab. […] Treatment dramatically changes the prognosis of CSS patients (over 90% survival in one year). Nevertheless, following suspension of steroids, recurrent disease is observed in up to 25% of patients in the first five years, with asthma and sinusitis being the most frequent manifestations.

• #9 Churg-Strauss Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/1178795-treatment

  The mainstay of treatment of Churg-Strauss disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), is combination of steroids and immunosuppressants agents. Medical management of cardiovascular, cardiac, renal, and gastrointestinal complications of EGPA, falls under the purview of subspecialty consultation. […] The recommended initial medications for treatment of severe manifestations of eosinophilic granulomatosis with polyangiitis (EGPA), including patients with EGPA-related peripheral neuritis, are corticosteroids, which are administered at high doses. In mild disease, 1 mg/kg of prednisone can be initiated. In more severe cases, the use of intravenous methylprednisolone at doses of 15 mg/kg on 13 successive mornings is a better option. Rapid correction of eosinophilia, leukocytosis, and elevations of sedimentation rate and LDH are characteristic of EGPA. Failure to provoke such corrections early in the course of therapy is associated with elevated risk for poor long-term outcome.

• #9 Churg-Strauss Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/1178795-treatment

  In December 2017, mepolizumab (Nucala), an interleukin-5 antagonist monoclonal antibody (IgG1 kappa), became the first drug approved by the US Food and Drug Administration (FDA). […] In September 2024, benralizumab (Fasenra) became the second drug approved by the FDA for the treatment of adults with EGPA. Benralizumab is a monoclonal antibody against the interleukin-5 receptor expressed on eosinophils. […] Cyclophosphamide treatment (titrated to the neutrophil count) generally is continued for 6-12 months after remission is established. […] Azathioprine, methotrexate, or ribavirin have possible roles in the treatment of CSD, but these drugs require additional study and should not be used without the participation of a subspecialist who can provide recommendations concerning dosage, anticipated benefits, and adverse effects. […] The suggestion has been made that males with CSD might attain some benefit from treatment with thalidomide. This approach requires considerable additional study and the participation of an expert who can provide information concerning appropriate dosage, anticipated benefits, and adverse effects.

• #10 Churg-Strauss syndrome | STROKE MANUAL

  https://www.stroke-manual.com/churg-strauss-syndrome/

  glucocorticoids are the mainstay of therapy (pulse prednisolone 15 mg/kg for 1-3 days, followed by prednisone 1 mg/kg per day) […] in some patients, other immunosuppressive drugs are added to the steroid regimen (steroid-sparing effect) […] cyclophosphamide bolus should be considered in severe cases […] rituximab has proven useful in the treatment of steroid-resistant cases, as well as in the prevention and treatment of relapse (however, efficacy is greatly reduced in ANCA-negative patients) […] interleukin-5 (IL-5) antagonist monoclonal antibody […] mepolizumab approved by the FDA for use in adult patients with EGPA […] benralizumab […] plasma exchange has been studied in EGPA and other ANCA-positive vasculitides without a clear benefit.

• #11 Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/19543

  The ACR-EULAR organizations are currently developing treatment criteria for AAV. Based on new clinical trial data, recent recommendations distinguish between GPA/MPA as one treatment group and EGPA as a separate group. Early recognition and the effective use of corticosteroids and immunosuppressants have significantly altered the natural history of EGPA, leading to improved prognosis and overall survival. Treatment involves 2 stages: induction of remission, defined as the absence of active disease symptoms, and maintenance therapy. […] Current treatment choices are based on classifying patients by the extent of involvement in limited or severe disease. Severe disease constitutes a life-threatening or organ-threatening disease, which includes active glomerulonephritis, pulmonary hemorrhage, cerebral vasculitis, progressive peripheral or cranial neuropathy, gastrointestinal bleeding, pericarditis, orbital pseudotumor, or myocarditis. Non-organ or life-threatening disease includes manifestations such as asthma, rhinosinusitis, cutaneous disease, or mild arthritis.

• #11 Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/19543

  For new-onset or relapsing EGPA without organ/life-threatening disease, treatment with glucocorticoids alone is recommended. Glucocorticoids alone achieve remission in over 90% of cases. Although relapses are common with this treatment, adding other agents has not been proven to improve outcomes. The recommended dose is 1 mg/kg/day or 60 mg/day maximum for 2 to 3 weeks, followed by a taper. […] For induction in relapsing or refractory EGPA without organ/life-threatening disease, mepolizumab (an IL-5 inhibitor) is recommended by the 2022 EULAR guidelines. Other IL-5 or IL-5 receptor inhibitors (eg, reslizumab, bevacizumab, depemokimab) have shown efficacy in small trials, but more robust data are lacking. […] Treatment strategies for frequent relapses or severe refractory disease often depend on end-organ involvement. Plasmapheresis is effective and preferred in rapidly progressive glomerulonephritis or alveolar hemorrhage. Intravenous immunoglobulin (IVIG), on the other hand, is considered for neuropathy or cardiomyopathy, which is refractory to conventional therapy.

• #12 EGPA (Formerly Churg-Strauss Syndrome): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/churg-strauss-syndrome-eosinophilic-granulomatosis-with-polyangiitis-egpa

  What is the treatment for EGPA (formerly Churg-Strauss syndrome)? Treatment for EGPA begins with a high dose of corticosteroids to reduce inflammation and eosinophils. When theyve reduced enough to relieve your symptoms, your disease is in remission. At this point, your provider will begin reducing your dose. Most people continue to take a low dose of corticosteroids to maintain remission. […] Your provider may prescribe additional medications if corticosteroids alone arent effective enough, or if you want to avoid taking a high dose of corticosteroids. […] Additional medications include immunosuppressants and biologics. Mepolizumab is the first U.S. Food and Drug Administration (FDA)-approved biologic therapy for EGPA. Benralizumbab, another biologic agent, performed equally well in a recent clinical trial. These recent breakthroughs in research offer new treatment options for people with EGPA.

• #13 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) Treatment & Management: Approach Considerations, Medical Care, Consultations

  https://emedicine.medscape.com/article/333492-treatment

  Systemic corticosteroid therapy is the cornerstone of management of eosinophilic granulomatosis with polyangiitis (EGPA). Selection of the agent and regimen is guided by the patient’s five factor score (FFS); see Workup/Five Factor Score. […] Rituximab, which is approved for use in granulomatosis with polyangiitis and microscopic polyangiitis, has proved useful in treatment of steroid-resistant EGPA, as well as for prevention and treatment of relapse. […] Major life-threatening organ involvement may require treatment with pulse doses of intravenous corticosteroids and other cytotoxic agents. Cyclophosphamide is typically given in intravenous pulses for 3 months; afterward, patients are converted to oral steroids plus azathioprine, mycophenolate, or methotrexate for maintenance therapy.

• #13 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) Treatment & Management: Approach Considerations, Medical Care, Consultations

  https://emedicine.medscape.com/article/333492-treatment

  In contrast, the American College of Rheumatology recommends combination therapy with corticosteroid and mepolizumab for non-severe disease. […] Patients with an FFS 1 or any life-threatening manifestations should be treated with intravenous methylprednisolone 1 g/day for 3 days along with induction immunosuppressive agents. […] To decrease cyclophosphamide toxicity, bladder protection with hydration and possibly mesna prophylaxis must be considered. […] Once an induction regimen has been completed, maintenance treatment with rituximab, azathioprine 2-3 mg/kg/day, or methotrexate 0.3 mg/kg/week is recommended for the next 18-24 months. […] Intravenous immunoglobulin (IVIG) can be considered as a second-line agent in patients with refractory disease, pregnancy, cardiac insufficiency, or peripheral neuropathy.

• #13 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) Treatment & Management: Approach Considerations, Medical Care, Consultations

  https://emedicine.medscape.com/article/333492-treatment

  Two monoclonal antibodies that are interleukin-5 (IL-5) antagonists are approved for use in adult patients with EGPA: mepolizumab and benralizumab. They are typically considered for induction therapy in patients who have relapsing-refractory disease without organ- or life-threatening manifestations and for maintaining remission, principally for the control of asthma and as corticosteroid-sparing agents. […] If a relapse recurs, re-evaluation with corticosteroid or re-induction therapy should be guided by the severity of patients signs and symptoms as well as the FFS. […] Patients with EGPA also require management of asthma. Treatment is the same as for asthma in the general population, with a stepwise approach tailored to asthma severity. […] In patients with non-severe EGPA (FFS = 0), the French Vasculitis Study Group recommends starting prednisone at a dosage of 1 mg/kg (maximum dose, 60 mg) for 2-3 weeks.

• #14 Rituximab in the treatment of refractory or relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/ar4313

  Eosinophilic granulomatosis with polyangiitis (EGPA) is part of antineutrophil cytoplasmic antibodies (ANCAs)-associated vasculitides. […] B cell-depleting therapy with rituximab (RTX) can be effective in ANCA-positive EGPA, but very few patients have been published to date. […] In our analysis on nine patients with EGPA resistant to standard therapy, rituximab proved to be an efficient and safe treatment for ANCA-positive and ANCA-negative patients. […] Preemptive retreatment with RTX, combined with standard maintenance immunosuppressants, resulted in a sustained treatment response. […] Our data on patients with EGPA resistant to standard therapy indicate that rituximab is an efficient and safe treatment for ANCA-positive and potentially also for ANCA-negative patients with EGPA.

• #15 Churg-Strauss syndrome – GPnotebook

  https://gpnotebook.com/en-GB/pages/rheumatology/churg-strauss-syndrome

  The vasculitic phase that follows is life-threatening but can often be treated effectively with immunosuppression. […] Induction treatment for most patients with ANCA associated vasculitis (AAV) should be with cyclophosphamide or rituximab and glucocorticoids. […] AAV should be considered to be a chronic disease needing long term immunosuppressive therapy. […] Rituximab should be considered as an alternative induction agent for those at high risk of infertility and infection. […] Although earlier evidence suggested that induction with rituximab may be especially useful in ANCA-positive EGPA, the REOVAS trial found no significant differences between cyclophosphamide and rituximab. […] Mortality remains high, and late death is due to cardiovascular disease, infection (secondary to treatment) and malignancy.

• #16 Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis | Nature Reviews Rheumatology

  https://www.nature.com/articles/s41584-023-00958-w

  Several cell types participate in the immunopathogenesis of the disease. Eosinophils are clearly central and are likely to mediate tissue damage, a concept supported by the evidence that targeting IL-5 (for example, using mepolizumab), a survival factor for eosinophils, is an effective therapy for EGPA. […] Given the rarity of EGPA, its heterogeneous clinical presentation and the clinical overlap with other vasculitic or eosinophilic disorders, the diagnosis of EGPA is often challenging. […] The 16 statements are reported and discussed below and in Table 2, and the overarching principles are reported in Box 1. […] For remission induction in patients with new-onset, active EGPA, glucocorticoids should be administered as initial therapy. […] In patients with severe disease (unfavourable prognostic factors discussed in Statement 6) cyclophosphamide or, as an alternative, rituximab, should be added.

• #16 Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis | Nature Reviews Rheumatology

  https://www.nature.com/articles/s41584-023-00958-w

  The MIRRA RCT tested the efficacy and safety of mepolizumab versus placebo in achieving remission (BVAS of zero and prednisolone dose 4mg per day) in patients with relapsing or refractory EGPA without organ- or life-threatening manifestations. […] Mepolizumab proved significantly more efficacious than placebo and had comparable toxicity. […] We recommend rituximab maintenance in patients with severe disease, particularly in those who achieved remission on rituximab. […] Mepolizumab is commonly used during remission maintenance, mainly for the control of asthma and to reduce glucocorticoid exposure. […] The IL-5 inhibitor mepolizumab in combination with glucocorticoids is recommended to induce remission in patients with relapsing-refractory EGPA without organ- or life-threatening manifestations.

• #17 Eosinophilic granulomatosis with polyangiitis – Wikipedia

  https://en.wikipedia.org/wiki/Eosinophilic_granulomatosis_with_polyangiitis

  Treatment for eosinophilic granulomatosis with polyangiitis includes glucocorticoids (such as prednisolone) and other immunosuppressive drugs (such as azathioprine and cyclophosphamide). In many cases, the disease can be put into a type of chemical remission through drug therapy, but the disease is chronic and lifelong. […] A systematic review conducted in 2007 indicated all patients should be treated with high-dose steroids, but in patients with a five-factor score of one or higher, cyclophosphamide pulse therapy should be commenced, with 12 pulses leading to fewer relapses than six. Remission can be maintained with a less toxic drug, such as azathioprine or methotrexate. […] On 12 December 2017, the FDA approved mepolizumab, the first drug therapy specifically indicated for the treatment of eosinophilic granulomatosis with polyangiitis. Patients taking mepolizumab experienced a „significant improvement” in their symptoms. Mepolizumab is a monoclonal antibody that targets interleukin-5, a major factor in eosinophil survival.

• #17 Eosinophilic granulomatosis with polyangiitis – Wikipedia

  https://en.wikipedia.org/wiki/Eosinophilic_granulomatosis_with_polyangiitis

  In addition to mepolizumab, a number of emerging targeted biotherapies including the anti-IgE monoclonal antibody omalizumab, immunomodulation with Interferon-, and B cell therapy with rituximab may lead to increasingly personalized treatment regimens for future EGPA patients. A review of EGPA treatments conducted in 2020 proposes integrating targeted biotherapies into EGPA management plans following failure of treatment with corticosteroids. […] On 18 September 2024, AstraZeneca announced FDA approval for Fasenra (benralizumab), a biologic drug therapy indicated for use in adult patients with EGPA, following the MANDARA Phase III trial results. Published in The New England Journal of Medicine, the first head-to-head non-inferiority trial of biologics in patients with relapsing or refractory EGPA measured the efficacy and safety of Fasenra against mepolizumab. Patients were randomized to receive either one 30mg subcutaneous injection of Fasenra, or three 100mg subcutaneous injections of mepolizumab every four weeks. Resultantly, nearly 60% of Fasenra-treated patients achieved remission, with 41% of Fasenra-treated patients fully tapered from oral corticosteroids compared to 26% in mepolizumab-treated patients.

• #18 FDA approves first drug for Eosinophilic Granulomatosis with Polyangiitis, a rare disease formerly known as the Churg-Strauss Syndrome | FDA

  https://www.fda.gov/news-events/press-announcements/fda-approves-first-drug-eosinophilic-granulomatosis-polyangiitis-rare-disease-formerly-known-churg

  Nucala is administered once every four weeks by subcutaneous injection by a health care professional into the upper arm, thigh, or abdomen. […] The primary efficacy assessment in the trial measured Nucalas treatment impact on disease remission (i.e., becoming symptom free) while on an OCS dose less than or equal to 4 mg of prednisone. Patients receiving 300 mg of Nucala achieved a significantly greater accrued time in remission compared with placebo. A significantly higher proportion of patients receiving 300 mg of Nucala achieved remission at both week 36 and week 48 compared with placebo. In addition, significantly more patients who received 300 mg of Nucala achieved remission within the first 24 weeks and remained in remission for the remainder of the 52-week study treatment period compared with patients who received the placebo.

• #19 Eosinophilic Granulomatosis with Polyangiitis – Vasculitis Foundation

  https://vasculitisfoundation.org/education/vasculitis-types/eosinophilic-granulomatosis-with-polyangiitis/

  In EGPA, both mepolizumab and benralizumab work by reducing the number of eosinophils in the body, and allow for a substantial reduction in the use of prednisone. […] Mepolizumab is indicated for patients with mild to moderate disease with mainly asthma, sinus, nose, and ear symptoms. […] Both mepolizumab and benralizumab are not indicated for more severe or life-threatening disease. […] In 2021 the American College of Rheumatology (ACR) published guidelines for the management of certain vasculitides, that were also endorsed by the Vasculitis Foundation (VF).

• #20 Researchers identify new choice of therapy for rare autoimmune disease EGPA – Faculty of Health Sciences

  https://healthsci.mcmaster.ca/researchers-identify-new-choice-of-therapy-for-rare-autoimmune-disease-egpa/

  Parameswaran Nair and a team of international researchers have identified a biologic drug called benralizumab as a therapeutic for people living with eosinophilic granulomatosis with polyangiitis. […] A biologic drug called benralizumab has been shown to be non-inferior to mepolizumab in the treatment of EGPA. […] Our findings show that benralizumab was just as effective as mepolizumab at reducing exacerbations and providing disease remission during the 52 weeks of the study, says Parameswaran Nair, a professor with McMasters Department of Medicine and a respirologist at St. Joes Firestone Institute for Respiratory Health. […] The single 30 mg subcutaneous dosing of benralizumab offers an advantage to patients over the three 100 mg subcutaneous dosing of mepolizumab, says Nair. […] The researchers noted that approximately 16 per cent more patients in the benralizumab group were able to abstain from using oral corticosteroids compared to the mepolizumab group.

• #20 Researchers identify new choice of therapy for rare autoimmune disease EGPA – Faculty of Health Sciences

  https://healthsci.mcmaster.ca/researchers-identify-new-choice-of-therapy-for-rare-autoimmune-disease-egpa/

  In our study, treatment with benralizumab allowed more patients to discontinue prednisone over a 52-week period compared to mepolizumab. […] Benralizumab was associated with greater blood eosinophil depletion than mepolizumab from week one onwards, says Nair. […] It is very gratifying that our research program at the Firestone Institute at St. Joes has led to the development of these new treatment options for patients with severe eosinophilic diseases, Nair says.

• #21 IVIG for Churg-Strauss Syndrome: How This Therapy Helps When Standard Treatments Fall Short – AmeriPharma® Specialty Care

  https://ameripharmaspecialty.com/ivig/ivig-for-churg-strauss-syndrome-how-this-therapy-helps-when-standard-treatments-fall-short/

  Various case reports have shown that IVIG therapy can be used as a second-line treatment in patients with severe Churg-Strauss syndrome. […] However, in some cases, IVIG therapy is recommended as a second-line treatment when the conventional therapies are not sufficient. […] Various case reports have shown that IVIG therapy can effectively treat patients with CSS when other standard treatments do not yield promising results. […] IVIG therapy is known to have anti-inflammatory properties. It can help reduce the inflammation in the blood vessels and eventually reduce the risk of severe organ damage. […] IVIG also helps to neutralize or block the activity of autoantibodies. […] In CSS patients, IVIG helps reduce the eosinophil level in the blood. It does this by blocking the activity of eosinophils. This prevents the excessive buildup of eosinophils in the tissues.

• #22 Biologic Therapy for Eosinophilic Granulomatosis With Polyangiitis: Building the Therapeutic Armamentarium – Pulmonology Advisor Biologic Therapy for Eosinophilic Granulomatosis With Polyangiitis: Building the Therapeutic Armamentarium

  https://www.pulmonologyadvisor.com/cch/biologic-therapy-for-eosinophilic-granulomatosis-with-polyangiitis-building-the-therapeutic-armamentarium/

  Emerging data show that certain biologics may produce enduring results often defined as remission, less reliance on oral glucocorticoids, and reduction in flares in patients with EGPA. […] Evidence now suggests that mepolizumab, a monoclonal antibody that targets IL-5, may help patients achieve EGPA remission. […] More than half of patients treated with mepolizumab (53%) had achieved remission vs 19% of patients receiving placebo. […] For any of these reasons, mepolizumab was effective in EGPA. […] Beyond mepolizumab, other biologic agents have been studied for the treatment of EGPA, including reslizumab, benralizumab, rituximab, and omalizumab. […] Omalizumab showed benefit as a steroid-sparing agent, with the mean dose reduced to 6.28 mg/d, and was associated with reduced asthma exacerbations and hospitalizations. […] For patients with EGPA, biologics may offer an alternative to high-dose steroids. By ameliorating asthma symptoms as well as eosinophilic-driven inflammation, biologics may prolong time to relapse and reduce adverse events associated with steroids.

• #23 Churg Strauss Syndrome – Vasculitis UK

  https://www.vasculitis.org.uk/about-vasculitis/churg-strauss-syndrome

  Corticosteroids Steroids like prednisolone form the backbone of treatment of people with EGPA. Steroids are often needed at quite high levels initially, and should be tapered off although this can take many years. Reduction of steroid treatment should be done very carefully as dropping steroid levels too quickly can result in EGPA becoming more active or not having enough steroid within the body. Steroids can have multiple side effects see page on corticosteroids. […] Nasal and inhaled steroids As well as steroid tablets, people with EGPA often need steroid sprays to treat their nose and sinus symptoms. These can help people breathe through their nose more easily and try to preserve their sense of smell. Steroid inhalers are used to control the asthma that almost all people with EGPA experience and prevent worsening of their breathing.

• #24 Eosinophilic Granulomatosis with Polyangiitis, formerly Churg-Strauss Syndrome (EGPA) : Johns Hopkins Vasculitis Center

  https://www.hopkinsvasculitis.org/types-vasculitis/churgstrauss-syndrome-css/

  EGPA usually responds to prednisone. Initially, high doses of oral prednisone are used in an attempt to get the disease into remission as quickly as possibly (e.g., using oral prednisone 40-60 mg/day). After the first month or so, this high dose of prednisone is gradually tapered down over the ensuing months. Other immunosuppressive drugs, such as azathioprine, cellcept, methotrexate, cyclophosphamide, or rituximab may be used in addition to prednisone. High doses of intravenous steroids (usually methylprednisolone) maybe useful for those patients with severe disease or for those who are unresponsive to the combination of oral prednisone used with other immunosuppressive medications. […] Prior to the advent of prednisone, EGPA was often a fatal disease. The majority of patients died from rampant, uncontrolled disease. With present therapy, constitutional symptoms begin to resolve quite quickly, with gradual improvement in cardiac and renal function, as well as improvement in the pain that results from peripheral nerve involvement. The course of therapy can last for 1 to 2 years, although the length and type of treatment depend on the severity of disease and the organs involved. The patients response to treatment and the continuation of disease control during lowering of the prednisone dose are the primary determinants of how long therapy is continued. Laboratory monitoring of blood tests is very helpful in gauging the activity of disease. Some of the most useful laboratory tests are the erythrocyte sedimentation rate (ESR) and the eosinophil count.

• #25 Eosinophilic Granulomatosis with Polyangiitis formally Churg Strauss Syndrome — Vasculitis Ireland Awareness (VIA)

  https://www.vasculitis-ia.org/vasculitis-types/eosinophilic-granulomatosis-with-polyangiitis-formally-churg-strauss-syndrome

  Patient education and lifestyle modifications: Educating patients about their condition, potential triggers, and the importance of medication adherence and lifestyle modifications (such as smoking cessation, regular exercise, and a healthy diet) can help improve long-term outcomes and quality of life. […] Treatment for EGPA is typically tailored to each individual’s specific needs and may require ongoing adjustments based on disease activity, treatment response, and the presence of comorbidities. Early diagnosis and aggressive treatment are essential to achieve and maintain remission, prevent disease flares, and minimise the risk of complications associated with EGPA.

Zobacz więcej