EDUCATION
EOSINOPHILIC GRANULOMATOSIS WITH POLYANGITIIS (EGPA, CHURG-STRAUSS SYNDROME)
Clinical features
Eosinophilic Granulomatosis with Polyangitiis (also known as Churg-Strauss syndrome) is a rare systemic vasculitis characterized by inflammation of the blood vessels, primarily of small size, leading to blood flow restriction and to consequent damage of vital organs and tissues.
EGPA can be associated with pulmonary, ear-nose-throat, cutaneous, cardiac, intestinal, renal and neurological manifestations.
The symptoms of EGPA can range from mild to life-threatening, and may vary among individuals, however almost all patients have asthma and/or sinus polyps, and hypereosinophylia (i.e., an increased level of white blood cells called eosinophils).
Other symptoms include:
EGPA can be associated with pulmonary, ear-nose-throat, cutaneous, cardiac, intestinal, renal and neurological manifestations.
The symptoms of EGPA can range from mild to life-threatening, and may vary among individuals, however almost all patients have asthma and/or sinus polyps, and hypereosinophylia (i.e., an increased level of white blood cells called eosinophils).
Other symptoms include:
- Fever
- Fatigue/malaise
- Rapid and sudden weight loss
- Muscle and joint pain
- Skin rashes
- Numbness or tingling of the hands or feet
- Sudden loss of strength in the hands or feet
- Chest pain or palpitations
- Increasing shortness of breath or coughing
- Abdominal pain
- Presence of blood in the stools
- Kidney disease
Etiopathogenesis
The etiopathogenesis of EGPA is not fully understood.
As a vasculitis, it is classified among autoimmune diseases; indeed, EGPA is known as an ANCA-associated vasculitis, as auto-antibodies attacking the body’s own blood protein (anti-neutrophil cytoplasmic antibody) can be detected in some patients.
However, infectious and environmental risk factors have been described, and they could play a role as triggers of EGPA onset or relapse, particularly on subjects with a genetic susceptibility.
As a vasculitis, it is classified among autoimmune diseases; indeed, EGPA is known as an ANCA-associated vasculitis, as auto-antibodies attacking the body’s own blood protein (anti-neutrophil cytoplasmic antibody) can be detected in some patients.
However, infectious and environmental risk factors have been described, and they could play a role as triggers of EGPA onset or relapse, particularly on subjects with a genetic susceptibility.
Diagnosis
The diagnosis of EGPA is not based on a single test, but rather considers a number of factors, including clinical history, laboratory tests, imaging, and, when indicated, a biopsy of an affected tissue or organ.
Among the most useful tests to diagnose EGPA are:
Among the most useful tests to diagnose EGPA are:
- Urinalysis: Excessive protein or presence of red blood cells may indicate inflammation of the kidneys.
- Blood tests: The ANCA test (i.e., the search for autoantibodies against anti-neutrophil cytoplasmic proteins), when positive, can be helpful in confirming diagnosis. However, a positive ANCA test is not a specific marker for EGPA diagnosis: indeed, only around 30-50 percent of individuals with EGPA are ANCA positive, and ANCA-test can be positive also in case of other ANCA-associated vasculitis (different from EGPA). Other common tests include blood inflammatory parameters, such as the erythrocyte sedimentation rate (ESR) and the C-reactive protein (CRP) test. All of these tests may support a diagnosis of EGPA, but are not conclusive on their own. A tissue biopsy is typically needed.
- Tissue biopsy: This surgical procedure removes a small tissue sample from an affected vessel or organ, which is examined under a microscope for signs of inflammation or tissue damage.
- Imaging studies: X-rays and computed tomography (CT) scans may reveal pulmonary or ear-nose-throat involvement.
- Heart echocardiogram: All patients with EGPA should be screened for heart involvement.
Treatment
EGPA is a serious but treatable disease. Treatment typically includes corticosteroids to reduce inflammation, mostly used in combination with immune-modulating/immunosuppressants therapies, such as methotrexate azathioprine, mycophenolate mofetil, or cyclophosphamide.
To date, novel therapeutic strategies (including the biologic agents rituximab and mepolizumab) have been developed, to reduce the need of steroids, the possible adverse events related to the long-term use of immune-modulating/immunosuppressants medications, as well as to guarantee a better control of the disease.
To date, novel therapeutic strategies (including the biologic agents rituximab and mepolizumab) have been developed, to reduce the need of steroids, the possible adverse events related to the long-term use of immune-modulating/immunosuppressants medications, as well as to guarantee a better control of the disease.
Disease course
Even with effective treatment, EGPA is a chronic illness with cycles of relapse and remission. For this reason, regular doctor visits and ongoing monitoring of lab and imaging tests are important to guarantee the disease control and to avoid serious complications.
Most relevant scientific publications and insights
C. Marvisi, R.A. Sinico, C. Salvarani, D. Jayne , D. Prisco, B. Terrier, G. Emmi, A. Vaglio, European EGPA Study Group
C. Marvisi et al.
New perspectives in eosinophilic granulomatosis with polyangiitis (EGPA): report of the first meeting of the European EGPA Study Group
P.A. Lyons, J.E. Peters, F. Alberici, J. Liley, R.M.R. Coulson, W. Astle, C. Baldini, F. Bonatti, M.C. Cid, H. Elding, G. Emmi, J. Epplen, L. Guillevin, D.R.W. Jayne, T. Jiang, I. Gunnarsson, P. Lamprecht, S. Leslie, M.A. Little, D. Martorana, F. Moosig, T. Neumann, S. Ohlsson, S. Quickert, G. A. Ramirez, B. Rewerska, G. Schett, R.A. Sinico, W. Szczeklik, V. Tesar, D. Vukcevic, European Vasculitis Genetics Consortium, B. Terrier, R.A. Watts, A. Vaglio, J.U. Holle, C. Wallace, K.G.C. Smith
P.A. Lyons et al.
Genome-wide association study of eosinophilic granulomatosis with polyangiitis reveals genomic loci stratified by ANCA status
S. Moiseev, X. Bossuyt, Y. Arimura, D. Blockmans, E. Csernok, J. Damoiseaux, G. Emmi, L.F. Flores-Suárez, B. Hellmich, D. Jayne, J.C. Jennette, M.A. Little, A.J. Mohammad, F. Moosig, P. Novikov, C. Pagnoux, A. Radice, K. Sada, M. Segelmark, Y. Shoenfeld, R. A. Sinico, U. Specks, B. Terrier, A.G. Tzioufas, A. Vaglio, M. Zhao, J.W. Cohen Tervaert, European EGPA Study Group
S. Moiseev et al.
International Consensus on ANCA Testing in Eosinophilic Granulomatosis with Polyangiitis
A. Canzian, N. Venhoff, M.L. Urban, S. Sartorelli, A.M. Ruppert, M. Groh, N. Girszyn, C. Taillé, F. Maurier, V. Cottin, C. de Moreuil, V. Germain, M. Samson, M. Jachiet, L. Denis, V. Rieu, P. Smets, G. Pugnet, A. Deroux, C.A. Durel, A. Aouba, P. Cathébras, C. Deligny, S. Faguer, H. Gil, B. Godeau, F. Lifermann, S. Phin-Huynh, M. Ruivard, P. Bonniaud, X. Puéchal, J.E. Kahn, J. Thiel, L. Dagna, L. Guillevin, A. Vaglio, G. Emmi, B. Terrier 1, French Vasculitis Study Group and the European EGPA Study Group
A. Canzian et al.
Use of Biologics to Treat Relapsing and/or Refractory Eosinophilic Granulomatosis With Polyangiitis: Data From a European Collaborative Study
M. Papo, R.A. Sinico, V. Teixeira, N. Venhoff, M.L. Urban, M. Iudici, J. Mahrhold, F. Locatelli, G. Cassone, F. Schiavon, B. Seeliger, T. Neumann, C. Kroegel, M. Groh, C. Marvisi, M. Samson, T. Barba, D. Jayne, A. Troilo, J. Thiel, B. Hellmich, S. Monti, C. Montecucco, C. Salvarani, J.E. Kahn, B. Bonnotte, C.A. Durel, X. Puéchal, L. Mouthon, L. Guillevin, G. Emmi, A. Vaglio, B. Terrier
M. Papo et al.
Significance of PR3-ANCA positivity in eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
M. Zampieri, G. Emmi, M. Beltrami, C. Fumagalli, M.L. Urban, L.L. Dei, A. Marchi, M. Berteotti, A. Tomberli, K. Baldini, A. Bettiol, S. Pradella, E. Silvestri, N. Marchionni, A. Vaglio, I. Olivotto, D. Prisco
M. Zampieri et al.
Cardiac involvement in eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome): Prospective evaluation at a tertiary referral centre
F. Maritati, F. Peyronel, P. Fenaroli, F. Pegoraro, V. Lastrucci, G.D. Benigno, A. Palmisano, G.M. Rossi, M.L. Urban, F. Alberici, P. Fraticelli, G. Emmi, M. Corradi, A. Vaglio
F. Maritati et al.
Occupational exposures and smoking in eosinophilic granulomatosis with Polyangiitis (Churg-strauss syndrome)
Bettiol A, Sinico RA, Schiavon F, Monti S, Bozzolo EP, Franceschini F, Govoni M, Lunardi C, Guida G, Lopalco G, Paolazzi G, Vacca A, Gregorini G, Leccese P, Piga M, Conti F, Fraticelli P, Quartuccio L, Alberici F, Salvarani C, Bettio S, Negrini S, Selmi C, Sciascia S, Moroni G, Colla L, Manno C, Urban ML, Vannacci A, Pozzi MR, Fabbrini P, Polti S, Felicetti M, Marchi MR, Padoan R, Delvino P, Caporali R, Montecucco C, Dagna L, Cariddi A, Toniati P, Tamanini S, Furini F, Bortoluzzi A, Tinazzi E, Delfino L, Badiu I, Rolla G, Venerito V, Iannone F, Berti A, Bortolotti R, Racanelli V, Jeannin G, Padula A, Cauli A, Priori R, Gabrielli A, Bond M, Tedesco M, Pazzola G, Tomietto P, Pellecchio M, Marvisi C, Maritati F, Palmisano A, Dejaco C, Willeit J, Kiechl S, Olivotto I, Willeit P, Prisco D, Vaglio A, Emmi G; Italian EGPA Consortium.
A. Bettiol et al.
Risk of acute arterial and venous thromboembolic events in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
Bettiol A, Urban ML, Bello F, Fiori D, Mattioli I, Lopalco G, Iannone F, Egan A, Dagna L, Caminati M, Negrini S, Bargagli E, Folci M, Franceschini F, Padoan R, Flossmann O, Solans R, Schroeder J, André M, Moi L, Parronchi P, Roccatello D, Sciascia S, Jayne D, Prisco D, Vaglio A, Emmi G; European EGPA Study Group.
A. Bettiol et al.
Sequential rituximab and mepolizumab in eosinophilic granulomatosis with polyangiitis (EGPA): a European multicentre observational study
Bettiol A, Urban ML, Dagna L, Cottin V, Franceschini F, Del Giacco S, Schiavon F, Neumann T, Lopalco G, Novikov P, Baldini C, Lombardi C, Berti A, Alberici F, Folci M, Negrini S, Sinico RA, Quartuccio L, Lunardi C, Parronchi P, Moosig F, Espígol-Frigolé G, Schroeder J, Kernder AL, Monti S, Silvagni E, Crimi C, Cinetto F, Fraticelli P, Roccatello D, Vacca A, Mohammad AJ, Hellmich B, Samson M, Bargagli E, Cohen Tervaert JW, Ribi C, Fiori D, Bello F, Fagni F, Moroni L, Ramirez GA, Nasser M, Marvisi C, Toniati P, Firinu D, Padoan R, Egan A, Seeliger B, Iannone F, Salvarani C, Jayne D, Prisco D, Vaglio A, Emmi G; European EGPA Study Group.
A. Bettiol et al.
Mepolizumab for Eosinophilic Granulomatosis With Polyangiitis: A European Multicenter Observational Study
Berti A, Atzeni F, Dagna L, Del Giacco S, Emmi G, Salvarani C, Vaglio A.
A. Berti et al.
Targeting the interleukin-5 pathway in EGPA: evidence, uncertainties and opportunities
Trivioli G, Marquez A, Martorana D, Tesi M, Kronbichler A, Lyons PA, Vaglio A.
G. Trivioli et al.
Genetics of ANCA-associated vasculitis: role in pathogenesis, classification and management
Bond M, Fagni F, Moretti M, Bello F, Egan A, Vaglio A, Emmi G, Dejaco C.
M. Bond et al.
At the Heart of Eosinophilic Granulomatosis with Polyangiitis: into Cardiac and Vascular Involvement.
Egan AC, Kronbichler A, Neumann I, Bettiol A, Carlson N, Cid MC, Emmi G, Gopaluni S, Harper L, Hauser T, Little MA, Luqmani RA, Mahr A, McClure M, Mohammad AJ, Nelveg-Kristensen KE, Ohlsson S, Peh CA, Rutherford M, Alamo BS, Scott J, Segelmark M, Smith RM, Szpirt WM, Tomasson G, Trivioli G, Vaglio A, Walsh M, Wester Trejo M, Westman K, Bajema IM, Jayne DRW.
AC. Egan et al.
The Sound of Interconnectivity; The European Vasculitis Society 2022 Report.
Bello F, Bettiol A, Silvestri E, Mattioli I, Urban ML, Palermo A, Mazzetti M, Malandrino D, Calcaterra I, Vaglio A, Di Minno MND, Emmi G, Prisco D.
F. Bello et al.
Evidence of subclinical atherosclerosis in eosinophilic granulomatosis with polyangiitis
Niccolai E, Bettiol A, Baldi S, Silvestri E, Di Gloria L, Bello F, Nannini G, Ricci F, Nicastro M, Ramazzotti M, Vaglio A, Bartolucci G, Emmi G, Amedei A, Prisco D.
E. Niccolai et al.
Gut Microbiota and Associated Mucosal Immune Response in Eosinophilic Granulomatosis with Polyangiitis (EGPA)
David C, Hamel Y, Smahi A, Diot E, Benhamou Y, Girszyn N, Le Gallou T, Lifermann F, Godmer P, Maurier F, Cottin V, Grados A, Aumaitre O, Néel A, Pugnet G, Masson C, Puéchal X, Mouthon L, Guillevin L, Bienvenu T, Terrier B; French Vasculitis Study Group.
E. Niccolai et al.
Identification of EPX Variants in Human Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss)
Delestre F, Brun AL, Thoreau B, Taillé C, Limal N, Puéchal X, Mouthon L, Guillevin L, Revel MP, Terrier B.
E. Niccolai et al.
Clinico-radiological correlation and prognostic value of baseline chest computed tomography in eosinophilic granulomatosis with polyangiitis
Rathmann J, Segelmark M, Englund M, Mohammad AJ.
E. Niccolai et al.
Stable incidence but increase in prevalence of ANCA-associated vasculitis in southern Sweden: a 23-year study
Emmi G, Bettiol A, Gelain E, Bajema IM, Berti A, Burns S, Cid MC, Tervaert Cohen JW, Cottin V, Durante E, Holle JU, Mahr AD, Martinez Del Pero M, Marvisi C, Mills J, Moiseev S, Moosig F, Mukhtyar C, Neumann T, Olivotto I, Salvarani C, Seeliger B, Sinico RA, Taillé C,
Terrier B, Venhoff N, Bertsias G, Guillevin L, Jayne DRW, Vaglio A.
E. Niccolai et al.
Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis
Rubenstein E, Maldini C, Vaglio A, Bello F, Bremer JP, Moosig F, Bottero P, Pesci A, Sinico RA, Grosskreutz J, Feder C, Saadoun D, Trivioli G, Maritati F, Rewerska B, Szczeklik W, Fraticelli P, Guida G, Gregorini G, Moroncini G, Hellmich B, Zwerina J, Resche-Rigon M, Emmi G, Neumann T, Mahr A.
E. Niccolai et al.
Cluster Analysis To Explore Clinical Subphenotypes Of Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss)
Bettiol A, Urban ML, Padoan R, Groh M, Lopalco G, Egan A, Cottin V, Fraticelli P, Crimi C, Del Giacco S, Losappio L, Moi L, Cinetto F, Caminati M, Novikov P, Berti A, Cameli P, Cathébras P, Coppola A, Durel CA, Folci M, Gullo AL, Lombardi C, Monti S, Parronchi P, Rivera CM, Solans R, Vacca A, Espígol-Frigolé G, Guarnieri G, Bianchi FC, Marchi MR, Tcherakian C, Kahn JE, Iannone F, Venerito V, Desaintjean C, Moroncini G, Nolasco S, Costanzo GAML, Schroeder JW, Ribi C, Tesi M, Gelain E, Mattioli I, Bello F, Jayne D, Prisco D, Vaglio A, Emmi G; European EGPA Study Group.
E. Niccolai et al.
Benralizumab for eosinophilic granulomatosis with polyangiitis: a retrospective, multicentre, cohort study
Cottu A, Groh M, Desaintjean C, Marchand-Adam S, Guillevin L, Puechal X, Beaumesnil S, Lazaro E, Samson M, Taille C, Durel CA, Diot E, Nicolas S, Guilleminault L, Ebbo M, Cathebras P, Dupin C, Yildiz H, Belfeki N, Pugnet G, Chauvin P, Jouneau S, Lifermann F, Martellosio JP, Cottin V, Terrier B; French Vasculitis Study Group.
E. Niccolai et al.