churg strauss syndrome


Summary: Widespread necrotizing angiitis with granulomas. Pulmonary involvement is frequent. Asthma or other respiratory infection may precede evidence of vasculitis. Eosinophilia and lung involvement differentiate this disease from POLYARTERITIS NODOSA.

Top Publications

  1. Noth I, Strek M, Leff A. Churg-Strauss syndrome. Lancet. 2003;361:587-94 pubmed
    ..We review the epidemiological evidence for an association of drug treatment with Churg-Strauss syndrome, the diverse diagnostic and pathological criteria for this syndrome, and treatment options. ..
  2. Pagnoux C, Guilpain P, Guillevin L. Churg-Strauss syndrome. Curr Opin Rheumatol. 2007;19:25-32 pubmed
    ..Furthermore, some patients may benefit from new biologic therapies under development, for example antiinterleukin-5 or antiimmunoglobulin E monoclonal antibodies. ..
  3. Hellmich B, Gross W. Recent progress in the pharmacotherapy of Churg-Strauss syndrome. Expert Opin Pharmacother. 2004;5:25-35 pubmed
    ..As relapses occur in > 25% of all patients, studies addressing the prevention of relapses in CSS are highly desirable in the future. ..
  4. Lau E, Carruthers D, Prasad N. Churg-Strauss syndrome as an unusual cause of spontaneous atraumatic intra-pericardial thrombosis. Eur J Echocardiogr. 2004;5:65-7 pubmed
    ..An unusual case of spontaneous atraumatic intra-pericardial thrombosis due to Churg-Strauss syndrome, which responded well to immunosuppressive therapy, is presented. ..
  5. Kallenberg C, Heeringa P, Stegeman C. Mechanisms of Disease: pathogenesis and treatment of ANCA-associated vasculitides. Nat Clin Pract Rheumatol. 2006;2:661-70 pubmed
    ..Several controlled trials have led to an evidence-based approach for the treatment of ANCA-associated vasculitis, and further studies, based on new insights into pathogenesis, are in progress. ..
  6. Keogh K, Specks U. Churg-Strauss syndrome: update on clinical, laboratory and therapeutic aspects. Sarcoidosis Vasc Diffuse Lung Dis. 2006;23:3-12 pubmed
    ..This review provides an update on the clinical diagnosis of Churg-Strauss syndrome in light of changing disease definitions and classifications, and focuses on evolving therapeutic approaches for this challenging systemic disorder. ..
  7. Agard C, Rendu E, Leguern V, Ponge T, Masseau A, Barrier J, et al. Churg-Strauss syndrome revealed by granulomatous acute pericarditis: two case reports and a review of the literature. Semin Arthritis Rheum. 2007;36:386-91 pubmed
    ..Whereas pericarditis with myocardial injury warrants immunosuppressive therapy, isolated pericarditis without other visceral involvement of poor prognosis only requires corticosteroid therapy. ..
  8. Petrakopoulou P, Franz W, Boekstegers P, Weis M. Vasospastic angina pectoris associated with Churg-Strauss syndrome. Nat Clin Pract Cardiovasc Med. 2005;2:484-9; quiz 490 pubmed
    ..Vasospastic angina pectoris associated with Churg-Strauss syndrome. Treatment with systemic corticosteroids and cyclophosphamide. ..
  9. Reumaux D, Duthilleul P, Roos D. Pathogenesis of diseases associated with antineutrophil cytoplasm autoantibodies. Hum Immunol. 2004;65:1-12 pubmed
    ..Mechanisms of neutrophil activation by ANCA include the engagement of Fcgamma receptors, the possible mechanisms of neutrophil-mediated tissue damage, and the neutrophil-endothelial interaction. ..

More Information


  1. Sinico R, Di Toma L, Maggiore U, Tosoni C, Bottero P, Sabadini E, et al. Renal involvement in Churg-Strauss syndrome. Am J Kidney Dis. 2006;47:770-9 pubmed
    ..The prevailing picture is ANCA-associated necrotizing crescentic glomerulonephritis; however, other forms of nephropathy also may occur. Outcome and long-term follow-up usually are good. ..
  2. Tsurikisawa N, Taniguchi M, Saito H, Himeno H, Ishibashi A, Suzuki S, et al. Treatment of Churg-Strauss syndrome with high-dose intravenous immunoglobulin. Ann Allergy Asthma Immunol. 2004;92:80-7 pubmed
    ..Patients with CSS who are resistant to corticosteroid treatment with or without cyclophosphamide may be treated effectively with IVIG therapy. ..
  3. Sablé Fourtassou R, Cohen P, Mahr A, Pagnoux C, Mouthon L, Jayne D, et al. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med. 2005;143:632-8 pubmed
    ..The association of ANCA positivity with clinical symptoms that indicate inflammation and necrosis of small vessels might characterize a predominantly vasculitic pattern of the Churg-Strauss syndrome. ..
  4. Bonaci Nikolic B, Nikolic M, Andrejevic S, Zoric S, Bukilica M. Antineutrophil cytoplasmic antibody (ANCA)-associated autoimmune diseases induced by antithyroid drugs: comparison with idiopathic ANCA vasculitides. Arthritis Res Ther. 2005;7:R1072-81 pubmed
    ..Different serological profiles could help in the differential diagnosis and adequate therapeutic approach to ANCA-positive ATD-treated patients with symptoms of systemic disease. ..
  5. Ramakrishna G, Midthun D. Churg-Strauss syndrome. Ann Allergy Asthma Immunol. 2001;86:603-13; quiz 13 pubmed
    ..Recognition of the multiorgan manifestations of CSS is crucial to clinical management. Whether a causal relationship exists between antileukotriene antagonists and onset of CSS remains unclear. ..
  6. Solans R, Bosch J, Pérez Bocanegra C, Selva A, Huguet P, Alijotas J, et al. Churg-Strauss syndrome: outcome and long-term follow-up of 32 patients. Rheumatology (Oxford). 2001;40:763-71 pubmed
    ..Outcome and long-term survival is usually good with steroids alone or in combination with immunosuppressive agents. The syndrome has a low mortality rate compared with other systemic vasculitides. ..
  7. McGavin C, Marshall A, Lewis C. Churg-Strauss syndrome with critical endomyocardial fibrosis: 10 year survival after combined surgical and medical management. Heart. 2002;87:E5 pubmed
    ..The good long term outcome is attributed to strict control of peripheral eosinophil count by oral corticosteroids. This case illustrates the damaging effects of hypereosinophilia on the heart. ..
  8. Gayraud M, Guillevin L, Le Toumelin P, Cohen P, Lhote F, Casassus P, et al. Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients. Arthritis Rheum. 2001;44:666-75 pubmed
    ..Based on these findings, we recommend that the intensity of the initial treatment be consistent with the severity of the disease. The use of the FFS and BVAS scores improved the ability to evaluate the therapeutic response. ..
  9. Choi Y, Im J, Han B, Kim J, Lee K, Myoung N. Thoracic manifestation of Churg-Strauss syndrome: radiologic and clinical findings. Chest. 2000;117:117-24 pubmed
  10. Della Rossa A, Baldini C, Tavoni A, Tognetti A, Neglia D, Sambuceti G, et al. Churg-Strauss syndrome: clinical and serological features of 19 patients from a single Italian centre. Rheumatology (Oxford). 2002;41:1286-94 pubmed
    ..The positive results of the treatment protocol used in this preliminary study deserve to be tested in controlled multicentre studies. ..
  11. Fujimoto S, Uezono S, Hisanaga S, Fukudome K, Kobayashi S, Suzuki K, et al. Incidence of ANCA-associated primary renal vasculitis in the Miyazaki Prefecture: the first population-based, retrospective, epidemiologic survey in Japan. Clin J Am Soc Nephrol. 2006;1:1016-22 pubmed
    ..The incidence of PRV did not differ between Japan and Europe, but WG was not widespread in Japan. Furthermore, the ratio of serum MPO to PR3-ANCA among Japanese with PRV was much higher than that found among European and US patients. ..
  12. Corradi D, Maestri R, Facchetti F. Postpartum Churg-Strauss syndrome with severe cardiac involvement: description of a case and review of the literature. Clin Rheumatol. 2009;28:739-43 pubmed publisher
  13. Ribi C, Cohen P, Pagnoux C, Mahr A, Arène J, Lauque D, et al. Treatment of Churg-Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized, open-label study of seventy-two patients. Arthritis Rheum. 2008;58:586-94 pubmed publisher
    ..AZA or pulse CYC was fairly effective in treating CS-resistant disease or major relapses. Over the long term, most patients continued to take oral CS, which might explain the high rate of CS-related adverse events. ..
  14. Schute L. [Complicated course of Churg-Strauss syndrome with eosinophilic perimyocarditis and pericardial tamponade. From DMW Nr. 41/2004]. Dtsch Med Wochenschr. 2005;130:176; author reply 176 pubmed
  15. Pecoraro A, Crescenzi L, Carucci L, Genovese A, Spadaro G. Heart failure not responsive to standard immunosuppressive therapy is successfully treated with high dose intravenous immunoglobulin therapy in a patient with Eosinophilic Granulomatosis with Polyangiitis (EGPA). Int Immunopharmacol. 2017;45:13-15 pubmed publisher
    ..Moreover, at a follow-up of 24months, the continuance of high dose IVIG therapy was required to maintain a sustained remission of the heart failure. ..
  16. Ishida K, Seki R, Inoue T, Iwamoto T, Hoshino M, Nakagawa T. [Clinical aspect of diffuse alveolar hemorrhage syndrome]. Nihon Kokyuki Gakkai Zasshi. 2003;41:851-6 pubmed
    ..Nine episodes (69%) had occurred as complications of primary diseases, four (31%) as the first symptoms of underlying diseases. Prognosis was poor in the former cases but in the latter, the prognosis was relatively favorable. ..
  17. Udono T, Abe T, Sato H, Tamai M. Bilateral central retinal artery occlusion in Churg-Strauss syndrome. Am J Ophthalmol. 2003;136:1181-3 pubmed
    ..Bilateral central retinal artery occlusion can occur in Churg-Strauss syndrome. Patients with this diagnosis should be carefully followed. ..
  18. Sevinc A, Hasanoglu H, Gokirmak M, Yildirim Z, Baysal T, Mizrak B. Allergic granulomatosis and angiitis in the absence of asthma and blood eosinophilia: a rare presentation of limited Churg-Strauss syndrome. Rheumatol Int. 2004;24:301-4 pubmed
    ..Histopathological examination of the open lung biopsy revealed granulomatous infiltration with histiocytes and eosinophilic leukocytes. This extremely rare variant of CSS is discussed. ..
  19. Harrold L, Andrade S, Eisner M, Buist A, Go A, Vollmer W, et al. Identification of patients with Churg-Strauss syndrome (CSS) using automated data. Pharmacoepidemiol Drug Saf. 2004;13:661-7 pubmed
    ..Automated claims data can be used to identify patients with CSS. This approach can facilitate better epidemiologic study of the risk factors for the condition. ..
  20. Fruguglietti M, Napoli L, Sciacco M, Ripolone M, Serafini M, Grimoldi N, et al. Severe acute multineuropathy in Churg-Strauss syndrome in a patient with a history of melanoma. Clin Neuropathol. 2009;28:125-8 pubmed
    ..Muscle biopsy showed neurogenic changes and one vessel surrounded by mononuclear cells. After a few days of corticosteroid therapy leukocytosis and eosinophilia normalized and the patient's clinical features stabilized. ..
  21. Tanizawa K, Kaji Y, Tanaka E, Inoue T, Sakuramoto M, Minakuchi M, et al. Massive eosinophilic pleural effusion preceding vasculitic symptoms in Churg-Strauss syndrome. Intern Med. 2010;49:841-5 pubmed
    ..CSS is rare as a cause of EPE, but should be included in the differential diagnosis of EPE in asthmatic patients. This is the first report showing that EPE can precede other vasculitic symptoms in CSS. ..
  22. Assaf C, Mewis G, Orfanos C, Geilen C. Churg-Strauss syndrome: successful treatment with mycophenolate mofetil. Br J Dermatol. 2004;150:598-600 pubmed
  23. Pavone L, Grasselli C, Chierici E, Maggiore U, Garini G, Ronda N, et al. Outcome and prognostic factors during the course of primary small-vessel vasculitides. J Rheumatol. 2006;33:1299-306 pubmed
    ..Patients with cerebral, hepatic, and renal involvement have the poorest prognosis for survival. Our data do not show that the prognostic relevance of these factors depends on the form of PSVV. ..
  24. Naschitz J, Loberman Z, Wolffson V, Babich J, Yeshurun D. Gangrene of the feet in Churg-Strauss syndrome. Vasc Med. 2003;8:129-30 pubmed
  25. Guillevin L, Pagnoux C. [Treatment of systemic vasculitides]. Rev Prat. 2008;58:541-4 pubmed
    ..The treatment duration of non-infectious vasculitides is typically 18 to 24 months. The improvement in prognosis is due to the treatment as well as to anti-infectious prophylaxis and careful management of potential complications. ..
  26. Oh M, Lee J, Kwon N, Choi D. Churg-Strauss syndrome: the clinical features and long-term follow-up of 17 patients. J Korean Med Sci. 2006;21:265-71 pubmed
    ..CSS was highly variable in its presentation and course. The manifestations may range from mild symptoms to life-threatening conditions. The outcome after long-term follow-up was as good as that of previous studies. ..
  27. Bluett R, McDonnell D, O Dowling C, Vaughan C. Eosinophilic myocarditis as a first presentation of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). BMJ Case Rep. 2017;2017: pubmed publisher
    ..Testing for FIP1L1-PDGFRA and other BCR-ABL1 mutations was negative. Ultimately, he was diagnosed with eosinophilic granulomatosis with polyangiitis, also known as Churg-Strauss syndrome. ..
  28. Narula N, Gupta S, Narula J. The primary vasculitides: a clinicopathologic correlation. Am J Clin Pathol. 2005;124 Suppl:S84-95 pubmed
    ..Incorporation of antineutrophil cytoplasmic antibodies in defining the pathogenesis of vasculitis has been particularly useful in diagnosing those small vessel vasculitides that are life threatening and need immediate intervention. ..
  29. Wolf M, Rose H, Smith R. Case records of the Massachusetts General Hospital. Case 28-2005. A 42-year-old man with weight loss, weakness, and a rash. N Engl J Med. 2005;353:1148-57 pubmed
  30. Partal A, Moshfeghi D, Alcorn D. Churg-Strauss syndrome in a child: retina and optic nerve findings. Br J Ophthalmol. 2004;88:971-2 pubmed
  31. Kawakami T, Soma Y, Hosaka E, Mizoguchi M. Churg-Strauss syndrome with cutaneous and neurological manifestations preceding asthma. Acta Derm Venereol. 2006;86:67-8 pubmed
  32. Mukherjee B, Chir B, Moon J, Sandrasagra M, Pennell D. Endomyocardial fibrosis in Churg-Strauss syndrome. Clin Cardiol. 2004;27:21 pubmed
  33. Todd D, Cockcroft D. Prolonged survival in Churg-Strauss syndrome. Ann Allergy Asthma Immunol. 2004;92:92-3 pubmed
  34. Chao C, Hsieh S, Shun C, Hsieh S. Skin denervation and cutaneous vasculitis in eosinophilia-associated neuropathy. Arch Neurol. 2007;64:959-65 pubmed
    ..Skin denervation with cutaneous vasculitis is a major manifestation of eosinophilia-associated neuropathy. ..
  35. Skrapari I, Kagkelari E, Charitatos E, Pantelidaki C, Gounaris T, Sioula E. Acute painless monocular visual loss due to central retinal artery occlusion in a patient with Churg-Strauss vasculitis. Clin Rheumatol. 2008;27:125-7 pubmed
    ..Acute blindness in CSS has been rarely described. Even more rarely, central retinal artery occlusion has been found to be the underlying cause of this infrequent clinical manifestation in CSS...
  36. Załeska M, Roszkowska Sliz B, Wiatr E, Tomkowski W, Bestry I, Roszkowski Sliz K. [Churg-Strauss syndrome--cardiac problem in lung disease department]. Pneumonol Alergol Pol. 2003;71:349-58 pubmed
    ..But echocardiographic picture of the heart was nor better during 2 months of observation. ..
  37. Bochner B, Gleich G. What targeting eosinophils has taught us about their role in diseases. J Allergy Clin Immunol. 2010;126:16-25; quiz 26-7 pubmed publisher
    ..This review summarizes what has been learned in vivo in both preclinical and clinical studies of eosinophil-directed therapies, with an emphasis on recent advances. ..
  38. Sinico R, Sabadini E, Maresca A. Mesalazine-induced Churg-Strauss syndrome in a patient with Crohn's disease and sclerosing cholangitis. Clin Exp Rheumatol. 2006;24:S104 pubmed
  39. Kluger N, Pagnoux C, Guillevin L, Frances C. Comparison of cutaneous manifestations in systemic polyarteritis nodosa and microscopic polyangiitis. Br J Dermatol. 2008;159:615-20 pubmed publisher
    ..Mononeuritis multiplex was associated with skin lesions in the MPA group (P < 0.05). The clinical or histological analysis of cutaneous lesions is not helpful for distinguishing PAN from MPA. ..
  40. Twardowsky A, Paz J, Pastorino A, Jacob C, Marques Dias M, Silva C. Chorea in a child with Churg-Strauss syndrome. Acta Reumatol Port. 2010;35:72-5 pubmed
    ..A rare case of CSS with chorea was reported, reinforcing the possibility of this disease in children with asthma, allergic rhinitis, hypereosinophilia and cutaneous vasculitis. ..
  41. Yoneda T, Katayama N, Kimura H, Fujimura M. [A case of Churg-Strauss syndrome with methylprednisolone sodium succinate allergy]. Nihon Kokyuki Gakkai Zasshi. 2009;47:1147-50 pubmed
    ..Since a challenge test using an intravenous infusion of methylprednisolone sodium succinate worsened the lower limb pain, fever and peripheral blood eosinophilia, a drug allergy was diagnosed together with Churg-Strauss syndrome. ..
  42. Cabral D, Uribe A, Benseler S, O Neil K, Hashkes P, Higgins G, et al. Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood. Arthritis Rheum. 2009;60:3413-24 pubmed publisher
    ..Diagnostic delays may result from poor characterization of childhood WG. Initial therapy varied considerably among participating centers. ..
  43. Holl Ulrich K. [Histopathology of systemic vasculitis]. Pathologe. 2010;31:67-76 pubmed publisher
    ..A conclusive morphological diagnosis depends on the right time of biopsy and selection of appropriate biopsy material. ..
  44. Vignaux O, Marmursztejn J, Cohen P, Bruguière E, Duboc D, Guillevin L, et al. [Cardiac imaging in ANCA-associated vasculitis]. Presse Med. 2007;36:902-6 pubmed
  45. Vega S J, Duclos H J. [Late reactivation of Churg-Strauss syndrome]. Rev Med Chil. 2006;134:79-84 pubmed
    ..Eight months after being on dialysis, he suffered a reactivation of the disease with lung hemorrhage and finally died, due to an upper gastrointestinal bleeding caused by a duodenal ulcer. ..
  46. Tanaka M, Mise N, Kurita N, Suzuki T, Hara K, Fujii A, et al. A case of Churg-Strauss syndrome with necrotizing crescentic glomerulonephritis accompanied by acute coronary syndrome due to vasospasm. Am J Kidney Dis. 2010;56:e5-9 pubmed publisher
    ..Slight abnormalities in urine sediment can be the clue to the diagnosis of severe kidney involvement of Churg-Strauss syndrome. ..
  47. Langford C. Vasculitis in the geriatric population. Rheum Dis Clin North Am. 2007;33:177-95 pubmed
    ..A thorough understanding of the vasculitic disease entities that may affect older patients as well as their diagnosis and management is essential in minimizing disease and treatment-related morbidity and mortality. ..
  48. d Ersu E, Ribi C, Monney P, Vincenti G, Schwitter J, Rotman S, et al. Churg-Strauss syndrome with cardiac involvement: case illustration and contribution of CMR in the diagnosis and clinical follow-up. Int J Cardiol. 2018;258:321-324 pubmed publisher
  49. Eisenberger U, Hess C. [Systemic ANCA-associated vasculitis--diagnosis and therapy]. Ther Umsch. 2008;65:295-301 pubmed publisher
    ..Thus, therapeutic efficacy must be outweighed against long-term toxicity to make the right choice for therapeutic intervention in ANCA-associated vasculitis. ..
  50. Brzosko I, Brzosko M, Ostanek L, Przepiera Bedzak H, Grabarek J. [Churg-Strauss syndrome--report of four cases]. Pol Arch Med Wewn. 2005;113:352-8 pubmed
    ..They were treated with combination of systemic corticosteroids and cyclophosphamide, one patient was additionally treated with plasmapheresis. In all four cases complete remission of the disease was achieved. ..
  51. Vaglio A, Martorana D, Maggiore U, Grasselli C, Zanetti A, Pesci A, et al. HLA-DRB4 as a genetic risk factor for Churg-Strauss syndrome. Arthritis Rheum. 2007;56:3159-66 pubmed
    ..001). These findings indicate that HLA-DRB4 is a genetic risk factor for the development of CSS and increases the likelihood of development of vasculitic manifestations of the disease. ..
  52. Kim Y, Lee K, Chung M, Han J, Chong S, Chung M, et al. Pulmonary involvement in Churg-Strauss syndrome: an analysis of CT, clinical, and pathologic findings. Eur Radiol. 2007;17:3157-65 pubmed
    ..This classification helps predict PFT data, underlying histopathology, and treatment response. ..