eMedicine Specialties > Neurology > Pediatric Neurology

Churg-Strauss Disease: Follow-up

Author: Robert Rust Jr, MD, Thomas E Worrell Jr Professor of Epileptology and Neurology, Co-Director of FE Dreifuss Child Neurology and Epilepsy Clinics, University of Virginia School; Clinical and Residency Training, Child Neurology, University of Virginia Hospital and Clinics
Contributor Information and Disclosures

Updated: Sep 28, 2006

Follow-up

Further Inpatient Care

  • Patients hospitalized for treatment of Churg-Strauss disease should be assessed, as should any hospitalized patient, for the risk of deep vein thrombosis, pulmonary embolus and, if cardiac dysfunction has been noted, cardiogenic embolus.
  • Treatment with steroids entails risks for gastrointestinal hemorrhage, electrolyte disturbance, and infection, which also must be considered. When steroids are administered, antacids or appropriate histamine-blocking agents should be administered to reduce the risk for gastrointestinal hemorrhage.

Further Outpatient Care

  • Administration of antacids or histamine-blocking agents to reduce the risk for gastrointestinal hemorrhage should be continued as long as patients are administered oral corticosteroids.
  • Long-term corticosteroid administration entails risk for electrolyte disturbances, infections, and fractures because of diminished bone mass. These risks must be reassessed continually during the course of therapy.
  • Alternative steroid-sparing anti-inflammatory therapies, selected from among the options noted in the treatment section, should be considered in patients requiring long-term corticosteroid therapy.

Deterrence/Prevention

  • A number of authorities believe that the administration of cysteinyl leukotriene-receptor antagonists for the treatment of asthma may provoke development of Churg-Strauss disease in some individuals. The use of inhaled steroids, rather than cysteinyl leukotriene-receptor antagonists during the taper phase of steroid treatment of an acute exacerbation of asthma may be a valuable alternative for avoiding this potentially provocative circumstance.

Complications

  • Particularly characteristic complications of the second phase of Churg-Strauss disease are chronic eosinophilic pneumonia and eosinophilic gastroenteritis.
  • Abdominal pain, diarrhea, gastrointestinal bleeding, and bowel perforation are important complications.
  • Raynaud phenomenon, arthralgias, or joint effusions are occasional complications.

Prognosis

  • Once an appropriate therapeutic intervention is undertaken, a good response usually is achieved within 4 weeks. Thereafter, the disease usually can be well controlled with low maintenance steroid doses. With modern therapy, the outlook for Churg-Strauss disease appears to be much better than in early reports. More than 90% of patients achieve remission after initial steroid treatment. Whether the apparently improved outlook, as compared to earlier reports, represents a change in the average severity of disease, improved recognition and diagnosis of milder cases, or improvements in therapy is not well understood. Patients demonstrating a favorable response usually retain an independent existence on steroid maintenance therapy. The relapse rate is approximately 25-30%.
  • Characteristically, patients with severe systemic vasculitis have a poor response to the initial phases of treatment. Both systolic and diastolic dysfunction associated with cardiomyopathy may improve with steroid therapy, although very low myocardial shortening fractions may not improve with this therapy. The outlook for patients with severe systemic vasculitis is quite guarded, since they have a considerable risk for dependent existence and progressive decline or premature death. The overall mortality rate may be as high as 25% within 5 years of diagnosis, half of these patients dying directly from vasculitis and half from secondary complications of vasculitis. The patients at highest risk for death or severe morbidity are those with severe myocardial or gastrointestinal vasculitis.

Miscellaneous

Medicolegal Pitfalls

  • These do not differ in any significant way from other areas of practice in medicine.
 


More on Churg-Strauss Disease

Overview: Churg-Strauss Disease
Differential Diagnoses & Workup: Churg-Strauss Disease
Treatment & Medication: Churg-Strauss Disease
Follow-up: Churg-Strauss Disease
References
[ CLOSE WINDOW ]

References

  1. Abril A, Calamia KT, Cohen MD. The Churg Strauss syndrome (allergic granulomatous angiitis): review and update. Semin Arthritis Rheum. Oct 2003;33(2):106-14. [Medline].

  2. Azzopardi C, Montefort S, Mallia C. Cardiac involvement and left ventricular failure in a patient with the Churg-Strauss syndrome. Adv Exp Med Biol. 1999;455:547-9. [Medline].

  3. Barry C, Davis S, Garrard P, Ferguson IT. Churg-Strauss disease: deterioration in a twin pregnancy. Successful outcome following treatment with corticosteroids and cyclophosphamide. Br J Obstet Gynaecol. Jun 1997;104(6):746-7. [Medline].

  4. Berger JR, Wei T, Wilson D. Idiopathic granulomatous angiitis of the CNS manifesting as diffuse white matter disease [letter; comment]. Neurology. Dec 1998;51(6):1774-5. [Medline].

  5. Bili A, Condemi JJ, Bottone SM, Ryan CK. Seven cases of complete and incomplete forms of Churg-Strauss syndrome not related to leukotriene receptor antagonists. J Allergy Clin Immunol. Nov 1999;104(5):1060-5. [Medline].

  6. Boggi U, Mosca M, Giulianotti PC, et al. Surviving catastrophic gastrointestinal involvement due to Churg-Strauss syndrome: report of a case. Hepatogastroenterology. Jul-Aug 1997;44(16):1169-71. [Medline].

  7. Booth AD, Almond MK, Burns A, et al. Outcome of ANCA-associated renal vasculitis: a 5-year retrospective study. Am J Kidney Dis. Apr 2003;41(4):776-84. [Medline].

  8. Brooklyn TN, Prouse P, Portmann B, Ramage JK. Churg-Strauss syndrome and granulomatous cholangiopathy. Eur J Gastroenterol Hepatol. Jul 2000;12(7):809-11. [Medline].

  9. Bruce IN, Bell AL. A comparison of two nomenclature systems for primary systemic vasculitis. Br J Rheumatol. Apr 1997;36(4):453-8. [Medline].

  10. Burke AP, Sobin LH, Virmani R. Localized vasculitis of the gastrointestinal tract. Am J Surg Pathol. Mar 1995;19(3):338-49. [Medline].

  11. Caballero J, Zayas R, Arana R, et al. [Churg-Strauss syndrome with pericardial and myocardial involvement]. Rev Esp Cardiol. Sep 1999;52(9):745-7. [Medline].

  12. Chen KR, Su WP, Pittelkow MR, Leiferman KM. Eosinophilic vasculitis syndrome: recurrent cutaneous eosinophilic necrotizing vasculitis. Semin Dermatol. Jun 1995;14(2):106-10. [Medline].

  13. Cho KH, Kim YG, Yang SG, et al. Inflammatory nodules of the lower legs: a clinical and histological analysis of 134 cases in Korea. J Dermatol. Aug 1997;24(8):522-9. [Medline].

  14. Chumbley LC, Harrison EG Jr, DeRemee RA. Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Report and analysis of 30 cases. Mayo Clin Proc. Aug 1977;52(8):477-84. [Medline].

  15. Churg A, Brallas M, Cronin SR, Churg J. Formes frustes of Churg-Strauss syndrome. Chest. Aug 1995;108(2):320-3. [Medline].

  16. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol. Mar-Apr 1951;27(2):277-301. [Medline].

  17. Cohen P, Guillevin L, Baril L, et al. Persistence of antineutrophil cytoplasmic antibodies (ANCA) in asymptomatic patients with systemic polyarteritis nodosa or Churg-Strauss syndrome: follow-up of 53 patients. Clin Exp Rheumatol. Mar-Apr 1995;13(2):193-8. [Medline].

  18. Davis MD, Daoud MS, McEvoy MT, Su WP. Cutaneous manifestations of Churg-Strauss syndrome: a clinicopathologic correlation. J Am Acad Dermatol. Aug 1997;37(2 Pt 1):199-203. [Medline].

  19. Dietz A, Hubner C, Andrassy K. [Macrolide antibiotic-induced vasculitis (Churg-Strauss syndrome)]. Laryngorhinootologie. Feb 1998;77(2):111-4. [Medline].

  20. Dillon MJ. Childhood vasculitis. Lupus. 1998;7(4):259-65. [Medline].

  21. Dinc A, Soy M, Pay S, et al. A case of Churg-Strauss syndrome presenting with cortical blindness. Clin Rheumatol. 2000;19(4):318-20. [Medline].

  22. Eustace JA, Nadasdy T, Choi M. Disease of the month. The Churg-Strauss Syndrome. J Am Soc Nephrol. Sep 1999;10(9):2048-55. [Medline].

  23. Falk RJ, Nachman PH, Hogan SL, Jennette JC. ANCA glomerulonephritis and vasculitis: a Chapel Hill perspective. Semin Nephrol. May 2000;20(3):233-43. [Medline].

  24. Ferro JM. Vasculitis of the central nervous system. J Neurol. Dec 1998;245(12):766-76. [Medline].

  25. Gross WL, Csernok E. Immunodiagnostic and pathophysiologic aspects of antineutrophil cytoplasmic antibodies in vasculitis. Curr Opin Rheumatol. Jan 1995;7(1):11-9. [Medline].

  26. Guillevin L, Fain O, Lhote F, et al. Lack of superiority of steroids plus plasma exchange to steroids alone in the treatment of polyarteritis nodosa and Churg-Strauss syndrome. A prospective, randomized trial in 78 patients. Arthritis Rheum. Feb 1992;35(2):208-15. [Medline].

  27. Hagen EC, Andrassy K, Csernok E, et al. Development and standardization of solid phase assays for the detection of anti-neutrophil cytoplasmic antibodies (ANCA). A report on the second phase of an international cooperative study on the standardization of ANCA assays. J Immunol Methods. Sep 13 1996;196(1):1-15. [Medline].

  28. Hauschild S, Csernok E, Schmitt WH, Gross WL. Antineutrophil cytoplasmic antibodies in systemic polyarteritis nodosa with and without hepatitis B virus infection and Churg-Strauss syndrome--62 patients. J Rheumatol. Jun 1994;21(6):1173-4. [Medline].

  29. Hellemans S, Dens J, Knockaert D. Coronary involvement in the Churg-Strauss syndrome. Heart. Jun 1997;77(6):576-8. [Medline].

  30. Hellmich B, Gross WL. Recent progress in the pharmacotherapy of Churg-Strauss syndrome. Expert Opin Pharmacother. Jan 2004;5(1):25-35. [Medline].

  31. Hellmich B, Csernok E, Gross WL. Proinflammatory cytokines and autoimmunity in churg-strauss syndrome. Ann N Y Acad Sci. Jun 2005;1051:121-31. [Medline].

  32. Hellmich B, Ehlers S, Csernok E, Gross WL. Update on the pathogenesis of Churg-Strauss syndrome. Clin Exp Rheumatol. Nov-Dec 2003;21(6 Suppl 32):S69-77. [Medline].

  33. Hoffman PM, Godfrey T, Stawell RJ. A case of Churg-Strauss syndrome with visual loss following central retinal artery occlusion. Lupus. 2005;14(2):174-5. [Medline].

  34. Jennette JC, Wilkman AS, Falk RJ. Anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis and vasculitis. Am J Pathol. Nov 1989;135(5):921-30. [Medline].

  35. Kawakami T, Soma Y, Kawasaki K, et al. Initial cutaneous manifestations consistent with mononeuropathy multiplex in Churg-Strauss syndrome. Arch Dermatol. Jul 2005;141(7):873-8. [Medline].

  36. Kim Y, Lee KS, Choi DC, et al. The spectrum of eosinophilic lung disease: radiologic findings. J Comput Assist Tomogr. Nov-Dec 1997;21(6):920-30. [Medline].

  37. Klemmer PJ, Chalermskulrat W, Reif MS, et al. Plasmapheresis therapy for diffuse alveolar hemorrhage in patients with small-vessel vasculitis. Am J Kidney Dis. Dec 2003;42(6):1149-53. [Medline].

  38. Lamprecht P. TNF-alpha inhibitors in systemic vasculitides and connective tissue diseases. Autoimmun Rev. Jan 2005;4(1):28-34. [Medline].

  39. Lamprecht P. Off balance: T-cells in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Clin Exp Immunol. Aug 2005;141(2):201-10. [Medline].

  40. Lane SE, Watts R, Scott DG. Epidemiology of systemic vasculitis. Curr Rheumatol Rep. Aug 2005;7(4):270-5. [Medline].

  41. Langford CA, Sneller MC. New developments in the treatment of Wegener''s granulomatosis, polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome. Curr Opin Rheumatol. Jan 1997;9(1):26-30. [Medline].

  42. Lanham JG. Churg-Strauss syndrome. Br J Hosp Med. May 6-19 1992;47(9):667-73. [Medline].

  43. Lhote F, Cohen P, Genereau T, et al. Microscopic polyangiitis: clinical aspects and treatment. Ann Med Interne (Paris). 1996;147(3):165-77. [Medline].

  44. Lie JT. Histopathologic specificity of systemic vasculitis. Rheum Dis Clin North Am. Nov 1995;21(4):883-909. [Medline].

  45. Lie JT. Nomenclature and classification of vasculitis: plus ca change, plus c''est la meme chose [editorial]. Arthritis Rheum. Feb 1994;37(2):181-6. [Medline].

  46. Martin-Suarez I, D'Cruz D, Mansoor M, et al. Immunosuppressive treatment in severe connective tissue diseases: effects of low dose intravenous cyclophosphamide. Ann Rheum Dis. Aug 1997;56(8):481-7. [Medline].

  47. Matsuo K, Tomioka T, Tajima Y, et al. Allergic granulomatous angitis (Churg-Strauss syndrome) with multiple intestinal fistulas. Am J Gastroenterol. Oct 1997;92(10):1937-8. [Medline].

  48. Metzler C, Lamprecht P, Hellmich B, et al. Leucoencephalopathy after treatment of Churg-Strauss syndrome with interferon {alpha}. Ann Rheum Dis. Aug 2005;64(8):1242-3. [Medline].

  49. Muschen M, Warskulat U, Perniok A, et al. Involvement of soluble CD95 in Churg-Strauss syndrome. Am J Pathol. Sep 1999;155(3):915-25. [Medline].

  50. Niles JL. Antineutrophil cytoplasmic antibodies in the classification of vasculitis. Annu Rev Med. 1996;47:303-13. [Medline].

  51. Pellissier JF, Figarella-Branger D, Serratrice G. [Neuromuscular diseases with eosinophilia]. Med Trop (Mars). 1998;58(4 Suppl):471-6. [Medline].

  52. Primack SL, Muller NL. Radiologic manifestations of the systemic autoimmune diseases. Clin Chest Med. Dec 1998;19(4):573-86, vii. [Medline].

  53. Rutgers A, Heeringa P, Tervaert JW. The role of myeloperoxidase in the pathogenesis of systemic vasculitis. Clin Exp Rheumatol. Nov-Dec 2003;21(6 Suppl 32):S55-63. [Medline].

  54. Sable-Fourtassou R, Cohen P, Mahr A, et al. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med. Nov 1 2005;143(9):632-8. [Medline].

  55. Sakamoto S, Ohba S, Eguchi K, et al. Churg-Strauss syndrome presenting with subarachnoid hemorrhage from ruptured dissecting aneurysm of the intracranial vertebral artery. Clin Neurol Neurosurg. Aug 2005;107(5):428-31. [Medline].

  56. Sanders JS, Stegeman CA, Kallenberg CG. The Th1 and Th2 paradigm in ANCA-associated vasculitis. Kidney Blood Press Res. 2003;26(4):215-20. [Medline].

  57. Sharma BK, Daga MK, Sharma M. A limited form of Churg-Strauss syndrome presenting without asthma and eosinophilia. Med J Aust. Nov 1 2004;181(9):498-9. [Medline].

  58. Somogyi A, Muzes G, Molnar J, Tulassay Z. Drug-related Churg-Strauss syndrome?. Adverse Drug React Toxicol Rev. Jun-Sep 1998;17(2-3):63-74. [Medline].

  59. Sonneville R, Lagrange M, Guidoux C, et al. [The association of cardiac involvement and ischemic stroke in Churg Strauss syndrome]. Rev Neurol (Paris). Feb 2006;162(2):229-32. [Medline].

  60. Steinfeld S, Golstein M, De Vuyst P. Chronic eosinophilic pneumonia (CEP) as a presenting feature of Churg-Strauss syndrome (CSS) [letter; comment]. Eur Respir J. Nov 1994;7(11):2098. [Medline].

  61. Sudo K, Tashiro K. Idiopathic granulomatous angiitis of the CNS manifesting as diffuse white matter disease [letter; comment]. Neurology. Dec 1998;51(6):1774; discussion 1775. [Medline].

  62. Sullivan EJ, Hoffman GS. Pulmonary vasculitis. Clin Chest Med. Dec 1998;19(4):759-76, ix. [Medline].

  63. Tatsis E, Schnabel A, Gross WL. Interferon-alpha treatment of four patients with the Churg-Strauss syndrome. Ann Intern Med. Sep 1 1998;129(5):370-4. [Medline].

  64. Terasaki F, Hayashi T, Hirota Y, et al. Evolution to dilated cardiomyopathy from acute eosinophilic pancarditis in Churg-Strauss syndrome. Heart Vessels. 1997;12(1):43-8. [Medline].

  65. Thuy GN, Cuguilliere A, Arteaga C, et al. [Idiopathic chronic eosinophilic pneumonia]. Med Trop (Mars). 1998;58(4 Suppl):455-8. [Medline].

  66. Vital C, Vital A, Canron MH, et al. Combined nerve and muscle biopsy in the diagnosis of vasculitic neuropathy. A 16-year retrospective study of 202 cases. J Peripher Nerv Syst. Mar 2006;11(1):20-9. [Medline].

  67. Watts RA, Lane SE, Bentham G, Scott DG. Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom. Arthritis Rheum. Feb 2000;43(2):414-9. [Medline].

  68. Watts RA, Scott DG. Classification and epidemiology of the vasculitides. Baillieres Clin Rheumatol. May 1997;11(2):191-217. [Medline].

  69. Weissler JC. Syndromes of severe asthma. Am J Med Sci. Mar 2000;319(3):166-76. [Medline].

  70. Wolf M, Rose H, Smith RN. 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. Sep 15 2005;353(11):1148-57. [Medline].

  71. Yoshihara K, Arimura Y, Kobayashi O, et al. [Clinical study on five myeloperoxidase specific anti-neutrophil cytoplasmic antibody (MPO-ANCA) positive Churg-Strauss syndrome cases]. Ryumachi. Oct 1998;38(5):696-704. [Medline].

  72. Zhao MH, Short AK, Lockwood CM. Antineutrophil cytoplasm autoantibodies and vasculitis. Curr Opin Hematol. Jan 1995;2(1):96-102. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

syndrome of allergic granulomatosis and angiitis, allergic angiitis and granulomatosis, allergic granulomatosis, Churg-Strauss syndrome, CSS, Churg Strauss disease, allergic granulomatous angiitis, eosinophilic angiitis, eosinophilic granulomatosis

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Robert Rust Jr, MD, Thomas E Worrell Jr Professor of Epileptology and Neurology, Co-Director of FE Dreifuss Child Neurology and Epilepsy Clinics, University of Virginia School; Clinical and Residency Training, Child Neurology, University of Virginia Hospital and Clinics
Robert Rust Jr, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, American Headache Society, American Neurological Association, Child Neurology Society, International Child Neurology Association, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Medical Editor

Robert Baumann, MD, Program Director, Professor, Departments of Neurology and Pediatrics, University of Kentucky
Robert Baumann, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American College of Epidemiology, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Kenneth J Mack, MD, PhD, Senior Associate Consultant, Department of Child and Adolescent Neurology, Mayo Clinic
Kenneth J Mack, MD, PhD is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, Phi Beta Kappa, and Society for Neuroscience
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.