eMedicine Specialties > Neurology > Pediatric Neurology

Churg-Strauss Disease: Treatment & Medication

Author: Robert Stanley Rust Jr, MD, MA, Thomas E Worrell Jr Professor of Epileptology and Neurology, Co-Director of FE Dreifuss Child Neurology and Epilepsy Clinics, Director, Child Neurology, University of Virginia; Chair-Elect, Child Neurology Section, American Academy of Neurology
Contributor Information and Disclosures

Updated: Jan 20, 2010

Treatment

Medical Care

Medical management of cardiovascular, cardiac, renal, and gastrointestinal complications of Churg-Strauss disease falls under the purview of subspecialty consultation. Drugs used in the treatment of Churg-Strauss disease are described in Medication.

Surgical Care

  • Surgical procedures in patients with Churg-Strauss disease most commonly entail biopsy of affected tissues.
  • In addition, surgical intervention may be indicated in patients who experience catastrophic complications, such as acute abdomen or intracranial hemorrhage.

Consultations

  • Consultations for patients with Churg-Strauss disease depend on the manifestations of the disease. The cardiopulmonary manifestations typically are most important. Consultations with the following may be required:
    • Pulmonologist
    • Cardiologist
    • Rheumatologist
    • Gastroenterologist
    • Urologist

Diet

No dietary factors are clearly known to assist in the management of Churg-Strauss disease, except as are related to the management of congestive heart failure, renal failure, or gastrointestinal complications. The management of these factors falls under the purview of subspecialists involved in the care of these organ systems.

Activity

No clear evidence suggests that particular activities influence the course of the illness once it is established. Environmental exposures and medical treatment of asthma that may influence the chances for development of Churg-Strauss disease are discussed in Causes.

Medication

For most patients, Churg-Strauss disease (CSD) is a readily treatable illness, and reports over the past few decades have shown better outcomes than were demonstrated in earlier case series. In part, this may be due to the inclusion of milder cases due to improved recognition. In particular, diagnostic sensitivity has been greatest for individuals whose initial presentation is with asthma (90% of cases in some case series). However, a major factor has been the availability of corticosteroids. Milder CSD may respond well to orally administered corticosteroids.

The recommended initial medications for treatment of severe manifestations of CSD, including patients with CSD–related peripheral neuritis, are corticosteroids, which are administered at high doses. Intravenous administration of methylprednisolone at doses of 15 mg/kg on 1-3 successive mornings is one of the most common initial approaches to severe cases. Rapid correction of eosinophilia, leukocytosis, and elevations of sedimentation rate and LDH are characteristic of CSD. Failure to provoke such corrections early in the course of therapy is associated with elevated risk for poor long-term outcome.

Intravenous treatment is followed by oral prednisone at a dose of approximately 1 mg/kg/d (usual, but absolute maximal daily dose should not exceed 80 mg/d), with ensuing taper once clinical improvement is noted.

Many patients with CSD manifest a favorable response to this monotherapeutic approach within a few days; however, in many cases, persistence of asthma prevents oral prednisone from being tapered to doses lower than 10-15 mg/d. In milder cases, initial treatment can be undertaken with the administration of oral corticosteroids at doses of 1 mg/kg/d (60 mg/d is the usual but not absolute maximal dose).

Corticosteroid treatment, whether oral or intravenous, has been combined with plasma exchange or plasmapheresis for cases that were difficult to treat. This combination appears to have conferred benefit in some patients. Some patients have demonstrated marked clinical improvement, accompanied by declining circulating pANCA titers, after treatment with intravenous immunoglobulin (IVIg). Some patients have been treated, either initially or during a subsequent phase of therapy, with the combination of daily oral prednisone and cyclophosphamide. This approach may enhance disease control and may have a sparing effect upon steroid dosage, thus diminishing steroid-related adverse effects. Prednisone taper in patients responding to the combined therapy can be undertaken after approximately 2 weeks.

The combination of high-dose corticosteroids and dapsone has been used in patients with severe Churg-Strauss disease and has proven effective in instances of Churg-Strauss myocarditis.27 Corticosteroid doses may be reduced after improvement in myocarditis is achieved.

Cyclophosphamide treatment (titrated to the neutrophil count) generally is continued for 6-12 months after remission is established. Pulse intravenous cyclophosphamide therapy in combination with corticosteroids appears to diminish the risk for various adverse effects seen in patients receiving oral cyclophosphamide daily. This form of therapy is also considered in patients whose disease responds poorly to corticosteroids. Dose, frequency, and total number of cyclophosphamide pulses are adjusted to disease response, blood counts, and renal function. Efficacy of this form of therapy is not, as yet, fully established.

Usually, collaborating with physicians specializing in renal medicine is the best way to undertake this form of therapy. Protocols must be utilized to ensure that renal function is preserved with regard to additionally administered medications and hydration. These protocols entail intense hydration and coadministration of 2-mercaptoethanesulfonate (mesna). Some studies have used initial pulse intravenous cyclophosphamide at doses as high as 0.6 g/m2 of body surface, but this dose must be reduced in accordance with the degree of impairment of renal function exhibited by the patient.

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. The use of thalidomide is contraindicated in women of childbearing age. None of the drugs noted in this paragraph should be used without the collaboration of subspecialists skilled in their use and familiar with the relative indications, dosage adjustments, potential benefits, and adverse effects. Therefore, none of these agents are reviewed in the following Medication section because the complex issues entailed with their use fall beyond the scope of this article.

Corticosteroids

These medications decrease the activity of the immune system in inflammatory reactions. The immune system is of critical importance in the pathophysiology of this disease.


Methylprednisolone (Medrol, Solu-Medrol, Depo-Medrol)

Moderate or severe cases often treated for 1-3 d with IV methylprednisolone (or equivalent dose of some other anti-inflammatory corticosteroid). Administer initial dose under close supervision, since rare instances of anaphylaxis after initial dose have been reported.

Adult

15 mg/kg IV, administered as single early morning dose for 1-3 d at onset of treatment

Pediatric

Administer as in adults

Phenytoin, phenobarbital, ephedrine, or rifampin may enhance clearance, lowering anticipated serum levels; may result in unpredictable change in response to warfarin, usual effect is decrease in anticoagulant effect, necessitating, in some instances, upward adjustment of dose based upon careful determination of PT; potassium-depleting diuretics may increase risk for hypokalemia; may increase requirements for oral hypoglycemic agents or insulin in patients with diabetes mellitus

Documented hypersensitivity; systemic fungal infection; some, but not all, patients receiving amphotericin B; concomitant cerebral malaria; latent or active amoebiasis; active chickenpox, measles; most patients with active tuberculosis; many patients with recent myocardial infarction; most patients with ulcerative colitis, active or latent peptic ulcer disease, impending GI perforation, or enteric abscess

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

May interfere with ascertainment of presence or location of infections; may interfere with ability of treated patients to contain and eliminate infectious pathogens; may cause electrolyte disturbances and may worsen congestive heart failure or hypertension in susceptible patients; may result in muscle weakness, loss of muscle mass, osteoporosis, vertebral compression fractures, aseptic necrosis of femoral heads, pathological fractures of long bones, tendon rupture, pancreatitis, ulcerative esophagitis, impaired wound healing, increased sweating, convulsions, pseudotumor cerebri, glaucoma, subcapsular cataracts, vertigo, headache, confusion or psychosis, menstrual irregularities, suppression of adrenocortical axis, expression of latent diabetes mellitus, or hirsutism; breastfeeding should be curtailed because these compounds appear in breastmilk and may result in growth suppression or any of the other potential complications noted above in feeding child


Prednisone (Sterapred)

Useful in initial management of mild cases (especially for asthma) and in taper and maintenance phases of therapy for Churg-Strauss disease.

Adult

1 mg/kg/d PO; not to exceed 60-80 mg/d

Pediatric

Administer as in adults

Phenytoin, phenobarbital, ephedrine, or rifampin may enhance clearance, lowering anticipated serum levels; may result in unpredictable change in response to warfarin, usual effect is decrease in anticoagulant effect, necessitating, in some instances, upward adjustment of dose based upon careful determination of PT; potassium-depleting diuretics may increase risk for hypokalemia; may increase requirements for oral hypoglycemic agents or insulin in patients with diabetes mellitus

Documented hypersensitivity; systemic fungal infection; some, but not all, patients receiving amphotericin B; concomitant cerebral malaria; latent or active amoebiasis; active chickenpox, measles; most patients with active tuberculosis; many patients with recent myocardial infarction; most patients with ulcerative colitis, active or latent peptic ulcer disease, impending GI perforation, or enteric abscess

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

May interfere with ascertainment of presence or location of infections; may interfere with ability of treated patients to contain and eliminate infectious pathogens; may cause electrolyte disturbances and may worsen congestive heart failure or hypertension in susceptible patients; may result in muscle weakness, loss of muscle mass, osteoporosis, vertebral compression fractures, aseptic necrosis of femoral heads, pathological fractures of long bones, tendon rupture, pancreatitis, ulcerative esophagitis, impaired wound healing, increased sweating, convulsions, pseudotumor cerebri, glaucoma, subcapsular cataracts, vertigo, headache, confusion or psychosis, menstrual irregularities, suppression of adrenocortical axis, expression of latent diabetes mellitus, or hirsutism; breastfeeding should be curtailed because these compounds appear in breastmilk and may result in growth suppression or any of the other potential complications noted above in feeding child

Cytotoxic agents

These agents inhibit cell growth and proliferation, reducing the activity of the immune system.


Cyclophosphamide (Cytoxan)

Synthetic drug, chemically related to nitrogen mustards, developed as antineoplastic agent. Biotransformed in liver, where constituent alkylating metabolites activated. These activated compounds interfere with growth of susceptible rapidly proliferating cells. Mechanism of action with regard to tumor cells may involve cross-linking of tumor cell DNA.

Adult

Pulse PO/IV therapy for patients with Churg-Strauss disease should be undertaken in collaboration with nephrologists, rheumatologists, or oncologists familiar with use of this agent and upon whom obligation to establish safe dosage and appropriate therapeutic plan should be placed

Pediatric

Administer as in adults

High doses of phenobarbital may increase rate of metabolism and leukopenic activity; may potentiate effect of succinylcholine chloride due to persistent inhibition of cholinesterase activity; anesthetic agents should be used with care in patients who have received cyclophosphamide within preceding 10 d

Documented hypersensitivity; severely depressed bone marrow function

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

In addition to those already noted, carefully follow hematologic profile—especially neutrophils and platelets—as well as urinalysis for presence of RBCs, which may indicate development of hemorrhagic cystitis; review of these procedures and management of fluids intended to prevent toxicity to urinary system fall beyond scope of this article and should be considered in consultation with physicians familiar with use of cyclophosphamide; has potential cardiac toxicity; suppression of immune response may expose patients to risk of serious infections; dosage may require adjustment in adrenalectomized patients; secondary malignancies have developed in patients treated with cyclophosphamide; known mutagenic effects render this drug unsafe for use in pregnancy; may have untoward effects on breastfeeding infant whose mother is treated with cyclophosphamide; may cause temporary or permanent sterility of patients (both men and women)

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. Davies DJ, Moran JE. Segmental necrotizing glomerulonephritis with antineutrophil antibody: possible arbovirus aetiology?. BMJ. 1982;285:606.

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

  3. 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].

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

  5. Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. Aug 1990;33(8):1094-100. [Medline].

  6. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. Feb 1994;37(2):187-92. [Medline].

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

  8. Kallenberg CG, Brouwer E, Weening JJ, Tervaert JW. Anti-neutrophil cytoplasmic antibodies: current diagnostic and pathophysiological potential. Kidney Int. Jul 1994;46(1):1-15. [Medline].

  9. Cohen P, Guillevin L, Baril L, Lhote F, Noel LH, Lesavre P. 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].

  10. Tervaert JW, Goldschmeding R, Elema JD, von dem Borne AE, Kallenberg CG. Antimyeloperoxidase antibodies in the Churg-Strauss syndrome. Thorax. Jan 1991;46(1):70-1. [Medline].

  11. Tervaert JW, Goldschmeding R, Elema JD, et al. Association of autoantibodies to myeloperoxidase with different forms of vasculitis. Arthritis Rheum. Aug 1990;33(8):1264-72. [Medline].

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

  13. Capizzi SA, Specks U. Does infection play a role in the pathogenesis of pulmonary vasculitis?. Semin Respir Infect. Mar 2003;18(1):17-22. [Medline].

  14. Wiik A. Autoantibodies in vasculitis. Arthritis Res Ther. 2003;5(3):147-52. [Medline].

  15. Arm JP, Lee TH. Sulphidopeptide leukotrienes in asthma. Clin Sci (Lond). May 1993;84(5):501-10. [Medline].

  16. Drazen JM, Israel E. Should antileukotriene therapies be used instead of inhaled corticosteroids in asthma? Yes. Am J Respir Crit Care Med. Dec 1998;158(6):1697-8. [Medline].

  17. Drazen JM, Israel E, O'Byrne PM. Treatment of asthma with drugs modifying the leukotriene pathway. N Engl J Med. Jan 21 1999;340(3):197-206. [Medline].

  18. Wechsler ME, Garpestad E, Flier SR, et al. Pulmonary infiltrates, eosinophilia, and cardiomyopathy following corticosteroid withdrawal in patients with asthma receiving zafirlukast. JAMA. Feb 11 1998;279(6):455-7. [Medline].

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

  20. D'Cruz DP, Barnes NC, Lockwood CM. Difficult asthma or Churg-Strauss syndrome?. BMJ. Feb 20 1999;318(7182):475-6. [Medline].

  21. Garcia-Marcos L, Schuster A, Perez-Yarza EG. Benefit-risk assessment of antileukotrienes in the management of asthma. Drug Saf. 2003;26(7):483-518. [Medline].

  22. Kemp JP. Recent advances in the management of asthma using leukotriene modifiers. Am J Respir Med. 2003;2(2):139-56. [Medline].

  23. Finkel TH, Hunter DJ, Paisley JE, Finkel RS, Larsen GL. Drug-induced lupus in a child after treatment with zafirlukast (Accolate). J Allergy Clin Immunol. Mar 1999;103(3 Pt 1):533-4. [Medline].

  24. Haim DY, Lippmann ML, Goldberg SK, Walkenstein MD. The pulmonary complications of crack cocaine. A comprehensive review. Chest. Jan 1995;107(1):233-40. [Medline].

  25. Orriols R, Munoz X, Ferrer J, Huget P, Morell F. Cocaine-induced Churg-Strauss vasculitis. Eur Respir J. Jan 1996;9(1):175-7. [Medline].

  26. Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore). Jan 1999;78(1):26-37. [Medline].

  27. Shanks M, Ignaszewski AP, Chan SY, Allard MF. Churg-Strauss syndrome with myocarditis manifesting as acute myocardial infarction with cardiogenic shock: case report and review of the literature. Can J Cardiol. Sep 2003;19(10):1184-8. [Medline].

  28. Tatsukawa H, Nagano S, Umeno Y, Oribe M. Churg-strauss syndrome with cholecystitis and renal involvement. Intern Med. Sep 2003;42(9):893-6. [Medline].

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

  30. 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].

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

  32. 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].

  33. 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].

  34. 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].

  35. 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].

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

  37. 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].

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

  39. 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].

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

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

  42. Caballero J, Zayas R, Arana R, Cano L, Berruezo A, Pinero C. [Churg-Strauss syndrome with pericardial and myocardial involvement]. Rev Esp Cardiol. Sep 1999;52(9):745-7. [Medline].

  43. 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].

  44. Cho KH, Kim YG, Yang SG, Lee DY, Chung JH. 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].

  45. 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].

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

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

  48. Dinç A, Soy M, Pay S, Simsek I, Erdem H, Sobaci G. A case of Churg-Strauss syndrome presenting with cortical blindness. Clin Rheumatol. 2000;19(4):318-20. [Medline].

  49. 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].

  50. 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].

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

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

  53. 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].

  54. 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].

  55. 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].

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

  57. 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].

  58. 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].

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

  60. 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].

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

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

  63. Klemmer PJ, Chalermskulrat W, Reif MS, Hogan SL, Henke DC, Falk RJ. Plasmapheresis therapy for diffuse alveolar hemorrhage in patients with small-vessel vasculitis. Am J Kidney Dis. Dec 2003;42(6):1149-53. [Medline].

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

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

  66. 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].

  67. Lhote F, Cohen P, Genereau T, Gayraud M, Guillevin L. Microscopic polyangiitis: clinical aspects and treatment. Ann Med Interne (Paris). 1996;147(3):165-77. [Medline].

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

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

  70. 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].

  71. Metzler C, Lamprecht P, Hellmich B, Reuter M, Arlt AC, Gross WL. Leucoencephalopathy after treatment of Churg-Strauss syndrome with interferon {alpha}. Ann Rheum Dis. Aug 2005;64(8):1242-3. [Medline].

  72. 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].

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

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

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

  76. Reid AJC, Harrison RA, Watkin SW, McCann BG, and Scott DGI. Churg-Strauss syndrome in a district hospital. Q J Med. 1998;91:219-229.

  77. 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.

  78. 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].

  79. 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].

  80. 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].

  81. 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].

  82. 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].

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

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

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

  86. 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].

  87. 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].

  88. Thuy GN, Cuguilliere A, Arteaga C, Miltgen J, Bonnet D. [Idiopathic chronic eosinophilic pneumonia]. Med Trop (Mars). 1998;58(4 Suppl):455-8. [Medline].

  89. 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].

  90. 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].

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

  92. 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].

  93. 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].

  94. 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

Churg-Strauss vasculitis, Churg-Strauss disease symptoms, Churg-Strauss disease causes, Churg-Strauss disease treatment, syndrome of allergic granulomatosis and angiitis, allergic angiitis and granulomatosis, allergic granulomatosis, Churg-Strauss syndrome, allergic granulomatous angiitis, eosinophilic angiitis, eosinophilic granulomatosis

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Robert Stanley Rust Jr, MD, MA, Thomas E Worrell Jr Professor of Epileptology and Neurology, Co-Director of FE Dreifuss Child Neurology and Epilepsy Clinics, Director, Child Neurology, University of Virginia; Chair-Elect, Child Neurology Section, American Academy of Neurology
Robert Stanley Rust Jr, MD, MA 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, Child Neurology 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, and Child Neurology Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

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

Amy Kao, MD, Assistant Professor, Department of Pediatrics, Division of Pediatric Neurology, Department of Neurology, Oregon Health and Science University; Consulting Staff, Shriners Hospital for Children
Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society
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.