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Semin Respir Crit Care Med 2006; 27(2): 148-157
DOI: 10.1055/s-2006-939518
Copyright © 2006 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

Churg-Strauss Syndrome

Karina A. Keogh1 , Ulrich Specks1
  • 1Division of Pulmonary and Critical Care Medicine, Department of Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota
Further Information

Publication History

Publication Date:
13 April 2006 (online)

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ABSTRACT

Churg-Strauss syndrome was originally called “allergic granulomatosis and angiitis,” describing the combination of eosinophilic inflammation, extravascular granulomas, and necrotizing vasculitis occurring in patients with severe asthma. It is now classified as a small-vessel vasculitis and, together with Wegener's granulomatosis and microscopic polyangiitis, as one of the vasculitides associated with antineutrophil cytoplasmic autoantibodies (ANCA). Glucocorticoid-sparing agents used in the treatment of asthma, such as leukotriene receptor antagonists, may unmask this particular form of vasculitis as oral glucocorticoids are withdrawn. ANCA occur in 40-75% of patients with active disease and typically react with myeloperoxidase. Patients' symptoms are defined by various degrees of eosinophilic inflammation and necrotizing vasculitis, which may affect any organ. On presentation, Churg-Strauss syndrome needs to be differentiated from other eosinophilic pneumonias, idiopathic hypereosinophilic syndrome, and Wegener's granulomatosis and microscopic polyangiitis. Churg-Strauss syndrome remains a rare disease with a poorly understood pathogenesis. Treatment consists primarily of glucocortoids. Patients who have ANCA at the time of presentation should be treated according to the treatment principles for ANCA-associated vasculitides. However, the exact role of glucocorticoid-sparing immunosuppressive agents and treatment options for refractory disease remain poorly studied.

KEYWORDS

Churg-Strauss syndrome - vasculitis - asthma - eosinophilia - ANCA - leukotriene receptor antagonists

REFERENCES

  • 1 Churg J, Strauss L. Allergic granulomatosis, allergic angiitis and periarteritis nodosa.  Am J Pathol. 1951;  27 277-301
    PubMedGoogle Scholar
  • 2 Jennette J C, Falk R J, Andrassy K et al.. Nomenclature of systemic vasculitides: The proposal of an international consensus conference.  Arthritis Rheum. 1994;  37 187-192
    CrossrefPubMedGoogle Scholar
  • 3 Lanham J G, Elkon K B, Pusey C D, Hughes G R. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome.  Medicine. 1984;  63 65-81
    CrossrefPubMedGoogle Scholar
  • 4 Sorensen S F, Slot O, Tvede N, Petersen J. A prospective study of vasculitis patients collected in a five year period: evaluation of the Chapel Hill nomenclature.  Ann Rheum Dis. 2000;  59 478-482
    PubMedGoogle Scholar
  • 5 Keogh K A, Specks U. Churg-Strauss syndrome. Clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists.  Am J Med. 2003;  115 284-290
    CrossrefPubMedGoogle Scholar
  • 6 Masi A T, Hunder G G, Lie J T et al.. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis).  Arthritis Rheum. 1990;  33 1094-1100
    CrossrefPubMedGoogle Scholar
  • 7 Chumbley L C, Harrison E G, DeRemee R A. Allergic granulomatosis and angiitis (Churg-Strauss syndrome).  Mayo Clin Proc. 1977;  52 477-484
    PubMedGoogle Scholar
  • 8 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. 1999;  78 26-37
    CrossrefPubMedGoogle Scholar
  • 9 Lie J T. Limited forms of Churg-Strauss syndrome.  Pathol Annu. 1993;  28(Pt 2) 199-220
    PubMedGoogle Scholar
  • 10 Churg A, Brallas M, Cronin S R, Churg J. Formes frustes of Churg-Strauss syndrome.  Chest. 1995;  108 320-323
    PubMedGoogle Scholar
  • 11 Churg A. Recent advances in the diagnosis of Churg-Strauss syndrome.  Mod Pathol. 2001;  14 1284-1293
    CrossrefPubMedGoogle Scholar
  • 12 Watts R A, Carruthers D M, Scott D GI. Epidemiology of systemic vasculitis: changing incidence or definition?.  Semin Arthritis Rheum. 1995;  25 28-34
    CrossrefPubMedGoogle Scholar
  • 13 Watts R A, Lane S E, Bentham G, Scott D G. Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom.  Arthritis Rheum. 2000;  43 414-419
    CrossrefPubMedGoogle Scholar
  • 14 Gonzalez-Gay M A, Garcia-Porrua C, Guerrero J, Rodriguez-Ledo P, Llorca J. The epidemiology of the primary systemic vasculitides in northwest Spain: implications of the Chapel Hill Consensus Conference definitions.  Arthritis Rheum. 2003;  49 388-393
    CrossrefPubMedGoogle Scholar
  • 15 Mahr A, Guillevin L, Poissonnet M, Ayme S. Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate.  Arthritis Rheum. 2004;  51 92-99
    CrossrefPubMedGoogle Scholar
  • 16 Reinhold-Keller E, Herlyn K, Wagner-Bastmeyer R, Gross W L. Stable incidence of primary systemic vasculitides over five years: results from the German vasculitis register.  Arthritis Rheum. 2005;  53 93-99
    CrossrefPubMedGoogle Scholar
  • 17 Loughlin J E, Cole J A, Rothman K J, Johnson E S. Prevalence of serious eosinophilia and incidence of Churg-Strauss syndrome in a cohort of asthma patients.  Ann Allergy Asthma Immunol. 2002;  88 319-325
    PubMedGoogle Scholar
  • 18 Harrold L R, Andrade S E, Go A S et al.. Incidence of Churg-Strauss syndrome in asthma drug users: a population-based perspective.  J Rheumatol. 2005;  32 1076-1080
    PubMedGoogle Scholar
  • 19 Reid A J, Harrison B DW, Watts R A, Watkin S W, McCann B G, Scott D GI. Churg-Strauss syndrome in a district hospital.  QJM. 1998;  91 219-229
    PubMedGoogle Scholar
  • 20 Solans R, Bosch J A, Perez-Bocanegra C et al.. Churg-Strauss syndrome: outcome and long-term follow-up of 32 patients.  Rheumatology (Oxford). 2001;  40 763-771
    CrossrefPubMedGoogle Scholar
  • 21 Sable-Fourtassou R, Cohen P, Mahr A et al.. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome.  Ann Intern Med. 2005;  143 632-638
    PubMedGoogle Scholar
  • 22 Worthy S A, Muller N L, Hansell D M, Flower C D. Churg-Strauss syndrome: the spectrum of pulmonary CT findings in 17 patients.  AJR Am J Roentgenol. 1998;  170 297-300
    CrossrefPubMedGoogle Scholar
  • 23 Silva C I, Muller N L, Fujimoto K, Johkoh T, Ajzen S A, Churg A. Churg-Strauss syndrome: high resolution CT and pathologic findings.  J Thorac Imaging. 2005;  20 74-80
    CrossrefPubMedGoogle Scholar
  • 24 Choi Y H, Im J G, Han B K, Kim J H, Lee K Y, Myoung N H. Thoracic manifestation of Churg-Strauss syndrome: radiologic and clinical findings.  Chest. 2000;  117 117-124
    CrossrefPubMedGoogle Scholar
  • 25 Sinico R A, Di Toma L, Maggiore U et al.. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome.  Arthritis Rheum. 2005;  52 2926-2935
    CrossrefPubMedGoogle Scholar
  • 26 Lesens O, Hansmann Y, Nerson J et al.. Severe Churg-Strauss syndrome with mediastinal lymphadenopathy treated with interferon therapy.  Eur J Intern Med. 2002;  13 458
    PubMedGoogle Scholar
  • 27 Sehgal M, Swanson J W, DeRemee R A, Colby T V. Neurologic manifestations of Churg-Strauss syndrome.  Mayo Clin Proc. 1995;  70 337-341
    PubMedGoogle Scholar
  • 28 Davis M D, Daoud M S, McEvoy M T, Su W P. Cutaneous manifestations of Churg-Strauss syndrome: a clinicopathologic correlation.  J Am Acad Dermatol. 1997;  37 199-203
    CrossrefPubMedGoogle Scholar
  • 29 Finan M C, Winkelmann R K. The cutaneous extravascular necrotizing granuloma (Churg-Strauss granuloma) and systemic disease: a review of 27 cases.  Medicine (Baltimore). 1983;  62 142-158
    PubMedGoogle Scholar
  • 30 Guillevin L, Lhote F, Gayraud M et al.. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome.  Medicine. 1996;  75 17-28
    CrossrefPubMedGoogle Scholar
  • 31 Morgan J M, Raposo L, Gibson D G. Cardiac involvement in Churg-Strauss syndrome shown by echocardiography.  Br Heart J. 1989;  62 462-466
    CrossrefPubMedGoogle Scholar
  • 32 Ramakrishna G, Connolly H M, Tazelaar H D, Mullany C J, Midthun D E. Churg-Strauss syndrome complicated by eosinophilic endomyocarditis.  Mayo Clin Proc. 2000;  75 631-635
    CrossrefPubMedGoogle Scholar
  • 33 Val-Bernal J F, Mayorga M, Garcia-Alberdi E, Pozueta J A. Churg-Strauss syndrome and sudden cardiac death.  Cardiovasc Pathol. 2003;  12 94-97
    CrossrefPubMedGoogle Scholar
  • 34 Stollberger C, Finsterer J, Winkler W B. Eosinophilic pericardial effusion in Churg-Strauss syndrome.  Respir Med. 2005;  99 377-379
    PubMedGoogle Scholar
  • 35 Hasley P B, Follansbee W P, Coulehan J L. Cardiac manifestations of Churg-Strauss syndrome: report of a case and review of the literature.  Am Heart J. 1990;  120 996-999
    CrossrefPubMedGoogle Scholar
  • 36 Hellemans S, Dens J, Knockaert D. Coronary involvement in the Churg-Strauss syndrome.  Heart. 1997;  77 576-578
    PubMedGoogle Scholar
  • 37 Kozak M, Gill E A, Green L S. The Churg-Strauss syndrome. A case report with angiographically documented coronary involvement and a review of the literature.  Chest. 1995;  107 578-580
    PubMedGoogle Scholar
  • 38 Roufosse F, Cogan E, Goldman M. The hypereosinophilic syndrome revisited.  Annu Rev Med. 2003;  54 169-184
    CrossrefPubMedGoogle Scholar
  • 39 Peen E, Hahn P, Lauwers G, Williams Jr R C, Gleich G, Kephart G M. Churg-Strauss syndrome: localization of eosinophil major basic protein in damaged tissues.  Arthritis Rheum. 2000;  43 1897-1900
    CrossrefPubMedGoogle Scholar
  • 40 Memain N, De B M, Guillevin L, Wechsler B, Meyer O. Delayed relapse of Churg-Strauss syndrome manifesting as colon ulcers with mucosal granulomas: 3 cases.  J Rheumatol. 2002;  29 388-391
    PubMedGoogle Scholar
  • 41 Pagnoux C, Mahr A, Cohen P, Guillevin L. Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, Wegener granulomatosis, Churg-Strauss syndrome, or rheumatoid arthritis-associated vasculitis.  Medicine (Baltimore). 2005;  84 115-128
    CrossrefPubMedGoogle Scholar
  • 42 Clutterbuck E J, Evans D J, Pusey C D. Renal involvement in Churg-Strauss syndrome.  Nephrol Dial Transplant. 1990;  5 161-167
    PubMedGoogle Scholar
  • 43 Kikuchi Y, Ikehata N, Tajima O, Yoshizawa N, Miura S. Glomerular lesions in patients with Churg-Strauss syndrome and the anti- myeloperoxidase antibody.  Clin Nephrol. 2001;  55 429-435
    PubMedGoogle Scholar
  • 44 Ames P R, Roes L, Lupoli S, Brancaccio V, Khamashta M A, Hughes G R. Thrombosis in Churg-Strauss syndrome. Beyond vasculitis?.  Br J Rheumatol. 1996;  35 1181-1183
    CrossrefPubMedGoogle Scholar
  • 45 Garcia G, Achouh L, Cobarzan D, Fichet D, Humbert M. Severe venous thromboembolic disease in Churg-Strauss syndrome.  Allergy. 2005;  60 409-410
    CrossrefPubMedGoogle Scholar
  • 46 Merkel P A, Lo G H, Holbrook J T et al.. High incidence of venous thrombotic events among patients with Wegener granulomatosis: the Wegener's Clinical Occurrence of Thrombosis (WeCLOT) Study.  Ann Intern Med. 2005;  142 620-626
    CrossrefPubMedGoogle Scholar
  • 47 Wallaert B, Gosset P, Prin L, Bart F, Marquette C H, Tonnel A B. Bronchoalveolar lavage in allergic granulomatosis and angiitis.  Eur Respir J. 1993;  6 413-417
    PubMedGoogle Scholar
  • 48 Schnabel A, Csernok E, Braun J, Gross W L. Inflammatory cells and cellular activation in the lower respiratory tract in Churg-Strauss syndrome.  Thorax. 1999;  54 771-778
    PubMedGoogle Scholar
  • 49 Kallenberg C G. Churg-Strauss syndrome: just one disease entity?.  Arthritis Rheum. 2005;  52 2589-2593
    CrossrefPubMedGoogle Scholar
  • 50 Schmitt W H, Csernok E, Kobayashi S, Klinkenborg A, Reinhold-Keller E, Gross W L. Churg-Strauss syndrome. Serum markers of lymphocyte activation and endothelial damage.  Arthritis Rheum. 1998;  41 445-452
    CrossrefPubMedGoogle Scholar
  • 51 Hurst S, Chizzolini C, Dayer J M, Olivieri J, Roux-Lombard P. Usefulness of serum eosinophil cationic protein (ECP) in predicting relapse of Churg and Strauss vasculitis.  Clin Exp Rheumatol. 2000;  18 784-785
    PubMedGoogle Scholar
  • 52 Cottin V, Tardy F, Gindre D, Vernet G, Deviller P, Cordier J F. Urinary eosinophil-derived neurotoxin in Churg-Strauss syndrome.  J Allergy Clin Immunol. 1995;  96 261-264
    CrossrefPubMedGoogle Scholar
  • 53 Kurosawa M, Nakagami R, Morioka J et al.. Interleukins in Churg-Strauss syndrome.  Allergy. 2000;  55 785-787
    CrossrefPubMedGoogle Scholar
  • 54 Drage L A, Davis M D, De Castro F et al.. Evidence for pathogenic involvement of eosinophils and neutrophilsin Churg-Strauss syndrome.  J Am Acad Dermatol. 2002;  47 209-216
    CrossrefPubMedGoogle Scholar
  • 55 Kiene M, Csernok E, Muller A, Metzler C, Trabandt A, Gross W L. Elevated interleukin-4 and interleukin-13 production by T cell lines from patients with Churg-Strauss syndrome.  Arthritis Rheum. 2001;  44 469-473
    CrossrefPubMedGoogle Scholar
  • 56 Schonermarck U, Csernok E, Trabandt A, Hansen H, Gross W L. Circulating cytokines and soluble CD23, CD26 and CD30 in ANCA- associated vasculitides.  Clin Exp Rheumatol. 2000;  18 457-463
    PubMedGoogle Scholar
  • 57 Muschen M, Warskulat U, Perniok A et al.. Involvement of soluble CD95 in Churg-Strauss syndrome.  Am J Pathol. 1999;  155 915-925
    CrossrefPubMedGoogle Scholar
  • 58 Guillevin L, Amouroux J, Arbeille B, Boura R. Churg-Strauss angiitis. Arguments favoring the responsibility of inhaled antigens.  Chest. 1991;  100 1472-1473
    CrossrefPubMedGoogle Scholar
  • 59 Cohen Tervaert J W, Kallenberg C GM. Anti-myeloperoxidase antibodies in Churg-Strauss syndrome.  J Neurol. 1993;  240 449-452
    PubMedGoogle Scholar
  • 60 Savage C O, Harper L, Holland M. New findings in pathogenesis of antineutrophil cytoplasm antibody-associated vasculitis.  Curr Opin Rheumatol. 2002;  14 15-22
    CrossrefPubMedGoogle Scholar
  • 61 Specks U. Antineutrophil cytoplasmic antibodies: are they pathogenic?.  Clin Exp Rheumatol. 2004;  22 S7-12
    PubMedGoogle Scholar
  • 62 Xiao H, Heeringa P, Hu P et al.. Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice.  J Clin Invest. 2002;  110 955-963
    CrossrefPubMedGoogle Scholar
  • 63 Pfister H, Ollert M, Frohlich L F et al.. Antineutrophil cytoplasmic autoantibodies against the murine homolog of proteinase 3 (Wegener autoantigen) are pathogenic in vivo.  Blood. 2004;  104 1411-1418
    CrossrefPubMedGoogle Scholar
  • 64 Little M A, Smyth C L, Yadav R et al.. Anti-neutrophil cytoplasm antibodies directed against myeloperoxidase augment leukocyte-microvascular interactions in vivo.  Blood. 2005;  106 2050-2058
    CrossrefPubMedGoogle Scholar
  • 65 Knoell D L, Lucas J, Allen J N. Churg-Strauss syndrome associated with Zafirlukast.  Chest. 1998;  114 332-334
    PubMedGoogle Scholar
  • 66 Wechsler M E, Garpestad E, Flier S R et al.. Pulmonary infiltrates, eosinophilia, and cardiomyopathy following corticosteroid withdrawal in patients with asthma receiving zafirlukast.  JAMA. 1998;  279 455-457
    CrossrefPubMedGoogle Scholar
  • 67 Wechsler M E, Finn D, Gunawardena D et al.. Churg-Strauss syndrome in patients receiving montelukast as treatment for asthma.  Chest. 2000;  117 708-713
    CrossrefPubMedGoogle Scholar
  • 68 Tuggey J M, Hosker H S. Churg-Strauss syndrome associated with montelukast therapy.  Thorax. 2000;  55 805-806
    CrossrefPubMedGoogle Scholar
  • 69 Hashimoto M, Fujishima T, Tanaka H et al.. Churg-Strauss syndrome after reduction of inhaled corticosteroid in a patient treated with pranlukast for asthma.  Intern Med. 2001;  40 432-434
    PubMedGoogle Scholar
  • 70 Guilpain P, Viallard J F, Lagarde P et al.. Churg-Strauss syndrome in two patients receiving montelukast.  Rheumatology (Oxford). 2002;  41 535-539
    CrossrefPubMedGoogle Scholar
  • 71 Solans R, Bosch J A, Selva A, Orriols R, Vilardell M. Montelukast and Churg-Strauss syndrome.  Thorax. 2002;  57 183-185
    CrossrefPubMedGoogle Scholar
  • 72 Weller P F, Plaut M, Taggart V, Trontell A. The relationship of asthma therapy and Churg-Strauss syndrome: NIH workshop summary report.  J Allergy Clin Immunol. 2001;  108 175-183
    CrossrefPubMedGoogle Scholar
  • 73 Masuzawa A, Moriguchi M, Tsuda T et al.. Churg-Strauss syndrome associated with hypersensitivity to acetaminophen.  Intern Med. 2005;  44 496-498
    CrossrefPubMedGoogle Scholar
  • 74 Hübner C, Dietz A, Stremmel W, Stiehl A, Andrassy K. Macrolide-induced Churg-Strauss syndrome in a patient with atopy.  Lancet. 1997;  350 563
    PubMedGoogle Scholar
  • 75 Orriols R, Munoz X, Ferrer J, Huget P, Morell F. Cocaine-induced Churg-Strauss vasculitis.  Eur Respir J. 1996;  9 175-177
    CrossrefPubMedGoogle Scholar
  • 76 Le Gall C, Pham S, Vignes S et al.. Inhaled corticosteroids and Churg-Strauss syndrome: a report of five cases.  Eur Respir J. 2000;  15 978-981
    CrossrefPubMedGoogle Scholar
  • 77 Sheffer A L, Rocklin R E, Goetzl E J. Immunologic components of hypersensitivity reactions to cromolyn sodium.  N Engl J Med. 1975;  293 1220-1224
    PubMedGoogle Scholar
  • 78 Bili A, Condemi J J, Bottone S M, Ryan C K. Seven cases of complete and incomplete forms of Churg-Strauss syndrome not related to leukotriene receptor antagonists.  J Allergy Clin Immunol. 1999;  104 1060-1065
    PubMedGoogle Scholar
  • 79 Lilly C M, Churg A, Lazarovich M et al.. Asthma therapies and Churg-Strauss syndrome.  J Allergy Clin Immunol. 2002;  109 S1-19
    CrossrefPubMedGoogle Scholar
  • 80 Langford C A, Klippel J H, Balow J E, James S P, Sneller M C. Use of cytotoxic agents and cyclosporine in the treatment of autoimmune disease. Part 2: inflammatory bowel disease, systemic vasculitis, and therapeutic toxicity.  Ann Intern Med. 1998;  129 49-58
    PubMedGoogle Scholar
  • 81 Gayraud M, Guillevin L, Cohen P et al.. Treatment of good-prognosis polyarteritis nodosa and Churg-Strauss syndrome: comparison of steroids and oral or pulse cyclophosphamide in 25 patients. French Cooperative Study Group for Vasculitides.  Br J Rheumatol. 1997;  36 1290-1297
    CrossrefPubMedGoogle Scholar
  • 82 Gayraud M, Guillevin L, le Toumelin 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;  44 666-675
    CrossrefPubMedGoogle Scholar
  • 83 Metzler C, Hellmich B, Gause A, Gross W L, de Groot K. Churg Strauss syndrome-successful induction of remission with methotrexate and unexpected high cardiac and pulmonary relapse ratio during maintenance treatment.  Clin Exp Rheumatol. 2004;  22 S52-S61
    PubMedGoogle Scholar
  • 84 Della Rossa A, Baldini C, Tavoni A et al.. Churg-Strauss syndrome: clinical and serological features of 19 patients from a single Italian centre.  Rheumatology (Oxford). 2002;  41 1286-1294
    CrossrefPubMedGoogle Scholar
  • 85 Assaf C, Mewis G, Orfanos C E, Geilen C C. Churg-Strauss syndrome: successful treatment with mycophenolate mofetil.  Br J Dermatol. 2004;  150 598-600
    CrossrefPubMedGoogle Scholar
  • 86 Tatsis E, Schnabel A, Gross W L. Interferon-a treatment of four patients with the Churg-Strauss syndrome.  Ann Intern Med. 1998;  129 370-374
    CrossrefPubMedGoogle Scholar
  • 87 Termeer C C, Simon J C, Schopf E. Low-dose interferon alfa-2b for the treatment of Churg-Strauss syndrome with prominent skin involvement.  Arch Dermatol. 2001;  137 136-138
    PubMedGoogle Scholar
  • 88 Simon H U, Seelbach H, Ehmann R, Schmitz M. Clinical and immunological effects of low-dose IFN-alpha treatment in patients with corticosteroid-resistant asthma.  Allergy. 2003;  58 1250-1255
    CrossrefPubMedGoogle Scholar
  • 89 Gordon A C, Edgar J D, Finch R G. Acute exacerbation of vasculitis during interferon-alpha therapy for hepatitis C-associated cryoglobulinaemia.  J Infect. 1998;  36 229-230
    CrossrefPubMedGoogle Scholar
  • 90 Metzler C, Lamprecht P, Hellmich B, Reuter M, Arlt A C, Gross W L. Leucoencephalopathy after treatment of Churg-Strauss syndrome with interferon {alpha}.  Ann Rheum Dis. 2005;  64 1242-1243
    CrossrefPubMedGoogle Scholar
  • 91 Lane S E, Watts R A, Shepstone L, Scott D G. Primary systemic vasculitis: clinical features and mortality.  QJM. 2005;  98 97-111
    CrossrefPubMedGoogle Scholar

Ulrich Specks

M.D., Division of Pulmonary and Critical Care Medicine

Mayo Clinic and Foundation, 200 First St. SW, Rochester, MN 55905

Email: specks.ulrich@mayo.edu

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