Mononeuropathy multiplex in a Churg-Strauss syndrome patient – case report
Keywords:
Churg-Strauss syndrome, ANCA-associated vasculitis, mononeuropathy multiplexAbstract
A 44 year-old male with asthma and allergic to grass pollen came
to our Emergency Department with dyspnea, productive cough,
paresthesia, dysaesthesia and dysphonia. He was admitted with
a diagnosis of community-acquired pneumonia. An acute axonal
polyneuropathy has developed preventing the patient from walking
becoming totally dependent on others. In spite of exhaustive
investigation the aetiology was not ascertained. Peripheral blood
eosinophilia and P-ANCA positivity with myeloperoxidase specificity led to the diagnostic hypothesis of ANCA associated vasculitis,
namely Churg-Strauss syndrome. Treatment with prednisolone
and cyclophosphamide achieved significant improvement, albeit
a disabling neuropathy persisting and not responding well to
intravenous immunoglobulin and rituximab.
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References
C Mukhtyar, L Guillevin, MC Cid et al. EULAR recommendations for the management of primary small and medium vessel vasculitis, Ann Rheum Dis 2009 ; 68 :310-317.
Pagnoux C, Guilpain P, Guillevin L. Churg-Strauss syndrome, Curr Opin Rheumatol 2007 ; 19 :25-32.
Sinico RA, 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.
Sablé-Fourtassou R, Cohen P, Mahr A et al. Antineutrophil Cytoplasmic Antibodies and the Churg-Strauss Syndrome, Ann Intern Med 2005 ; 143 :632-638.
Lhote F, Cohen P, Guilpain P, Guillevin L. Syndrome de Churg et Strauss, Rev Prat 2008 ; 58 :1165-1174.
Taniguchi M, Tsurikisawa N, Higashi N, Saito H, Mita H, Mori A, Sakakibara H, Akiyama K. Treatment for Churg-Strauss Syndrome : Induction of remission and efficacy of intravenous immunoglobulin therapy, Allergol Int 2007 ; 56 :97-103.
Grau RG. Churg-Strauss syndrome : 2005-2008 update. Curr Rheumatol Rep 2008 ; 10 :453-458.
Lovric S, Erdbruegger U, Kümpers P et al. Rituximab as rescue therapy in anti-neutrophil cytoplasmic antibody-associated vasculitis : a single-centre experience with 15 patients, Nephrol Dial Transplant 2009 ; 24 :179-185.
Stasi R, Stipa E, Del Poeta G, Amadori S, Newland A, Provan D. Long-term observation of patients with anti-neutrophil cytoplasmic antibody-associated vasculitis treated with rituximab, Rheumatology 2006 ; 45 :1432-1436.
Eriksson P. Nine patients with anti-neutrophil cytoplasmic antibodypositive vasculitis successfully treated with rituximab, J Intern Med 2005 ; 257 :540-548.
Stone J, Merkel P, Spiera R et al. Rituximab versus Cyclophosphamide for ANCA-Associated Vasculitis, N Engl J Med 2010 ; 363 : 221-232.
Jones R, Tervaert J, Hauser T et al. Rituximab versus Cyclophosphamide in ANCA-Associated Renal Vasculitis, N Engl J Med 2010 ; 363 :211-220
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