Background: Churg-Strauss syndrome (CSS), or eosinophilic granulomatosis with polyangiitis, is a rare syndrome that affects small- to medium-sized arteries and veins. Criteria for the diagnosis include: asthma (wheezing, expiratory rhonchi), eosinophilia of more than 10% in peripheral blood, paranasal sinusitis, pulmonary infiltrates (may be transient), histological proof of vasculitis with extravascular eosinophils, and mononeuritis multiplex or polyneuropathy. The worldwide incidence of CSS is approximately 2.5 cases per 100,000 adults per year and its incidence in the United States is one to three cases per 100,000 adults per year.1 In the Philippines, the exact incidence is unknown with very few published case reports about it.

Setting: University of the Philippines-Philippine General Hospital (UP-PGH), a tertiary training hospital in Manila, Philippines

The Case: A 40-year-old Filipino male with a history of adult onset asthma and recurrent sinusitis manifesting with inability to dorsiflex the left ankle (foot drop), various dermatologic lesions, and arthralgia. Complete blood count showed hypereosinophilia. Electromyography revealed asymmetric moderate to severe sensory and motor denervation of limbs compatible with polyneuropathy. Skin biopsy revealed lymphocytic vasculitis. P-ANCA was positive. During his incumbent hospitalization, the skin lesions, arthralgia and neurologic manifestations improved on administration of high dose steroids. Pregabalin was used to control pain secondary to the mononeuritis multiplex.

Significance: To report a rare case of Churg- Strauss syndrome presenting as foot drop. This case highlights the importance of considering Churg- Strauss syndrome among adult patients presenting with neurologic complaint (inability to dorsiflex the left ankle/foot drop) and various dermatologic lesions.

Keywords: Churg-Strauss syndrome, mononeuritis multiplex, ANCA-associted vasculitis  

1. Eustace JA, Nadasdy T, Choi M; Disease of the month. The Churg Strauss Syndrome. J Am Soc Nephrol, 10(9):2048-55, 1999.
2. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY; The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum, 33(8):1094-100, Aug 1990
3. Churg J, Strauss L; Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol, 27(2):277-301, Mar-Apr 1951.
4. Hellmich B, Ehlers S, Csernok E, Gross WL; Update on the pathogenesis of Churg-Strauss syndrome. Clin Exp Rheumatol, 21(6 Suppl 32):S69-77, Nov-Dec 2003.
5. Vaglio A, Martorana D, Maggiore U, Grasselli C, Zanetti A, Pesci A; HLA-DRB4 as a genetic risk factor for Churg-Strauss syndrome. Arthritis Rheum, 56(9):3159-66, Sep 2007.
6. Lanham JG, Elkon KB, Pusey CD, Hughes GR; Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore), 63(2):65, 1984.
7. Pagnoux C, Guillevin L; Churg-Strauss syndrome: evidence for disease subtypes? Curr Opin Rheumatol, 22(1):21, 2010.
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 (Baltimore), 78(1):26-37, Jan 1999 et al.; Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore), 78(1):26-37, Jan 1999.
9. Hattori N, Ichimura M, Nagamatsu M, Li M, Yamamoto K, Kumazawa K, Mitsuma T, Sobue G: Clinicopathological Features of Churg-Strauss Syndrome-Associated Neuropathy. Brain.; 122 (Pt3):427, 1999.
10. Allen JN, Davis WBL. Eosinophilic Lung Diseases. Am J Respir Crit Care Med.; 150 (5 Pt 1): 1423, 1994.
11. Lie JT. Histopathologic specificity of systemic vasculitis. Rheum Dis Clin North Am, 21(4):883-909, Nov 1995.
12. Katzenstein AL; Diagnostic features and differential diagnosis of Churg-Strauss syndrome in the lung. A review. Am J Clin Pathol, 114(5):767-72, Nov 2000.
13. Chumbley LC, Harrison EG Jr, DeRemee RA; Allergic granulomatosis and angiitis (Churg-Strauss syndrome): report and analysis of 30 cases. Mayo Clin Proc. 52:477-484, 1977.
14. Davis MD, Daoud MS, McEvoy MT, Su WP; Cutaneous manifestations of Churg-Strauss syndrome: a clinicopathologic correlation. J Am Acad Dermatol, 37(2 Pt 1):199-203, 1997
15. Davies DJ, Moran JE; Segmental necrotizing glomerulonephritis with antineutrophil antibody: possible arbovirus aetiology?. BMJ. 285:606, 1982.
16. Kallenberg CG, Brouwer E, Weening JJ, Tervaert JW; Anti-neutrophil cytoplasmic antibodies: current diagnostic and pathophysiological potential. Kidney Int, 46(1):1-15, Jul 1994
17. 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,13(2):193-8, Mar-Apr 1995.
18. Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O, Thibult N, Cassasus P; Prognostic factors in polyarteritis nodosa and Churg–Strauss syndrome. A prospective study in 342 patients.Medicine (Baltimore),75:17–28. 1996.
19. Grau RG; Churg-Strauss syndrome: 2005-2008 update. Curr Rheumatol Rep, 10(6):453-8. Dec 2008.
20. Casian A, Jayne D; Plasma exchange in the treatment of Wegener’s granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and renal limited vasculitis. Curr Opin Rheumatol, 23(1):12-7. Jan 2011.
21. Koukoulaki M, Smith KG, Jayne DR; Rituximab in Churg-Strauss syndrome. Ann Rheum Dis, 65(4):557-9, 2006.
22. Pepper RJ, Fabre MA, Pavesio C, Gaskin G, Jones RB, Jayne D, Pusey CD, Salama AD. Rituximab is effective in the treatment of refractory Churg-Strauss syndrome and is associated with diminished T-cell interleukin-5. Rheumatology (Oxford) 47(7): 1104-5, 2008.