Guha P, Thakur I, Ray A, Chatterjee SK
Correspondence: Dr Pradipta Guha, dr.pradipta@yahoo.co.in
ABSTRACT
Churg-Strauss syndrome (CSS), or allergic granulomatosis, is a rare disease manifested by tissue infiltration, hypereosinophilia and vasculitis. Renal involvement may be seen in up to 50% of cases. We report the case of a 25-yearold man who presented with a history of fever for two months, tingling, numbness, and paraesthesia of the upper limbs and left lower limb, along with diarrhoea for one month and an inability to walk for the past seven days. Serial laboratory investigations helped to reach the final diagnosis of CSS with mononeuritis multiplex, and skin, pulmonary and gastrointestinal involvement with hypertension. This is due to renal involvement in the form of focal segmental glomerulosclerosis without any nephrotic range proteinuria, which is a very rare clinical entity. The patient’s symptoms were relieved after the administration of an unconventional mode of therapy.
Keywords: Churg-Strauss syndrome, focal segmental glomerulosclerosis, immunosuppressive therapy
Singapore Med J 2013; 54:e13-e15; http://dx.doi.org/10.11622/smedj.2013018
REFERENCES
1. Harrold LR, Andrade SE, Go AS, et al. Incidence of Churg-Strauss syndrome in asthma drug users: a population-based perspective. J Rheumatol 2005; 32:1076-80. | ||||
2. Barbiano di Belgiojoso G, Genderini A, Sinico RA, et al. Acute renal failure due to microscopic polyarteritis with the same histological and clinical patterns in a father and his son. Contrib Nephrol 1991; 94:107-14. | ||||
3. Schmitt WH, Csernok E, Kobayashi S, et al. Churg-Strauss syndrome: serum markers of lymphocyte activation and endothelial damage. Arthritis Rheum 1998; 41:445-52. http://dx.doi.org/10.1002/1529-0131(199803)41:3<445::AID-ART10>3.0.CO;2-3 | ||||
4. Solans R, Bosch JA, Pérez-Bocanegra C, et al Churg-Strauss syndrome: outcome and long-term follow-up of 32 patients. Rheumatology (Oxford) 2001; 40:763-71. http://dx.doi.org/10.1093/rheumatology/40.7.763 | ||||
5. Nochy D, Daugas E, Droz D, et al. The intrarenal vascular lesions associated with primary antiphospholipid syndrome. J Am Soc Nephrol 1999; 10:507-18. | ||||
6. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951; 27:277-301. | ||||
7. Clutterbuck EJ, Evans DJ, Pusey CD. Renal involvement in Churg-Strauss syndrome. Nephrol Dial Transplant 1990; 5:161-7. http://dx.doi.org/10.1093/ndt/5.3.161 | ||||
8. Wechsler ME, Garpestad E, Flier SR, et al. Pulmonary infiltrates, eosinophilia, and cardiomyopathy following corticosteroid withdrawal in patients with asthma receiving zafirlukast. JAMA 1998; 279:455-7. http://dx.doi.org/10.1001/jama.279.6.455 | ||||
9. Lanham JG, Elkon KB, Pusey CD, Hughes GR. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore) 1984; 63:65-81. http://dx.doi.org/10.1097/00005792-198403000-00001 | ||||
10.Tesar V, Ríhová Z, Jancová E, Rysavá R, Merta M; European randomized trials. Current treatment strategies in ANCA-positive renal vasculitislessons from European randomized trials. Nephrol Dial Transplant 2003; 18 (Suppl 5):v2-4. http://dx.doi.org/10.1093/ndt/gfg1032 | ||||
11. Cooper BJ, Bacal E, Patterson R. Allergic angiitis and granulomatosis. Prolonged remission induced by combined prednisone–azathioprine therapy. Arch Intern Med 1978; 138:367-71. http://dx.doi.org/10.1001/archinte.1978.03630270021012 | ||||
12. Sinico RA, Di Toma L, Maggiore U, et al. Renal involvement in Churg-Strauss syndrome. Am J Kidney Dis 2006; 47:770-9. http://dx.doi.org/10.1053/j.ajkd.2006.01.026 | ||||