Churg-Strauss syndrome presenting with focal segmental glomerulosclerosis without proteinuria: response to unconventional therapy

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.

Actinic Granuloma with Focal Segmental Glomerulosclerosis

“…Diabetes mellitus has been found in about 37–40% of patient with AG, and may be caused by injury of elastic fiber from hyperglycemic state [ 12 ]. As for the renal condition, focal segmental glomerulosclerosis has been described in association with various granulomatous diseases (e.g., sarcoidosis, Wegener's granulomatosis, Churg-Strauss syndrome, and Kimura's disease) [ 13 , 14 , 15 , 16 ]. However, the association of AG and focal segmental glomerulosclerosis has never been reported.…”