Eosinophilic granulomatosis with polyangiitis complicated with rapidly progressive glomerulonephritis in a young man who is a healthy cyclist

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare systemic vasculitis. Rapidly progressive glomerulonephritis (RPGN) is a rare complication of EGPA. We report a case of a 60-year-old man, who is also a skilled cyclist, who was hospitalized to investigate a symptomatology that had arisen over the previous months and worsened in the last few weeks, to the point of limiting normal everyday activities. The physical examination revealed the presence of livedo reticularis of the four limbs, purpura of the lower limbs, arthritis of the ankles, and low-grade fever; the patient showed intense asthenia, loss of appetite, retrosternal heartburn, and a scarcely pharmacologically controlled asthma. He also reported weight loss (about 5 kg in the last 6 months). Rapidly progressing renal failure was observed with hyper-eosinophilia (4.7 thousand/μL eosinophils, 44% of total leukocytes), pulmonary opacities on chest computed tomography (CT), and sinusitis on CT of the facial massif. The search for antibodies directed against neutrophil cytoplasm (ANCA) revealed a high level of pANCA (pANCA ++, ELISA anti-MPO 666 UI/ml), associated with an increment of inflammation indicators. The induction therapy was high-dosage intravenous glucorticoids and cyclophosphamide, to improve the short and long-term prognosis. After 7 months of treatment, the patient reported a considerable improvement of the symptoms, which at that point did not necessitate pharmacological interventions. The eosinophils value was 0 cells/mm³, the inflammation indexes were back to the norm, and the renal function appeared significantly improved.

Keywords: eosinophilic granulomatosis with polyangiitis; ANCA-associated vasculitis; Churg-Strauss Syndrome; rapidly progressive glomerulonephritis.