Management of Churg-Strauss Syndrome (CSS) in the setting of Disseminated Histoplasmosis

Abstract

Churg-Staruss Syndrome (CSS), also known as Eosinophilic Granulomatosis with Polyangiitis, is characterized by asthma, chronic rhinosinusitis, eosinophilia and vasculitis. We describe a case of CSS management in the setting of disseminated histoplasmosis. A 60-year-old male with CSS was admitted with fever, pancytopenia and transaminitis. He was on prednisone, methotrexate (MTX) and Cellcept. Given concern for infection MTX and Cellcept were discontinued. Patient had recently torn down a deck with numerous bird droppings. Infectious work up revealed elevated Fungitell, Histoplasma antigen and antibody levels. Bone marrow biopsy and blood cultures were positive for Histoplasma Capsulatum confirming the diagnosis of disseminated histoplasmosis. Patient was treated with Amphotericin and Voriconazole. One month later patient had uncontrolled rhinosinusitis and asthma despite maximal therapy including oral and inhaled corticosteroids. Cellcept and MTX could not be resumed with disseminated histoplasmosis. He was treated with high dose IVIG and Omalizumab with significant improvement in symptoms. The mainstay of CSS treatment is corticosteroids. However, cytotoxic drugs are required for refractory disease. The patient described was on immunosuppressants prior to developing disseminated histoplasmosis, which precluded continuation of immunosuppression, thus leading to uncontrolled disease. This presented a challenge in management. High dose IVIG has been found to be effective in EGPA patients with cardiomyopathy or mononeuritis multiplex. The activation and induction of Treg cells is responsible for this efficacy. Our patient’s asthma and rhinosinusitis improved with IVIG and Omalizumab therapy. High dose IVIG and Omalizumab could be used to treat asthma and rhinosinusitis in CSS patients when immunosuppressants are contraindicated.