Edward Charbek^1,, Rajaa AlMourani², Rugheed Ghadban³, David A. Stoeckel⁴

The relationship between some infections and vasculitides especially antineutrophil cytoplasmic antibodies (ANCA) mediated disease is well established. However, the mechanisms by which infection triggers ANCA formation are not fully understood. We report a case of granulomatosis with polyangiitis (GPA) occurring concurrently with human monocytic ehrlichiosis (HME) and discuss the possible relation between the two disease processes. A 49 year old Caucasian female presented with septic shock with multiple organ dysfunction syndrome that was attributed to HME. The patient was noted to have a small crusted lesion on the left nasolabial fold. Over the course of her hospitalization, it gradually expanded and eroded into the nasal cavity. After appropriate workup, she was diagnosed with GPA. HME hasn’t been clearly linked to any type of vasculitis. Review of the literature revealed scarce evidence limited to case reports and retrospective studies describing the association between GPA and HME. We highlight in this case a possible link between HME and GPA, however, the exact relationship between HME and GPA remains unclear and warrants further study.

granulomatosis with polyangiitis, human monocytic ehrlichiosis, vasculitis