A 29-year-old woman chiropractor with repeated episodes of
bacteremia and positive for cytoplasmic
antineutrophil cytoplasmic antibody (
c-ANCA) and antiproteinase-3 (PR3) antibody had an 11-year history of
asthma and repeated hospitalization. While hospitalized she developed a spiking nocturnal
fever with
chills and
erythema of the lower leg. Her
fever resolved spontaneously, but
corticosteroid therapy and
antibiotics did not effectively reduce her symptoms. Plain
X-ray film and CT showed pulmonary changes, and lung biopsy
granulomas without
vasculitis. Immunological studies showed a positive PR3-ANCA (
c-ANCA) test, and skin biopsy of the crural
erythema showed
foreign body granuloma. Multiple blood cultures were positive for several strains of bacteria, including Fusobacterium necrophurum, Mycobacterium fortuitum, and Clostridium species. When placed in a single room and monitored, she did not develop new
fever or
erythema. Because self-injury was assumed, she was diagnosed as having Münchausen syndrome. This is, to our knowledge, the first report of Münchausen syndrome with PR3-ANCA positivity. In patients with repeated
infections, the possibility of self-injury, the development of PR3-ANCA positivity, and pulmonary
granuloma with a disease profile similar to
vasculitis syndrome should be considered in the definitive diagnosis.