Background
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)
is a group of chronic multisystem autoimmune diseases with substantial
mortality and morbidity and frequent relapses. AAV is characterized by
predominant small vessel inflammation and necrosis in conjunction with
ANCA in 60%–90% of cases at disease onset. With the introduction of
treatment with corticosteroids and immunosuppressant drugs, the
remission rate has improved in most cases, but patients with AAV are
still at increased risk of death compared with age-and sex-matched
members of the general population[1]. Infection
plays a critical role in these patients’ adverse
outcomes[2]. Approximately 20%–60% of patients
with AAV develop infections[3], and severe
infections are associated with permanent organ damage and high
mortality. Opportunistic viral infections, such as cytomegalovirus (CMV)
infection, herpes zoster (HZ), and fungal or bacterial infections, can
be seen in patients with AAV[3].
Moreover, the complexity of the disease condition and other
treatment-related adverse reactions as well as infections are often
confusing. Fequently, it is difficult to distinguish them because of
their similar manifestations. Here, we describe a case of pulmonary
mucormycosis in an old female patient with AAV to highlight these
challenges.