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.