Presentation

A 78-year-old woman presented to the respiratory disease department with exertional dyspnea. Three years previously, she was diagnosed with seropositive rheumatoid arthritis (RA) with concomitant usual interstitial pneumonia (UIP) in a different hospital and had been on a regimen of glucocorticoids and tacrolimus hydrate (Fig. 1a). Bilateral fine crackles were heard on chest auscultation. Chest computed tomography revealed bilateral honeycombing with basal predominance, progression of fibrosis in the left lower pulmonary lobe, and multiple recently developed nodules in the clustered cystic airspaces in the right lower pulmonary lobe (Fig. 1b). Although the sputum culture result was negative for mycosis, Aspergillus galactomannan antigen value was 3.5 (cutoff index, < 0.5). Therefore, chronic pulmonary aspergillosis with concomitant RA-associated interstitial pneumonia was diagnosed. Despite treatment with antifungal agents, including voriconazole and itraconazole, and reversal of tacrolimus, she died 20 months after clinical presentation due to progression of aspergillosis.

Fig. 1
figure 1

a Computed tomography 3 years before presentation, revealing bilateral honeycombing with basal predominance. The radiological pattern was usual interstitial pneumonia. b Computed tomography revealed multiple recently developed nodules in the clustered cystic airspaces in the right lower pulmonary lobe and progression of fibrosis in the left lower pulmonary lobe

Discussion

Chronic pulmonary aspergillosis is a well-recognized complication of immunosuppression, and Aspergillus species grow and multiply in preexisting cavities or cysts in patients with pulmonary disease, including chronic obstructive pulmonary disease or sarcoidosis [1]. However, few cases of honeycomb cysts with aspergillomas have been reported, and the etiologies underlying UIP are usually idiopathic pulmonary fibrosis (IPF) or systemic sclerosis [2,3,4]. Although aspergillosis in patients with RA has been reported, to our best knowledge, multiple mycetoma in the lungs with honeycombed interstitial fibrosis associated with RA has not been reported. Patients with RA and UIP have a poor prognosis [5]. Since Aspergillus species usually form in preexisting lung lesions, we believe existing interstitial lung disease was more involved than steroids or immunosuppressive agents in the onset of pulmonary aspergillosis in this patient. When treating patients with RA and comorbid lung disease, clinicians should pay attention to lung infections, especially when administering steroids and immunosuppressive agents.