Recurring Allergic Brochopulmonary Aspergillosis in a School Child with Primary Ciliary Dyskinesia

Background Allergic bronchopulmonary aspergillosis (ABPA) is a rare but severe complication of bronchial diseases with an impaired mucociliary clearance. Besides numerous publications in patients with cystic fibrosis and severe bronchial asthma, data of people with primary ciliary dyskinesia (PCD) and ABPA remain scarce, especially in children.

Case Report Here, we report about a school-aged patient with PCD (nNO: 20 nl/min; IF: absence of RSPH9; TEM: normal, gene: RSPH4:, c.1453C>T homozygous) and atopy, who experienced two episodes of ABPA within the last 2 years. Both episodes were characterized by a decline of FEV₁ without acute signs of illness. The first episode started in 5/2021 with a decline of% predicted (pp)FEV₁ from 70% to 42% and an increase of total IgE (t-IgE) to 1793 kU/L (ULN 240 kU/L), the eosinophil blood count was 230/nl. An methylprednisolone pulse therapy (15mg/kg bodyweight and day), over three days, and oral itraconazol (200mg BID) was conducted. This therapy increased ppFEV₁ to 74% and decreases t-IgE to 1045 kU/L in 8/2021. The further course was stable (ppFEV₁ of 89% in 4/2022 and a decrease of t-IgE to 867 kU/l). In 10/2022 ppFEV₁ decreased again to 65% and the t-IgE increased to 3225 kU/L, the eosinophil blood count was 700/nl. Sputum cultures grew Aspergillus. A MRI revealed progressive bilateral mucus plugging with new areas of consolidation. A therapy with itraconazol (200mg BID) and oral prednisolone (1mg/kg bodyweight/d) was initiated but did not prove effective (ppFEV₁ dropped to 50% and t-IgE increased to 3747 kU/L). Three weeks later the patient was admitted to the hospital again and received methylprednisolone pulsesover three days (dose: 15mg/kg bodyweight/d) and oral itraconazol was changed to oral voriconazole (200mg BID). There are no indoor plants or domestic animals in the family’s flat. However, mould remediation in the building was done in 9/2022, which is an indicator of an increased exposure to Aspergillus fumigatus.

Conclusion ABPA occurs in children with PCD and should be considered, when FEV₁ declines and no other reasons can be found. Methylprednisolone pulses and antifungal treatment were efficient as acute treatment in this case. Nevertheless, to avoid future relapses of the ABPA, measures to prevent fungal exposure and prophylactic antifungal or anti-inflammatory treatment should be implemented for these patients.

Publication History

Article published online:
09 March 2023