RT Journal Article
SR Electronic
T1 Allergic bronchopulmonary mycosis due to rhizopus nigricans in two siblings with and without CF?
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP PA1247
DO 10.1183/13993003.congress-2016.PA1247
VO 48
IS suppl 60
A1 Stefanie Benzrath
A1 Anne Schlegtendal
A1 Folke Brinkmann
A1 Cordula Koerner-Rettberg
YR 2016
UL http://erj.ersjournals.com/content/48/suppl_60/PA1247.abstract
AB Background: Non-Aspergillus allergic bronchopulmonary mycosis is rare and represents a challenge concerning diagnosis and therapy. We present the cases of a 7 year old girl with FEV1 120-147% predicted, pseudomonas negative, atopic predisposition, total IgE 283 kU/l, and of her 6 year old previously healthy brother (no CF, no history of asthma) who developed symptoms of fever, dyspnoe, wheezing and a marked decline in physical capacity at almost the same time.Methods: Lung function, serologic and skin tests, imaging and interventions on exposition were used to clarify the diagnosis.Results: As both siblings were affected and total IgE initially did not rise asthma was suspected; however no steroid inhalation proved ineffective as did a course of oral antibiotics. After 6 weeks of symptoms the girl's IgE rose to 1500 kU/l, FEV1 dropped to 94% predicted, and ABPA was suspected; however no Aspergillus fumigatus specific IgE or IgG could be detected. A trial of systemic steroids showed cessation of symptoms and a rise in FEV1 to >140%. Finally a sensitization against Rhizopus nigricans, a rare mould, was found in both siblings. Both children improved by absence from domestic environment, and both needed systemic steroids to control symptoms and omalizumab to reduce oral steroids. The search for the domestic source of exposition is still ongoing.Conclusion: This rare case shows that allergic reactions to rare moulds affects both healthy and CF patients similarily and should kept in mind if asthma like symptoms without sensitization to typical allergens occur.