Multiple lung nodules, eosinophilia and severe asthma

ABSTRACT

We present an unusual clinical scenario of a severe asthmatic with persistent eosinophilia and multiple peripheral pulmonary nodules. Biopsy of these nodules was consistent with a diagnosis of angiolymphoid hyperplasia with eosinophilia (ALHE). ALHE is a rare condition that usually affects women and commonly affects subcutaneous tissues of head and neck. Only a handful of cases have reported pulmonary involvement. To our knowledge, this is the first case of pulmonary ALHE that has been treated with benralizumab, an anti-interleukin-5 receptor alpha monoclonal antibody, which resulted in improvement in symptoms as well as airway and blood eosinophilia and had a modest effect on the size of the pulmonary nodules. We also highlight a novel imaging finding of beading and focal nodular dilation of sub-segmental branches of pulmonary arteries, which could reflect proliferation of small-caliber vessels and hyperplastic endothelial lining seen on histopathology.

RÉSUMÉ

Nous présentons un scénario clinique inhabituel d’un patient atteint d’asthme grave avec une éosinophilie persistante et de multiples nodules pulmonaires périphériques. La biopsie de ces nodules était compatible avec un diagnostic d’hyperplasie angiolymphoïde avec éosinophilie (HALE). La HALE est une maladie rare qui affecte habituellement les femmes et affecte le plus souvent les tissus sous-cutanés de la tête et du cou. Seuls quelques cas ont rapporté une atteinte pulmonaire. À notre connaissance, il s’agit du premier cas de HALE pulmonaire traité avec du benralizumab, un anticorps monoclonal anti-récepteur de l’interleukine-5 alpha, qui a entraîné une amélioration des symptômes ainsi que de l’éosinophilie des voies respiratoires et du sang, et a eu un effet modeste sur la taille des nodules pulmonaires. Nous soulignons également une nouvelle découverte par imagerie de perlage et de dilatation nodulaire focale des branches sous-segmentaires des artères pulmonaires, ce qui pourrait refléter la prolifération de vaisseaux de petit calibre et de revêtement endothélial hyperplasique observé en histopathologie.

KEYWORDS:

• Asthma
• eosinophilia
• pulmonary nodules
• angiolymphoid hyperplasia with eosinophilia
• benralizumab

Acknowledgment

We acknowledge K. Radford’s assistance with the immunofluorescence staining.

Statement of ethics

The subject has given their written informed consent to publish their case and images.

Disclosure statement

A. Bhalla is supported by the Frederick E. Hargreave fellowship in Airway Diseases. P. Nair is supported by the Frederick E. Hargreave Teva Innovation Chair in Airway Diseases. He has received honoraria from AZ, Sanofi, Teva, Merck, Novartis and Equillium and his university has received research grants from AZ, Teva, Sanofi, Novartis, BI and Methapharm. E.A. Haider, J-C. Cutz and M. Trus do not have any relevant disclosures to declare.

Funding

This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.

Authors contributions

P. Nair conceived the idea and is the guarantor of the paper. P. Nair and A. Bhalla wrote the initial draft. P. Nair, A. Bhalla and E.A. Haider edited the manuscript. J.C. Cutz provided the pathology images. E.A. Haider provided the radiology images. All authors provided clinical care to the patient and have approved the final draft for submission.