[CIS PIDD] [cis-pidd] HIES and lung transplantation

[CIS-PIDD]

Hi all,



Thanks in advance for your collective expertise regarding a recent referral:


55F with AD hyper IgE syndrome (known STAT 3 L706M mutation) referred by Respiratory to help assess suitability for lung transplantation. She has previously been managed by a colleague.


·         HIES diagnosed in the '80s, recurrent childhood infections (predominantly cutaneous early on)

o   Previous recurrent skin furunculosis and mycobacterium terrae tenosynovitis

o   Eczema reasonably well controlled (more prominent as a child)

o   Blind in R eye secondary to ?polymicrobial infection

o   A trial of treatment with IVIg in the late '90s provided no symptomatic benefit and was ceased

o   Never had interferon gamma therapy

·         Previous mucinous tumour of the ovary

o   TAH and BSO 10 years ago, no recurrence since

·         L ear high frequency hearing loss - aminoglycoside related.

·         Recently lung infections have become most prominent, with progressive cystic/saccular bronchiectasis.

o   Colonized with pseudomonas aeruginosa (last sputum 22/02/16 - abundant growth pseudomonas aeruginosa -sens to tazocin, ciprofloxacin, resistant to gentamicin) and Aspergillus fumigatus in past

§  No evidence of fungal or opportunistic infection on CT chest

o   Recurrent infective exacerbations about 2x per year on average.

o   On prophylactic azithromycin; tobramycin nebs 80mg BD - for years.

o   Severe obstruction on recent PFTS

§  Seretide 250/25 2 puffs BD and Salbutamol MDI

o   Oxygen dependant since Jan 16.

o   Exercise tolerance dropped from 1-2km in late 2015 to just around house since early Jan this year (minimal improvement in ex tolerance despite multiple courses of broad spectrum antibiotics)

o   Previous R sided empyema in Jan 15 - had pleural drainage with ICC and received intra-pleura thrombolytic therapy (tPA-DNAse); no VATS decortication/thoracotomy.

·         Reports repeated and problematic skin infections / boils with previous courses of steroids (last received approx 20 years ago) and we understand this was considered the main factor precluding consideration of a lung transplant in the past.

·         Other background:

o   Ex-smoker (smoked 4-5 cigs/day during teenage years; ceased about 40 years ago)

o   ETOH - rarely drinks

o   Medically retired; used to work in finances (in police station) for 35yrs

o   Previously worked on family's farm, exposed to herbicides.

o   Hobbies: wood works (not wearing mask usually)

o   Pet - dog; no birds

o   Travel - Europe last year; Bali 5 yrs ago

o   Married - two grown up kids (daughter - has hyper IgE syndrome as well)

o   No previous fractures/

She has been reviewed by an ID physician who has stated "there are no infectious disease contraindications to transplantation" but clearly she is high risk for complications. I have only been able to find one paper describing a patient with HIES having a lung transplant in the absence of HSCT (NIH group describing WU polyomavirus in Respiratory epithelial cells post transplant).

I would be grateful to hear from others regarding their experience with lung transplant in HIES and/or opinions/suggestions on the above case.

Kind Regards,

Ben


Dr Ben McGettigan FRACP FRCPA
Clinical Immunologist

Address: Immunology Dept, Fiona Stanley Hosptial, L1 Pathology, Locked Bag 100
Palmyra DC, Western Australia 6961


Email: Benjamin.McGettigan at health.wa.gov.au<mailto:Benjamin.McGettigan at health.wa.gov.au>
Website: www.health.wa.gov.au<http://www.health.wa.gov.au>

Delivering a Healthy WA


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