[CIS PIDD] [cis-pidd] CVID, bronchiectasis, atypical mycobacteria, hemoptysis

[CIS-PIDD]

Hi Mikko,

Thanks for your reply.

The 63yo female has a long history of recurrent pneumonia episodes since childhood. Bronchiectasis was diagnosed at age 22yo. She was diagnosed with CVID at Barnes Jewish in 2006 (at the age of 54y). She has been on IVIG since 2007. First episode of hemoptysis in 2005 with recurrence in 2013, at which time BAL grew Mycobacteria massilense, treated with IV antibiotics for 5wks (discontinued due to side effects). In 2014 she had pneumonia again, with leukopenia and bone marrow bx was negative. BAL in 2015 + for Mycobacterium avium. HRCT chest in January 2016 showed multifocal bronchiectasis with nodularity throughout the lung fields but concentrated in mid lung. She is ANA -, SSB/SSA- on previous studies. Lately she is having hemoptysis (bright red blood in mucous ~teaspoon amounts or tinged tissues). She has one AFB culture pending for speciation. One negative AFB cx prior after round of triple therapy. Pulmonologist says she has “2years” left with her current lungs. She is an active professional woman with a career.

The other pt is a retired plastic surgeon. Baseline immunoglobulins (need to find these), reportedly low IgG2 and IgG3, as well as low IgM. Bronchiectasis and hemoptysis. Recent new mycobacterial culture + pending sensitivities. Sometimes has up to 300cc blood loss. Followed by pulmonary.

I just inherited both patients, as I learn more will keep you posted.

Thanks!!
Elena
On Jun 15, 2016, at 12:48 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:

Hi Elena,

Tell me about CVID compications in adults...

However, your q really makes me curious, since not one of our CVIDs have had atypical mycobacteria despite (all too common) bronchiectasis. And we have checked.

However, our STAT1/STAT3s do...

Of course climate might have some effect I guess in case of mycobacteria, but our patients do definitely encounter these and the tap water in some areas of Helsinki City with old pipes has really high counts...??

At least I would not proceed to LTx w/o some gene digging...

Mikko

Oyl Mikko Seppänen
Harvinaissairauksien yksikkö (HAKE)

Head, Rare Disease Center,
Helsinki University Hospital (HUH)
FINLAND

phone +358 947180201
GSM +358 50 4279606
fax +358 9 47174703

CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> kirjoitti 15.6.2016 kello 16.52:

Hello Colleagues,

I am seeing more adults with PIDD in practice now. Coming from the pediatric side, it is interesting (and scary at times) to see some of the complications-- that I’ve not had to routinely manage yet-- of CVID later in life.

Yesterday I saw two patients one male in their 50s and a 63yo female with CVID, bronchiectasis, atypical mycobacteria and recurrent hemoptysis. Both are followed by pulmonologists locally and one has been to National Jewish for evaluation. It seems there are differing opinions on the treatment of atypical mycobacteria in these patients.

My questions are:
1/ are there any guidelines you use for these patients on acute management of hemoptysis episodes?
2/ how aggressively do you follow cultures for atypical mycobacteria and treat/retreat/stay on treatment?
3/ for patients with CVID and bronchiectasis whose pulmonologists feel they are at “end stage” do you initiate a lung transplant evaluation? if so where? (we are in South FL)

Thanks!
Elena

Allergy Associates of the Palm Beaches
840 US Highway 1, Suite 235
North Palm Beach, FL 33408

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