Dj vu all over again Jonathan Singer MD MS University of - - PowerPoint PPT Presentation

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Dj vu all over again Jonathan Singer MD MS University of - - PowerPoint PPT Presentation

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5/9/2015 Disclosures None Dj vu all over again Jonathan Singer MD MS University of California, San Francisco HPI HPI continued 49 y/o woman presents for lung transplant Pond cleaned up, symptoms persist evaluation for

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SLIDE 1

5/9/2015 1

Déjà vu all over again

Jonathan Singer MD MS University of California, San Francisco

Disclosures

  • None

HPI

  • 49 y/o woman presents for lung transplant

evaluation for Hypersensitivity Pneumonitis

  • Exposures: Open cheese whey pond behind

home; pigeons

  • Serologies: +avian and +fungal precipitins

HPI continued

  • Pond cleaned up, symptoms persist
  • Pt moved to another town in same region
  • Treated intermittently with prednisone and

azathioprine over 18 years

  • Periodic respiratory decompensation, unknown triggers
  • At transplant eval, on 6LPM
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SLIDE 2

5/9/2015 2 Physiologic Data PFTs:

FEV1 0.74 L (27%) FVC 0.88 L (25%) TLC 2.39 L (44%) DLCO: could not obtain

Bilateral lung transplant: 3/2009

  • Uneventful operative course
  • Immunosuppression: Tacrolimus, prednisone,

mycophenolate mofetil

CT: Airtrapping, Bronch: purulence, aspergillus+ CT: Airtrapping, Bronch: purulence, aspergillus+ Needs reevaluation…

Year 1 Year 2 Year 3 Year 4

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SLIDE 3

5/9/2015 3 Ideas on what might be going on? Approach to acute allograft dysfunction

  • Goal: Identify reversible cause
  • Airway issue
  • Infection
  • Acute rejection
  • Drug reaction
  • Identify reversible cause of allograft dysfunction
  • Airway issue
  • Infection
  • Acute rejection
  • Drug reaction
  • Negative workup = Chronic Lung Allograft Dysfunction
  • Bronchiolitis obliterans syndrome (BOS)
  • Restrictive allograft syndrome (RAS)
  • Acute fibrinoid organizing pneumonia (AFOP)

Approach to acute allograft dysfunction

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SLIDE 4

5/9/2015 4 Dynamic CT scan with air trapping Transbronchial Biopsy

Airway epithelial surface Granuloma Alveoli Airway smooth muscle

Diagnosis: Recurrent HP

  • Pulsed with 40 mg of prednisone for a month

followed by taper

  • Upon direct questioning, patient noted pigeons

roosting outside her window

  • Son put chicken wire around window sill, ineffective

at keeping away pigeons

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SLIDE 5

5/9/2015 5

CT: airtrapping Bronch: multiple subepithelial granulomas

Year 1 Year 2 Year 3 Year 4

Further Follow-up

  • Patient moves…again
  • All subsequent biopsies negative for granulomas
  • Lung function stabilizes
  • Last seen 3/2015- doing well with stable DOE

Disease recurrence in the allograft

  • 1,394 lung transplant recipients
  • Recurrence in 15 (1%)

Collins, Radiology 2001

Disease Number % Sarcoidosis 9/26 35% LAM 2/21 10% Pulmonary Langerhans 1/4 25% Talc Granulomatosis 1/1 100% Diffuse Panbronchiolitis 1/1 100% Alveolar Proteinosis (PAP) 1/2 50%

Other published case reports

  • Sarcoidosis
  • Lymphangioleiomatosis (LAM)
  • Pulmonary Langerhans cell histiocytosis (PLCH)
  • Talc granulomatosis
  • Diffuse panbronchiolitis
  • Pulmonary alveolar proteinosis (PAP)
  • Desquamative interstitial pneumonitis (DIP)
  • Giant cell interstitial pneumonia (Hard metal lung/Cobalt)
  • Alpha-1 antitrypsin deficiency related COPD
  • Bronchoalveolar carcinoma

~30 studies reported recurrence of primary disease after transplant

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SLIDE 6

5/9/2015 6 Hypersensitivity Pneumonitis

  • Extrinsic allergic alveolitis caused by inhaled organic

particles

  • > 300 potential causal agents
  • Antigen unknown ~40 % of cases1
  • Avoidance difficult if agent unknown
  • HP = 1% of lung transplants performed in U.S.
  • No published reports on outcomes

Selman, AJRCCM 2012

Lung Transplantation for HP at UCSF

  • Extrinsic

Kern RM. CHEST. 2014

Lung Transplantation for HP at UCSF

  • Retrospective cohort study of LTx at UCSF between

2000-2013

  • All LTx for ILD re-reviewed by multidisciplinary ILD

approach

  • Clinical history, radiology, VATS and explant

pathology

Kern RM. CHEST. 2014

Lung Transplantation for HP at UCSF

  • Of 406 LTx, 31 were for HP (8%)
  • Only 23 of 31 HP cases were diagnosed before LTx (74%)
  • 2 definitive, 1 probable case of recurrence after LTx

Kern RM. CHEST. 2014

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SLIDE 7

5/9/2015 7 Survival after LT for HP is quite good Conclusions

  • HP is a surprisingly common indication for

transplant

  • Hard to diagnose accurately
  • HP can recur after lung transplant
  • Identifying antigen is important
  • Be aware that it is possible: immunosuppression

is not protective

Thank you.