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1. In consultation with the pulmonology service, inhaled fluticasone was

initiated because of a history of asthma and obstructive lung disease on
spirometry.

2. The soft tissue density portion of the lesion had some punctate areas of
increased density representing inspissated mucus or calcification.

3. The patient underwent right thoracotomy and open lung- wedge resection

4. A biopsy of the right nasal turbinate was performed Nasal mucosal cultures
were negative.

The ‘hole’ story of a lung: Questions

Aadil Kakajiwala1 & Priyanka Fernandes2 & Bruce R. Pawel 3,4 & Sandra Amaral1,4

Received: 23 June 2016 /Revised: 24 June 2016 /Accepted: 27 June 2016 /Published online: 18 July
2016 # IPNA 2016 Keywords Renal transplantation . Immunosuppression . Fevers . Pulmonary
cavitary lesion

Background

A 14 year-old male patient with prune belly syndrome and chronic kidney disease (CKD) who
underwent his second renal transplant 2 years earlier was admitted with a worsening cough.

Transplant and immunosuppression

The patient underwent his first living-related donor renal transplant at the age of 3 years from his
mother. Eight years later, the transplant failed secondary to recurrent urinary tract infections (UTI)
and chronic allograft nephropathy. The first transplant had been managed with thymoglobulin
induction, prednisone, tacrolimus, mycophenolate mofetil (MMF), and sirolimus. He received a
second living-related donor renal transplant from his father at the age of 12 years. He was induced
with thymoglobulin and maintained on prednisone, MMF, and tacrolimus. One month after his
second transplant, he was noted to have BK viremia, with titers of 5,236,070 copies/ml. In
response, MMF was discontinued, and he was started on leflunomide. Four months later, he
experienced a gradual increase in creatinine, with renal biopsy findings of mild chronic allograft
nephropathy, focal interstitial inflammation, tubular atrophy, and focal vascular hyalinosis,
suggestive of calcineurin toxicity. He was started on sirolimus for immunosuppression (with a goal
sirolimus level of 5–8 ng/ml) and continued on a very low dose of tacrolimus of 1 mg twice daily,
prednisone 5 mg (initially daily and switched to every other day 8 months after the transplant),
and leflunomide 15 mg daily. He developed significant Epstein Barr virus (EBV) viremia 14 months
after transplantation (titers up to 903,013 copies/ml). The EBV viremia improved by 18 months
posttransplantation (52,498 copies/ml). BK viremia also resolved and leflunomide was
discontinued. Twenty months after transplantation, his tacrolimus was discontinued due to
another rise in EBV titers (up to 645,692 copies/ml).

Past medical history

Following his second transplant, he had multiple admissions for UTIs and fevers of unknown origin.
About a year after the transplant, he presented with fevers and abdominal pain. His white cell
count was 31,000/μl and absolute neutrophil count 23,636/μl. Blood and urine cultures at the
time were negative. Within this context, his EBV DNA titers were found to be elevated to 903,013
copies/ml. Computed tomography (CT)

Summary
 In consultation with the pulmonology service, inhaled fluticasone was initiated because of a
history of asthma and obstructive lung disease on spirometry. Mycoplasma and pertussis
polymerase chain reaction (PCR) were negative. Two days after admission, a CT scan without
contrast of the sinuses, chest, abdomen, and pelvis was obtained due to elevated EBV DNA titers
(3,186,970 copies/ml). CT of the lungs showed a nonspecific 2.8-cm cavitary lesion in the right
lower lung [Fig. 1]. The soft tissue density portion of the lesion had some punctate areas of
increased density representing inspissated mucus or calcification.

There were patchy opacities in the right middle and left lower lobes most suggestive of
atelectasis. There were no pathologically enlarged lymph nodes in the chest, abdomen, or pelvis.
The patient underwent right thoracotomy and open lung-wedge resection. Tissue from the lung
biopsy specimen was sent for staining and cultures for aerobic and anaerobic bacteria, fungi, and
mycobacteria, as per recommendation from the immunocompromised infectious diseases service.

Biopsy images and histology are displayed in Fig. 2. Based on the results, our patient was started
on appropriate therapy. The CT scan of the sinuses showed near complete opacification of the
paranasal sinuses, with an air–fluid level in the right maxillary sinus suggesting acuity. Hyperdense
secretions were noted in the ethmoid air cells, with lacy septations and mucosal thickening of the
sphenoid sinuses. A biopsy of the right nasal turbinate was performed. Nasal mucosal cultures
were negative. Nasal mucosal pathology showed nonkeratinized squamous mucosa with focal mild
inflammation, fibrovascular tissue with numerous mucous glands, and mild chronic inflammation.

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