1. Western States Pediatric Pulmonary Case Conference May 26th, 2010
Presented by
Kathryn Akong, MD,PhD
Pediatric Pulmonology Fellow, UCSD

2. Our Case
Setting: December, inpatient consultation requested by UCSD pediatric hospitalist
Patient: 16 yo, presenting with fever, dyspnea and pleuritic chest pain
history of Crohn’s disease
recent admission for fever, abdominal pain; discharged 4d prior to this admission

3. Pertinent History
Diagnosed with Crohn’s disease 15mo prior to admission
diagnosis made by endoscopy, and biopsies (pan-colitis, crypt abscesses)
complicated by recurrent pancreatitis, thought to be related to IBD flares
required chronic steroids and infliximab for remission
started infliximab 6 mo ago, weaned off steroids 4 mo ago
last dose infliximab given 2 mo prior to admission

4. Pertinent History No clinical or laboratory evidence of IBD flare
Recent admission 1 week prior for abdominal pain
No clinical or laboratory evidence of IBD flare
Remained clinically stable, no respiratory distress or hypoxia
Infliximab dose held due to concern for possible infection
Discharged with 3d course of ciprofloxacin for possible UTI

5. Pertinent History
Medications: Amitriptyline qHS, Omeprazole qday, Infliximab IV q2mo
SH: Parents separated, lives with mom and sister. No tobacco smoke exposure.
FH: Maternal grandmother has RA, mother has DM2, uncle has MS

6. CXR at prior admission

7. CT Abdomen—Lung Cuts
“Peripheral nodular lung infiltrates, right greater than left, likely due to infectious process, possibly opportunistic”

8. CT Chest

9. CT Chest What should be done next?
“Innumerable sub-pleural/peripheral nodular-reticulonodular opacities noted most prominently in the lung bases with more confluent dependent opacification of lung bases”
What should be done next?

10. CT-Guided Needle Biopsy
“Severe acute and chronic inflammation, with small foci of necrosis consistent with abscess.
No micro-organisms identified with special stains”

11. Now What? Discharged home with close follow up in GI and ID clinic
No empiric antibiotics given, only 3d course of ciprofloxacin for possible UTI
Discharged home with close follow up in GI and ID clinic

12. Case Continued
Re-admitted 4 days after discharge, for worsening pleuritic pain and dyspnea
Pulmonary consultation on hospital day #1:
new fevers to 102, and non-productive cough at home
denied abdominal pain, diarrhea or hematochezia
normal oxygen saturation on RA, on supplemental oxygen for dyspnea
Afebrile, RR 20’s
decreased breath sounds at bilateral bases

13. CXR
“Development of small bilateral pleural effusions and bibasilar opacities representing compressive atelectasis and/or pneumonia”

14. CT chest *
“Multiple mass lesions in lungs with new bibasilar consolidations and bilateral pleural effusions. Many of the masses were not present on previous examination, and masses noted previously have enlarged.”

15. Multiple Lung Nodules Malignancy Infection Inflammatory
Metastatic solid tumors
Non-Hodgkins lymphoma
Primary pulmonary lymphoma
Infection
Abscesses
Bacteremia
Recurrent aspiration
Septic Emboli
Septic thrombophlebitis
Endocarditis
Fungi
Histoplasmosis
Coccidioidomycosis
Cryptococcus
Invasive Aspergillosis
Inflammatory
Wegener’s granulomatosis
Rheumatoid Arthritis
Lymphomatoid Granulomatosis
Amyloidosis
Sarcoidosis
Pulmonary AVMs
Pneumoconioses
Paragonimus

16. Laboratory Studies ESR >140, CRP 7.0 Electrolytes normal 12
Albumin 3.7
AST 60
ALT 110
Alk phos 602
Amylase, lipase normal 21
555 36
Elevated, stable from prior
80P/1B/12L
ANA, other autoimmune serologies all negative

17. Microbiology Studies CT guided needle biopsy:
Bacterial culture: negative
Fungal culture: negative
AFB smear/culture: negative
MTD NAAT: negative
Cocci serology: negative
Quantiferon test: negative
HIV antibody: negative
Bronchoalveolar lavage fluid:
Bacterial,fungal cultures: negative
AFB smear/culture: negative
MTD NAAT: negative
Pleural fluid:
Bacterial,fungal cultures: negative
AFB smear/culture: negative
MTD NAAT: negative

18. Lung Wedge Biopsy *
“Granulomatous inflammation with microabscess formation”
Pathologist comment: Necrobiotic granulomas in the lung have been reported in the literature in the context of Crohn disease (European Respiratory Review 2009; 18:111, 47-50). The inflammation in the current case shows more necrosis and abscess formation than described in this article, making an infectious etiology more of a consideration. However, histologic stains for bacteria, fungi and acid-fast organisms (AFB and FITE) are negative on the tissue sections. Please correlate with microbiologic culture results.

19. Final Answer?
Granulomatous Pulmonary Nodules related to Crohn’s Disease

20. Crohn’s Disease
Immune-mediated inflammatory disease, can affect any portion of GI tract
Clinically characterized by GI manifestations (abdominal pain, diarrhea, hematochezia)
Histologically characterized by non-caseating granulomas in GI tract
Up to 20% present with extra-intestinal symptoms, such as:
arthritis
uveitis
anemia
Pulmonary involvement is rare
pulmonary fibrosis
vasculitis
bronchitis
laryngotracheitis
pulmonary nodules

21. Lung Nodules in Crohn’s Disease
Unclear prevalence, only case reports available (Mayo clinic review estimates 0.1%)
Presenting symptoms include dyspnea (64%), cough (36%), fever (27%)
Can precede diagnosis of IBD
Nodules tend to be peripheral, basilar
Histology usually with some degree of granulomatous inflammation
Important to rule out any infectious etiology
Case series reported resolution of lung pathology after infliximab or steroid therapy
Casey et al, Am J Surg Pathol 2003; 27(2): 213–219
Krishnan et al, Pediatrics 2006; 117(4):

22. Pediatric Case Reports
11yo M with cough, dyspnea
14 yo F with CD, fever/cough/back pain
Al-Binali et al, Pediatr Pulmonol 2003; 36:76–80
Krishnan et al, Pediatrics 2006;117:

23. Patient Follow Up Infliximab resumed, first dose given in hospital
No evidence of infectious process
Infliximab resumed, first dose given in hospital
Clinical improvement
Seen in Pulmonary clinic 1mo later, still doing well

24. Follow Up Chest CT
3 wks later
2 mo later

25. Thank You!