Transcript Western States Pediatric Pulmonary Case Conference

Western States
Pediatric Pulmonary
Case Conference
May 26th, 2010
Presented by
Kathryn Akong, MD,PhD
Pediatric Pulmonology Fellow, UCSD
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
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
Pertinent History
 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
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
CXR at prior admission
CT Abdomen—Lung Cuts
“Peripheral nodular lung infiltrates, right greater than left, likely due to infectious
process, possibly opportunistic”
CT Chest
CT Chest
“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?
CT-Guided Needle Biopsy
“Severe acute and chronic inflammation, with small foci of necrosis consistent with abscess.
No micro-organisms identified with special stains”
Now What?
 No empiric antibiotics given, only 3d course of ciprofloxacin for possible UTI
Discharged home with close follow up in GI and ID clinic
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
CXR
“Development of small bilateral pleural effusions and bibasilar opacities representing
compressive atelectasis and/or pneumonia”
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.”
Multiple Lung Nodules
Malignancy
Metastatic solid tumors
Non-Hodgkins lymphoma
Primary pulmonary lymphoma
Infection
Abscesses
 Bacteremia
 Recurrent aspiration
Inflammatory
Septic Emboli
Wegener’s granulomatosis
Septic thrombophlebitis
Rheumatoid Arthritis
 Endocarditis
Lymphomatoid Granulomatosis
Fungi
Amyloidosis
 Histoplasmosis
Sarcoidosis
 Coccidioidomycosis
Pulmonary AVMs
Pneumoconioses
Paragonimus
 Cryptococcus
 Invasive Aspergillosis
Laboratory Studies
ESR >140, CRP 7.0
12
21
555
36
80P/1B/12L
Electrolytes normal
Albumin 3.7
AST 60
ALT 110
Alk phos 602
Elevated, stable from prior
Amylase, lipase normal
ANA, other autoimmune serologies all negative
Microbiology Studies
CT guided needle biopsy:
Bacterial culture: negative
Fungal culture: negative
AFB smear/culture: negative
MTD NAAT: 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
Cocci serology: negative
Quantiferon test: negative
HIV antibody: negative
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 acidfast organisms (AFB and FITE) are negative on the tissue sections. Please correlate with microbiologic
culture results.
Final Answer?
Granulomatous Pulmonary Nodules related to Crohn’s Disease
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
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):1440-43
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;117:1440-1443
Patient Follow Up
 No evidence of infectious process
 Infliximab resumed, first dose given in hospital
 Clinical improvement
 Seen in Pulmonary clinic 1mo later, still doing well
Follow Up Chest CT
3 wks later
2 mo later
Thank You!