Western States Pediatric Pulmonary

Case Conference

Sunil Kamath MD

4-20-11

Mentor: Daneli Salinas MD

History of Present Illness

• 8 year old African American female with a history of

chronic cough for 2 years presents to the CHLA ED with

fever (102.4), abdominal pain, and hemoptysis for 1day

• Hemoptysis

– First episode

– Brown/red tinged mucus

Pertinent History

• Chronic cough:

– Began 2 years prior to admission (PTA)

– Waxing and waning course

– Wet cough with associated chest congestion

– When productive, sputum was yellow/green

– Usually treated by PMD with antibiotics for bronchitis

– Occasional wheezing

– No respiratory distress or hospitalizations

• Diagnosed with asthma 3 months PTA

– Started on budesonide daily and albuterol as needed

– No improvement in symptoms

Pertinent History

• 3 weeks PTA patient diagnosed with Scarlet fever (fever, red eyes,

sore throat, rash with subsequent desquamation, and + “throat test”)

– Treated with Amoxicillin

• Review of systems:

– Rare snoring

– Sweats at night but does not soak the sheets

– No travel outside of the country

– No apnea or cyanosis

– No sinusitis or otitis media

– No fevers, body aches, or joint pain

– No hematuria

– No weight loss

Pertinent History

• Birth History

– Full term normal spontaneous vaginal delivery

– No complications

• Surgeries: none

• No known drug allergies

• Family History: No history of cardiopulmonary disease

• Social History: Lives with great-grandma(adopted mom), grandma, godmother,

5yo biological brother also adopted by this family, and an uncle

– No pets or smokers

– Uncle was incarcerated 2 years ago for a few months

Physical Exam

Temp 37.2 C

HR 146 beats per minute

RR 26 breaths per minute

BP 129/72 mmHg

SpO2 98 % breathing room air

Height 128cm (25-50%)

Weight 24kg (10-25%)

BMI 15kg/m2 (10-25%)

Physical Exam

GENERAL lying in bed eating in no apparent distress

HEENT

normocephalic/atraumatic; TM clear bilaterally; no nasal polyps

no tonsillar hypertrophy

NECK trachea is midline

+ cervical lymphadenopathy (<1cm, mobile, non-tender)

THORACIC CAGE symmetric rise

LUNGS: decreased aeration in the left lower lobe

no wheezes, crackles, or rhonchi

HEART regular rate and rhythm

no murmurs, rubs, or gallops

ABDOMEN soft, non-tender, nondistended

no HSM

EXTREMITIES mild clubbing

no cyanosis or edema; 2+ pulses

SKIN dry hypopigmented patches on face and back

Labs

WBC 25.65 K/uL

HGB 12.2 g/dL

HCT 34.8 %

PLT 272 K/uL

Segs 71 %

Bands 16 %

Lymph 10 %

Eos 0 %

Chem 8 WNL

ESR 100 mm/hr

CRP 21.1 mg/dL

What is your assessment and plan?

• Summary: 8 year old African American female with

a history of chronic cough for 2 years presents

with fever, abdominal pain, and hemoptysis for

1day.

– Cervical lymphadenopathy

– Decreased breath sounds in the left lower lobe

– Mild clubbing

– CXR with infiltrates

– Elevated white count and inflammatory markers

Differential Diagnosis:

chronic cough with hemoptysis • Infectious:

– MTB, NTM, bacterial pneumonia, etc..

• Bronchiectasis secondary to:

– cystic fibrosis, PCD, or chronic infection

• Rheumatologic disease:

– Goodpasture's syndrome, Wegners granulomatosis, SLE, JRA

• Cardiac:

– Pulmonary hypertension, mitral stenosis, chronic heart failure

• Idiopathic pulmonary hemosiderosis

Diagnostic Plan

• Evaluate for infectious etiologies:

• resp culture, fungal culture, AFB x3, sputum AFB PCR, PPD, HIV Ab,

cocci, histo, ASO, mycoplasma titers…

• ECHO

• Sweat Cl test

• Complete PFTs

• CT chest w/ and w/o contrast

• Bronchoscopy with BAL

• Evaluate for Rheumatic etiologies

Results

ECHO Normal

CBG 7.44/28//19

Sweat Cl 12 and 19 mEq/L

PPD Negative

AFB Negative X3

ASO Negative

HIV Ab Non-reactive

Quant, Tb Gold Intermediate

Aspergillus RAST Negative

Histo Ag urine Negative

Cocci Ab Negative

Blastomyces Ab Negative

Mycoplasma Ab IgG + 3.77

(0.91-1.09)

Mycoplasma Ab IgM +1798

(770-950)

Respiratory Culture Strep

Pneumoniae

ACE Normal

Smith Ab Negative

RNP Ab Negative

Glomerular BMAb Negative

C-ANCA Negative

P-ANCA Negative

ANA Negative

NBT Normal

QUIGs Normal

Complement Normal

PFT

FVC 101 %predicted

FEV1 97 %predicted

FEF25-75% 118 %predicted

VC 97 %predicted

RV 112 %predicted

TLC 101 %predicted

N2 Delta/L 1.1%

DLCO/VA 6 ml/mHg/min/L

O2 sat 98%

PETCO2 34 torr

Chest CT

•Bilateral areas of

poorly defined ground

glass nodularity

•Area of dense

consolidation in the

left lower lobe

•Possible areas of

underlying interstitial

lung disease

Bronchoscopy Results

Bronchoscopy Trachea and mainstem bronchi normal. Very mild

mucosal erythema. Secretions were thin and clear.

Nasal cilia brush biopsy Insufficient sample

BAL cytopathology Few Macrophages and epithelial cells in a mucinus

background. No evidence of viral inclusions or

microorganisms.

BAL Fluid Hazy Light Pink

RBC 1920

WBC 90 (Segs 38%, Lymph 11%, Mono 32%)

Respiratory Culture Strep Viridans

Fungal Culture Negative

Viral Culture Negative

TB PCR Negative

Respiratory Viral Panel Negative

Hospital Course

HD#1 Started on Ceftriaxone and Azithromycin

HD#2 Fevers resolved

HD#3 Hemoptysis and cough improving

HD#4 Bronchoscopy performed

HD#5 •Patient is doing well and clinically stable for discharge

•Do we know what caused this patient’s illness?

•Should we send her home?

•What is the next step?

HD#11 Thorascopic lung biospy of the left lower lobe

Lung Biospy Results

•ORGANIZING PNEUMONIA

•FIBROBLASTIC PLUGS

•MILD CELLULAR INTERSTITIAL

PNEUMONITIS

•MILD CHRONIC BRONCHIOLITIS

•RECENT HEMORRHAGE

•NO VASCULITIS

•NO GRANULOMAS

•FEW EOSINOPHILS

Lung Biospy Results

•FIBROBLASTIC FOCI

•RECENT

HEMORRHAGE

H&E

Trichrome

Management

• Cryptogenic Organizing Pneumonia (COP)

– Solumedrol 15mg/kg/dose IV Q month for 3-6 months

• Discharge plan:

– Xopenex HFA twice a day

– Calcium and Vitamin D

– Will need stress dose of steroids for illnesses

• Follow up:

– Received second month of steroids

– Cough improved

– No hemoptysis

– First follow up appointment this month

Cryptogenic Organizing

Pneumonia (COP) • Epidemeology:

– 6-7/1000

– Mean age 58years

• Etiology:

Clinical Atlas of Interstitial Lung Disease. Sharma. 2006

• Clinical Symptoms:

– May mimic community-acquired PNA

– Mild fever, nonproductive cough, sweats, anorexia,

fatigue, weight loss, and mild dyspnea

– Hemoptysis is rare

• Physical Exam:

– Inspiratory crackles

– Wheezing and clubbing are rare

• Labs:

– Neutrophilia, ↑ ESR, ↑CRP

Cryptogenic Organizing

Pneumonia

Cryptogenic Organizing

Pneumonia

• PFT

– Mild restrictive disease w/o obstruction

– ↓DLCO

• CXR

– bilateral, bibasilar, peripheral, and sometimes migratory

patchy alveolar pattern

• HRCT

– consolidation and ground-glass pattern

Cryptogenic Organizing

Pneumonia

• BAL

– moderate increase of lymphocytes, neutrophils, and

eosinophils

• Histopathology

– excessive proliferation of granulation tissue within small

airways(i.e., proliferative bronchiolitis) and alveolar

ducts

– chronic inflammation in surrounding alveoli

Cryptogenic Organizing

Pneumonia

• Treatment:

– Steroids (3-6 months)

• Prednisone 1-2mg/kg/day

• Methylprednisolone 15-30mg/kg/day Qmonth

• Immune modulators

• Outcome

– Complete disappearance of infiltrates in 65-85% Rx

with steroids

– Relapses are common

Bronchiolitis Obliterans vs

Cryptogenic Organizing

Pneumonia

Pediatric Respiratory Medicine. Taussig. 2008

Steroids Poor response Good response

Outcome Poor Good

BO vs COP

Williams KM et al. JAMA. 2009;302.No3.:306-314 Clinical Atlas of Interstitial Lung Disease. Sharma. 2006

Mosaic hypoluciencies

indicative of air trapping

Patchy consolidation and

ground-glass opacities

BO vs COP

Lynch D A et al. Radiology 2005;236:10-21 Pediatric Respiratory Medicine. Taussig. 2008

Bronchial airway lumen

obliteration by submucosal

fibrosis

Loose plugs of connective

tissue in an alveolar duct

()and adjacent alveolar

spaces

References

1. Epler GR, Colby TV, McLoud TC, et al. Bronchiolitis obliterans

organizing pneumonia. N Engl J Med. Jan 17 1985;312(3):152-8

2. Cordier JF. Cryptogenic organising pneumonia. Eur Respir

J. 2006;28(422).

3. Kwan, Ali. Bronchiolitis Obliterans Organizing Pneumonia. Emedicine.

4. King T. Cryptogenic Organizing Pneumonia. Uptodate.

5. Al-Ghanem Sara, Al-Jahdali Hamdan, Bamefleh, Khan Ali Nawaz.

Bronchiolitis obliterans organizing pneumonia: Pathogenesis, clinical

features, imaging and therapy review. Annals of Thoracic Medicine.

Vol3. Iss 2. Aptril-June 2008.

6. White KA, Ruth-Sahd LA. Bronchiolitis obliterans organizing

pneumonia. Crit Care Nurse. 2007; 27:53-66.

7. Epler GR. Bronchiolitis obliterans organizing pneumonia. Arch Intern

Med. Vol 161. Jan 22, 2001.

THANK YOU