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 HR RR BP SpO2 37.2 C 146 beats per minute 26 breaths per minute 129/72 mmhg 98 % breathing room air Height 128cm (25-50%) Weight 24kg (10-25%) BMI 15kg/m 2 (10-25%)
Physical Exam GENERAL HEENT NECK THORACIC CAGE LUNGS: HEART ABDOMEN EXTREMITIES SKIN lying in bed eating in no apparent distress normocephalic/atraumatic; TM clear bilaterally; no nasal polyps no tonsillar hypertrophy trachea is midline + cervical lymphadenopathy (<1cm, mobile, non-tender) symmetric rise decreased aeration in the left lower lobe no wheezes, crackles, or rhonchi regular rate and rhythm no murmurs, rubs, or gallops soft, non-tender, nondistended no HSM mild clubbing no cyanosis or edema; 2+ pulses dry hypopigmented patches on face and back
Labs WBC HGB 25.65 K/uL 12.2 g/dl HCT 34.8 % PLT 272 K/uL Segs 71 % Bands 16 % Lymph 10 % Eos 0 % Chem 8 ESR CRP WNL 100 mm/hr 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
Infectious: Differential Diagnosis: chronic cough with hemoptysis 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
Evaluate for infectious etiologies: Diagnostic Plan 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 NBT Normal QUIGs Normal Complement Normal ACE Normal Smith Ab Negative RNP Ab Negative Glomerular BMAb Negative C-ANCA Negative P-ANCA Negative ANA Negative PPD AFB ASO HIV Ab Quant, Tb Gold Aspergillus RAST Histo Ag urine Cocci Ab Blastomyces Ab Negative Negative X3 Negative Non-reactive Intermediate Negative Negative Negative Negative Mycoplasma Ab IgG + 3.77 (0.91-1.09) Mycoplasma Ab IgM +1798 (770-950) Respiratory Culture Strep Pneumoniae
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% P ET CO2 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 Bronchoscopy Results Nasal cilia brush biopsy Insufficient sample BAL cytopathology BAL Fluid Respiratory Culture Fungal Culture Viral Culture TB PCR Respiratory Viral Panel Trachea and mainstem bronchi normal. Very mild mucosal erythema. Secretions were thin and clear. Few Macrophages and epithelial cells in a mucinus background. No evidence of viral inclusions or microorganisms. Hazy Light Pink RBC 1920 WBC 90 (Segs 38%, Lymph 11%, Mono 32%) Strep Viridans Negative Negative Negative Negative
Hospital Course HD#1 HD#2 HD#3 HD#4 HD#5 Started on Ceftriaxone and Azithromycin Fevers resolved Hemoptysis and cough improving Bronchoscopy performed 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 H&E FIBROBLASTIC FOCI Trichrome RECENT HEMORRHAGE
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
Epidemeology: 6-7/1000 Mean age 58years Etiology: Cryptogenic Organizing Pneumonia (COP) 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 Cryptogenic Organizing Pneumonia Physical Exam: Inspiratory crackles Wheezing and clubbing are rare Labs: Neutrophilia, ESR, CRP
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 Steroids Poor response Good response Outcome Poor Good Pediatric Respiratory Medicine. Taussig. 2008
BO vs COP Mosaic hypoluciencies indicative of air trapping Patchy consolidation and ground-glass opacities Williams KM et al. JAMA. 2009;302.No3.:306-314 Clinical Atlas of Interstitial Lung Disease. Sharma. 2006
BO vs COP Bronchial airway lumen obliteration by submucosal fibrosis Loose plugs of connective tissue in an alveolar duct ( )and adjacent alveolar spaces Pediatric Respiratory Medicine. Taussig. 2008 Lynch D A et al. Radiology 2005;236:10-21
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.
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