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Clinicopathological Conference The Johns Hopkins Hospital December 1, 2009

Timeline. March 08: SOB, cough, pul infiltrates; Idiopathic Bronchiolitis Obliterans Organizing Pneumonia (BOOP) DxedJune 08: Successfully tapered off steroidsEarly December 08 to early Jan 09: increasing SOB, coughbilat pul infiltrates, refractory hypoxemiacorticosteroids, antibiotic start

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Clinicopathological Conference The Johns Hopkins Hospital December 1, 2009

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    1. Clinicopathological Conference   The Johns Hopkins Hospital December 1, 2009 Clinical Discussant: David B. Pearse, M.D. Pulmonary and Critical Care Medicine

    2. Timeline March 08: SOB, cough, pul infiltrates; Idiopathic Bronchiolitis Obliterans Organizing Pneumonia (BOOP) Dxed June 08: Successfully tapered off steroids Early December 08 to early Jan 09: increasing SOB, cough bilat pul infiltrates, refractory hypoxemia corticosteroids, antibiotic started

    3. Timeline Mid Jan 09: Sicker Lung bx: BOOP End Jan 09: Febrile on 100 mg/day methylprednisilone Diffuse nodular infiltrates, LLL consolidation Severe hypoxemic respiratory failure Refractory atrial arrhythmias; death

    4. Idiopathic BOOP (or Cryptogenic Organizing Pneumonia) Middle aged or older; non or ex-smokers Subacute URI presentation Persistent cough, dyspnea, fever Patchy bilateral alveolar/interstitial infiltrates Path: organizing pneumonia with granulation tissue buds in alveoli and bronchioles No other associated diseases

    5. Idiopathic BOOP 80% steroid responsive 1 or 2 relapses common during steroid taper but relapses remain steroid responsive do not affect overall mortality

    6. BOOP (or Organizing Pneumonia) Bacterial infections: Strep, Staph, Chlamydia, Legionella, Mycoplasma, Nocardia Viruses: HSV, HIV, Influenza, Parainfluenza, CMV Fungi: Cryptococcus, Pneumocystis Drugs/Toxins Connective Tissue Disease Transplantation

    7. BOOP (or Organizing Pneumonia) Bacterial infections: Strep, Staph, Chlamydia, Legionella, Mycoplasma, Nocardia Viruses: HSV, HIV, Influenza, Parainfluenza, CMV Fungi: Cryptococcus, Pneumocystis Drugs Connective Tissue Disease Transplantation

    8. Approach to Patient Initial illness likely idiopathic BOOP Consistent host and presentation Consistent transbronchial biopsy Complete response to steroid treatment

    9. Approach to Patient What was the second illness in Dec 08?

    10. Approach to Patient What was the second illness in Dec 08? Assuming this was a single illness………

    11. Second Illness: Key Findings Subacute presentation (2 weeks) Corticosteroid, cephalosporin- unresponsive Bilat upper lobe nodular interstitial onset Progressed to alveolar-filling process Fever despite 100 mg methylprednisilone Lung biopsy: ?BOOP

    12. Differential Dx of Progressive Alveolar-Filling with Respiratory Failure Pulmonary edema Infection Autoimmune Idiopathic Malignant

    13. Differential Dx of Alveolar-Filling with Respiratory Failure Pulmonary edema Infection Autoimmune Idiopathic Malignant

    14. Alveolar-Filling with Subacute Respiratory Failure Infection Autoimmune Pulmonary hemorrhage syndromes Wegener’s Granulomatosis Microscopic polyangitis Goodpasture’s Syndrome Systemic Lupus Erythematosis Idiopathic Malignant

    15. Alveolar-Filling with Subacute Respiratory Failure Infection Autoimmune Pulmonary hemorrhage syndromes Wegener’s Granulomatosis Goodpasture’s Syndrome Systemic Lupus Erythematosis Microscopic polyangitis Idiopathic Idiopathic BOOP Eosinophilic Pneumonia Desquamative Interstitial Pneumonitis Pulmonary Alveolar Proteinosis Malignant

    16. Alveolar-Filling with Subacute Respiratory Failure Infection Autoimmune Pulmonary hemorrhage syndromes Wegener’s Granulomatosis Goodpasture’s Syndrome Systemic Lupus Erythematosis Microscopic polyangitis Idiopathic Acute Interstitial Pneumonia (Hamman Rich) Eosinophilic pneumonia Desquamative Interstitial Pneumonitis Pulmonary alveolar proteinosis Malignant Alveolar cell carcinoma lymphoma

    17. Most Likely Diagnosis: Infection Case-specific requirements for infectious agent: Able to infect with near-normal immunity Subacute (weeks) presentation Bilateral upper lobe interstitial/nodular infiltrates Exacerbated by steroids, progress to resp failure Unresponsive to typical broad-spectrum antibiotics Can have BOOP or BOOP-like pathology Not routinely cultured, culture difficult or takes time

    18. Infections that Reasonably Fit Bacteria Nocardia asteroides* Mycobacterium tuberculosis Nontuberculous mycobacteria Fungi Cryptococcus neoformans * Histoplasma capsulatum Blastomyces dermatitis Coccidioides immitis (Pneumocystis jiroveci *) Virus Cytomegalovirus *

    19. Differential Dx: My Short List Cryptococcus Nocardia Cytomegalovirus Progressive Disseminated Histoplasmosis Mycobacteria tuberculosis (or M. kansasii) (Pneumocystis)

    20. If BOOP was present on lung biopsy: Cryptococcus Nocardia Cytomegalovirus

    21. If BOOP was not present on lung biopsy: Favor Histoplasmosis because of calcified lung nodule

    22. Histoplasmosis Most common endemic mycosis in US After inhalation, transient RES dissemination Can see lower lobe calcified histoplasmoma Latent infection until immunity suppressed Upper lobe reactivation mimics TB Exacerbated by steroids, may not see granulomas Pericarditis and endocarditis with arrhythmias

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