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RE39 YO died due to SOB 53 YO AA male with Chest pain

RE39 YO died due to SOB 53 YO AA male with Chest pain. BMHGT 05/27/09. 53 YO with Chest Pain. Discussants : Dr.Bart Williams Dr.Butler. 39 YO AAF with SOB. 39 has DM II ; HTN ; Hx of CHF,Anemia CKD -3 with heavy proteinuria (5/08) ANA panel negative C3 C4 NORMAL

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RE39 YO died due to SOB 53 YO AA male with Chest pain

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  1. RE39 YO died due to SOB53 YO AA male with Chest pain BMHGT 05/27/09

  2. 53 YO with Chest Pain • Discussants : • Dr.Bart Williams • Dr.Butler

  3. 39 YO AAF with SOB • 39 has DM II ; HTN ; Hx of CHF,Anemia • CKD -3 with heavy proteinuria (5/08) • ANA panel negative C3 C4 NORMAL • RENAL USD Echogenic kidneys • Chronic Shortness of breath • Chronic pyuria • Chronic LE edema on diuretics

  4. Chronic Shortness of Breath History of CHF: echo normal 2/09 Lung infiltrates : Transbronchial Biopsy BOOP Started on Cytoxan PLUS STEROIDS IN 5/08

  5. Events of 05/2009 • Admitted 5/1/09 with Dx CHF • Echo normal • Diuretics stopped • Pulmonary consult obtained

  6. Test results • ESR >120 • CRP 6.3; >10 • CXR/CT Chest bilateral lung infiltrates • Echo Normal; RA –ive ; ANA –ive • MRI brain normal • ANCA –ive • Got better with steroids and Nebs and cytoxan

  7. Course of Events on 5/10/09 • Came to ER with SOB • Treated with steroids • Went home • Died following week

  8. BOOP • First described in 1901 • More cases reported by Epler in 1985 • Age incidence 4th-7th decades • Incidence 6-7/100,000 admissions • Not related to smoking

  9. BOOP • aka.. cryptogenic organizing pneumonia • Pathological entity • Excessive proliferation of granulation tissue within small airways and alveolar ducts, associated chronic inflammation ofsurrounding airways

  10. Classification Idiopathic Post infection- mycoplasma, legionella, CMV, adneovirus, influenza, chlamydia, PCP, crytococcus Drug induced-amiodarone, bleomycin, gold, dilantin, cocaine, carbamezapine Rheumatologic-SLE, RA, DM-PM, Sjogren’s, AS, Behcet syndrome, PMR Immunologic disorders- common variable immunodeficiency, essential mixed cryglobulinemia, GVHD Classification Focal nodule Bone marrow transplantation Lung transplantation Miscellaneous- HIV, XRT, myelodysplastic syndrome, lymphoma, chronic thyroiditis, alcoholic cirrhosis, IBD, tryptophan, textile dye printing, seasonal syndrome with cholestasis BOOP

  11. KEY FEATURES • Intraluminal organizing fibrosis in distal airspaces • Patchy distribution • Preserved lung architecture • Uniform temporal appearance • Mild interstitial chronic inflammation • Fibrinous exudates • Accumulation of foamy MACS in alveoli • Connective tissue polyps

  12. KEY NEGATIVE FINDINGS Lack of interstitial fibrosis Absence of granulomas Lack of neutrophils/abscesses Absence of necrosis Lack of hyaline membranes Lack of prominent eosinophil infiltration Lack of vasculitis

  13. BOOP Alveolar ducts interstitial disorder late crackles patchy infiltrates on CXR Reduced TLC and DLCO Lymphocytes in BAL Good response to RX Good prognosis BO Distal bronchioles Airflow disorder Early crackles Normal CXR Reduced FEV1, FEV1/FVC Neutrophils in BAL Poor response to Rx Poor prognosis BOOP vs Bronchiolitis Obliterans

  14. RADIOGRAPHIC FINDINGS CXR- bilateral, diffuse, patch, peripheral alveolar opacities Reticular interstitial pattern in minority of cases Ground glass opacities in > 2/3 of cases Pleural effusions , cavities, pleural thickening and honeycombing (RARE) All lung zones may be affected Severity correlates with the extent of histological involvement of respiratory and alveolar ducts HRCT- patchy air space consolidation in peripheral and lower lung zones, ground glass opacities, small nodular opacities, bronchial wall thickening and dilatation

  15. INVESTIGATIONS • BAL- higher percentage of lymphocytes, neutrophils, eosinophils, low CD4/CD8 • “mixed pattern” of cellularity • Video assisted thorascopic lung biopsy…GOLD STANDARD • Transbronchial biopsy not ideal as may miss representative lesion, and does not adequately allow exclusion of associated lesions

  16. INVESTIGATIONS Routine labs non-specific Leucocytosis-50% Increased ESR -100mm/hr or > + CRP Auto Ab (-) or in very low titre PFT’s- decreased VC with normal flow rates...mild to moderate restrictive defect, decr. DLCO

  17. TREATMENT • Spontaneous improvement is rare • Prednisone @ 1mg/kg/d for 1-3 mths, then 40mg/d x 3mths, then 10-20mg/d or every other day x 1 year • Methylprednisone 125 to 250mg Q6hx 3-5 days

  18. TREATMENT • If deterioration occurs despite steroids or if not tolerated cytotoxic agent…… cyclophosphamide 2mg/kg/d as a single dose (not to exceed 150 mg/d) • Erythromycin, inhaled triamcinolone have been used anecdotally

  19. TREATMENT • Relapses may occur when steroids withdrawn • Monitor clinically with CXR, PFTs • Normalization of CXR and clinical improvement in 2/3 of patients over weeks to months • If > 3 recurrences may require continuous prednisone, cyclophosphamide or both

  20. OUTCOMES • 1/3 pts have persistent disease • Total recovery 65 to 85% of patients • Mortality 5%

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