RE39 YO died due to SOBRE39 YO died due to SOB53 YO AA male with Chest 53 YO AA male with Chest
painpain
BMHGT BMHGT
05/27/0905/27/09
53 YO with Chest Pain
Discussants :Discussants :• Dr.Bart WilliamsDr.Bart Williams• Dr.Butler Dr.Butler
39 YO AAF with SOB
39 has DM II ; HTN ; Hx of CHF,Anemia39 has DM II ; HTN ; Hx of CHF,Anemia CKD -3 with heavy proteinuria (5/08)CKD -3 with heavy proteinuria (5/08) ANA panel negative C3 C4 NORMAL ANA panel negative C3 C4 NORMAL RENAL USD Echogenic kidneys RENAL USD Echogenic kidneys Chronic Shortness of breathChronic Shortness of breath Chronic pyuria Chronic pyuria Chronic LE edema on diuretics Chronic LE edema on diuretics
Chronic Shortness of Breath
History of CHF: echo normal 2/09History of CHF: echo normal 2/09Lung infiltrates : Transbronchial Biopsy Lung infiltrates : Transbronchial Biopsy
BOOP BOOP
Started on Cytoxan PLUS STEROIDS IN 5/08Started on Cytoxan PLUS STEROIDS IN 5/08
Admitted 5/1/09 with Dx CHF Echo normal Diuretics stopped Pulmonary consult obtained
Events of 05/2009Events of 05/2009
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
Course of Events on 5/10/09
Came to ER with SOB Treated with steroids Went home Died following week
BOOPBOOP
First described in 1901First described in 1901
More cases reported by Epler in 1985More cases reported by Epler in 1985
Age incidence 4Age incidence 4thth-7-7thth decades decades
Incidence 6-7/100,000 admissionsIncidence 6-7/100,000 admissions Not related to smokingNot related to smoking
BOOPBOOP
aka.. cryptogenic organizing pneumoniaaka.. cryptogenic organizing pneumonia
Pathological entityPathological entity
Excessive proliferation of granulation Excessive proliferation of granulation tissue within small airways and alveolar tissue within small airways and alveolar ducts, associated chronic inflammation ofducts, associated chronic inflammation of surrounding airwayssurrounding airways
BOOP BOOP ClassificationClassification
IdiopathicIdiopathic Post infection- mycoplasma, Post infection- mycoplasma,
legionella, CMV, adneovirus, legionella, CMV, adneovirus, influenza, chlamydia, PCP, influenza, chlamydia, PCP, crytococcuscrytococcus
Drug induced-amiodarone, Drug induced-amiodarone, bleomycin, gold, dilantin, bleomycin, gold, dilantin, cocaine, carbamezapinecocaine, carbamezapine
Rheumatologic-SLE, RA, DM-Rheumatologic-SLE, RA, DM-PM, Sjogren’s, AS, Behcet PM, Sjogren’s, AS, Behcet syndrome, PMRsyndrome, PMR
Immunologic disorders- Immunologic disorders- common variable common variable immunodeficiency, essential immunodeficiency, essential mixed cryglobulinemia, GVHDmixed cryglobulinemia, GVHD
ClassificationClassification Focal noduleFocal nodule Bone marrow transplantationBone marrow transplantation Lung transplantationLung transplantation Miscellaneous- HIV, XRT, Miscellaneous- HIV, XRT,
myelodysplastic syndrome, myelodysplastic syndrome, lymphoma, chronic thyroiditis, lymphoma, chronic thyroiditis, alcoholic cirrhosis, IBD, alcoholic cirrhosis, IBD, tryptophan, textile dye printing, tryptophan, textile dye printing, seasonal syndrome with seasonal syndrome with cholestasischolestasis
KEY FEATURESKEY FEATURES
Intraluminal organizing fibrosis in distal Intraluminal organizing fibrosis in distal airspacesairspaces
Patchy distributionPatchy distribution Preserved lung architecturePreserved lung architecture Uniform temporal appearanceUniform temporal appearance Mild interstitial chronic inflammationMild interstitial chronic inflammation Fibrinous exudatesFibrinous exudates Accumulation of foamy MACS in alveoliAccumulation of foamy MACS in alveoli Connective tissue polypsConnective tissue polyps
KEY NEGATIVE FINDINGSKEY NEGATIVE FINDINGS
Lack of interstitial fibrosisLack of interstitial fibrosis Absence of granulomasAbsence of granulomas Lack of neutrophils/abscessesLack of neutrophils/abscesses Absence of necrosisAbsence of necrosis Lack of hyaline membranes Lack of hyaline membranes Lack of prominent eosinophil infiltrationLack of prominent eosinophil infiltration Lack of vasculitisLack of vasculitis
BOOP vs Bronchiolitis ObliteransBOOP vs Bronchiolitis Obliterans
BOOPBOOP Alveolar ductsAlveolar ducts interstitial disorderinterstitial disorder late crackleslate crackles patchy infiltrates on CXRpatchy infiltrates on CXR Reduced TLC and DLCOReduced TLC and DLCO Lymphocytes in BALLymphocytes in BAL Good response to RXGood response to RX Good prognosisGood prognosis
BOBO Distal bronchiolesDistal bronchioles Airflow disorderAirflow disorder Early cracklesEarly crackles Normal CXRNormal CXR Reduced FEV1, FEV1/FVCReduced FEV1, FEV1/FVC Neutrophils in BALNeutrophils in BAL Poor response to RxPoor response to Rx Poor prognosisPoor prognosis
RADIOGRAPHIC FINDINGSRADIOGRAPHIC FINDINGS
CXR- bilateral, diffuse, patch, peripheral alveolar CXR- bilateral, diffuse, patch, peripheral alveolar opacities opacities
Reticular interstitial pattern in minority of casesReticular interstitial pattern in minority of cases Ground glass opacities in > 2/3 of casesGround glass opacities in > 2/3 of cases Pleural effusions , cavities, pleural thickening and honeycombing Pleural effusions , cavities, pleural thickening and honeycombing
(RARE)(RARE) All lung zones may be affectedAll lung zones may be affected Severity correlates with the extent of histological involvement of Severity correlates with the extent of histological involvement of
respiratory and alveolar ductsrespiratory and alveolar ducts
HRCT- patchy air space consolidation in peripheral and HRCT- patchy air space consolidation in peripheral and lower lung zones, ground glass opacities, small nodular lower lung zones, ground glass opacities, small nodular opacities, bronchial wall thickening and dilatationopacities, bronchial wall thickening and dilatation
INVESTIGATIONSINVESTIGATIONS
BAL- higher percentage of lymphocytes, BAL- higher percentage of lymphocytes, neutrophils, eosinophils, low CD4/CD8neutrophils, eosinophils, low CD4/CD8
““mixed pattern” of cellularity mixed pattern” of cellularity Video assisted thorascopic lung biopsy…GOLD Video assisted thorascopic lung biopsy…GOLD
STANDARDSTANDARD Transbronchial biopsy not ideal as may miss Transbronchial biopsy not ideal as may miss
representative lesion, and does not adequately representative lesion, and does not adequately allow exclusion of associated lesionsallow exclusion of associated lesions
INVESTIGATIONSINVESTIGATIONS
Routine labs non-specificRoutine labs non-specific Leucocytosis-50%Leucocytosis-50% Increased ESR -100mm/hr or >Increased ESR -100mm/hr or > + CRP+ CRP Auto Ab (-) or in very low titreAuto Ab (-) or in very low titre PFT’s- decreased VC with normal flow PFT’s- decreased VC with normal flow
rates...mild to moderate restrictive defect, rates...mild to moderate restrictive defect, decr. DLCOdecr. DLCO
TREATMENTTREATMENT
Spontaneous improvement is rareSpontaneous improvement is rare
Prednisone @ 1mg/kg/d for 1-3 mths, then Prednisone @ 1mg/kg/d for 1-3 mths, then 40mg/d x 3mths, then 10-20mg/d or every 40mg/d x 3mths, then 10-20mg/d or every other day x 1 yearother day x 1 year
Methylprednisone 125 to 250mg Q6hx 3-5 Methylprednisone 125 to 250mg Q6hx 3-5 daysdays
TREATMENTTREATMENT
If deterioration occurs despite steroids or if If deterioration occurs despite steroids or if not toleratednot tolerated cytotoxic agent…… cytotoxic agent…… cyclophosphamide 2mg/kg/d as a single cyclophosphamide 2mg/kg/d as a single dose (not to exceed 150 mg/d)dose (not to exceed 150 mg/d)
Erythromycin, inhaled triamcinolone have Erythromycin, inhaled triamcinolone have been used anecdotallybeen used anecdotally
TREATMENTTREATMENT
Relapses may occur when steroids Relapses may occur when steroids withdrawnwithdrawn
Monitor clinically with CXR, PFTsMonitor clinically with CXR, PFTs Normalization of CXR and clinical Normalization of CXR and clinical
improvement in 2/3 of patients over weeks to improvement in 2/3 of patients over weeks to monthsmonths
If > 3 recurrences may require continuous If > 3 recurrences may require continuous prednisone, cyclophosphamide or bothprednisone, cyclophosphamide or both
OUTCOMESOUTCOMES
1/3 pts have persistent disease1/3 pts have persistent disease Total recovery 65 to 85% of patientsTotal recovery 65 to 85% of patients Mortality 5%Mortality 5%