Disclosures Timely Topics in I have nothing to disclose! Pulmonary Medicine Lorriana Leard, MD Associate Professor of Clinical Medicine Chief of Clinical Operations UCSF Pulmonary, Critical Care, Allergy & Sleep Medicine Lorriana.Leard@ucsf.edu Overview � Cases with new management strategies that you might face in practice….or just want to Case of Shortness of Breath know about…. 1
Case of Shortness of Breath Case of Shortness of Breath PE: 114/73 HR 70 RR 16 57 year old man presents with mild O2 Sat 96% RA (93% walking) dyspnea on exertion x 2 month, no cough Inspiratory crackles at both bases Normal cardiac exam, no JVD, no edema � PMH: GERD No clubbing, joint deformities, rashes � Meds: Omeprazole 40mg daily � SH: lifetime non-smoker, pharmacist � FH: no family history of lung disease What is next best step in Case of Shortness of Breath management? Lasix A. Levofloxacin B. 71% C. PPD D. HRCT scan Echocardiogram E. 24% 5% 0% 0% x n D n i m s i P a a c a a P c L s r x g o T C o l i f R d o v H r a e c L o h c E 2
CXR is Not Useful for Differentiating ILD’s Case of Shortness of Breath FVC 5.10 (83%) FEV1 4.42 (90%) HP FEV1 / FVC 0.87 IPF TLC 7.40 (88%) DLCO 23.41 (57%) CTD- NSIP ILD HRCT Scan Specific HRCT Findings � ILD protocol � Honeycombing � Inspiratory plus expiratory images � Reticulation � Prone as well as supine � Ground Glass Opacities � Traction Bronchiectasis � Air trapping � Nodules � Emphysema 3
HRCTs are essential in ILD Case of Shortness of Breath • Pattern of abnormality on HRCT scan may suggest a specific ILD • HRCT findings guide subsequent diagnostic tests • HRCT findings may be sufficient for diagnosis Case of Shortness of Breath Case of Shortness of Breath 4
Case of Shortness of Breath Case of Shortness of Breath What is the best thing to tell a Case of Shortness of Breath patient at this point? Prednisone at 1 mg/kg should be started A. to try to reverse this disease. There is no available treatment. Most B. 69% patients live 3-5 years from time of diagnosis. You should be seen by a specialist and C. your case discussed with a 28% multidisciplinary team before any treatment is started. 3% 0% You should have a lung biopsy to D. establish the diagnosis. . . . . . . . . . . . b . s e a r g t y g k n e b u / g b l n l m e a a l e 1 i s e a v t v e a a a b h e o d d n n l l o u u s o s i o i h h n e d r s s e u e u r h o o P T Y Y 5
ILD Classification Multidisciplinary Approach to Diagnosis Pulmonary Fibrosis Assessment Information 1.0 Step Method Provided 0.9 Exposure-related: Idiopathic Connective Other: 1 Individual HRCT - Occupational interstitial tissue disease: - Sarcoidosis 0.8 - Environmental pneumonia (IIP) - Scleroderma - Vasculitis/Diffuse alveolar 0.7 HRCT, clinical - Avocational - Rheum arthritis hemorrhage 2 Individual 0.6 data Idiopathic - Medication - Sjogrens - Langherhans cell pulmonary - MCTD histiocytosis 0.5 Discussion HRCT, clinical fibrosis - Dermatomyositis - Lymphagioleiomyomatosis 0.4 3 (clinician & (IPF) data - Pulmonary alveolar radiologist) 0.3 proteinosis - Eosinophilic pneumonias 0.2 Individual Desquamative interstitial Respiratory bronchiolitis - Neurofibromatosis pneumonia (DIP) interstitial lung dis. (RBILD) (clinician, HRCT, clinical 0.1 4 radiologist & data, SLB 0 Acute interstitial Cryptogenic organizing pathologist) 1 2 3 4 5 pneumonia (AIP) pneumonia (COP) HRCT, clinical 5 Discussion Nonspecific interstitial Lymphocytic interstitial data, SLB pneumonia (NSIP) pneumonia (LIP) Modified from: Flaherty KR, et al. AJRCCM. 2004;170:904-910. Differentiating Diseases Don’t stop with a diagnosis of Predicts Prognosis “Pulmonary Fibrosis” � Reasons for a specific diagnosis: 1950-90’s: Lumpers 2000: Splitters DIP � Many forms are treatable � Treatment depends on the diagnosis � Prognosis varies /IPF � Eligibility for clinical trials Bjoraker et al, AJRCCM ‘98 6
Case of Shortness of Breath Idiopathic Pulmonary Fibrosis � Average survival from diagnosis: 2.5-3 years � Afflicts men more than women � No apparent race or ethnic predilection � HRCT: subpleural, basilar predominant reticulation, � Path: UIP pattern honeycombing, and traction bronchiectasis Systematic Review / Meta-Analysis What do you recommend? Prednisone, Azathioprine and N- A. Acetylcysteine (NAC) Nintenadib B. 76% C. Pirfenidone D. Palliative care consultation as there is no treatment for this disease 17% B or C E. 3% 3% 0% b e C n . d i . r . o . o r i a . p n d t B i a o e n t i l h n t e u f s t i r a N i n z P o A c , e e r n a o c s e n i v i d t e a r l i P l a Canestaro WJ, et. al. CHEST 2016; 149(3):756-766 P 7
Systematic Review / Meta- Analysis Conclusions � Pirfenidone and Nintedanib ASCEND � are first treatments shown to slow rate of disease progression (FVC decline) � Long term mortality impact not clear King et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. NEJM 2014;370:2083 ASCEND ASCEND: Study design � Enrolled 555 highly-selected patients with IPF As sessment of Pirfenidone to C onfirm E fficacy � Randomized to pirfenidone or placebo x 52 wks a nd Safety in Idiopathic Pulmonary Fibrosis � Performed in response to an FDA request for an additional trial to support approval 1° : Pirfenidone � Designed to enrich subjects for disease FVC n = 278 progression (as measured by change in FVC) IPF 52 wks 2° : n = 555 6MWT distance; Placebo PFS; n = 277 dyspnea; death King. NEJM 2014;370:2083 King. NEJM 2014;370:2083 8
ASCEND: Subjects ASCEND: 1°Endpoint Relative difference = 45% Baseline Pirfenidone Placebo P value < 0.001 (n=278) (n=277) Age, years 68 68 Male sex 80% 77% FVC 68% 69% DLCO 44% 44% 6MWT distance 415 421 Dyspnea (UCSD) 34 37 Definite UIP HRCT 96% 95% King. NEJM 2014;370:2083 King. NEJM 2014;370:2083 INPULSIS: Study design � Enrolled 1066 patients with IPF/likely IPF INPULSIS � Randomized (3:2) to nintedanib/placebo for 52 wks nintedanib 1° : n = 638 FVC Richeldi et al. Efficacy and safety of IPF nintedanib in idiopathic pulmonary 2° : 52 wks n = 1066 acute fibrosis. NEJM 2014;370:2071 exacerbation; Placebo QOL; n = 423 death Richeldi NEJM 2014;370:2071 9
INPULSIS: Subjects INPULSIS: 1°Endpoint Baseline INPULSIS 1 INPULSIS II Nintedanib Placebo Nintedani Placebo b Age, years 67 67 66 67 Male sex 81% 80% 78% 78% FVC 80% 81% 80% 82% Mean difference 109.9 (71.3, 148.6) DLCO 48% 48% 47% 46% P value < 0.001 Oxygen sat 96% 96% 96% 96% SGRQ score 40 40 40 39 Richeldi NEJM 2014;370:2071 Richeldi NEJM 2014;370:2071 But what about when the ILD Summary disease still does progress.. � Don’t stop at “pulmonary fibrosis” � And now a word about Lung Transplant… � History is essential � HRCT scans are a key diagnostic tool � The diagnosis of ILD should be made using a multidisciplinary approach � Treatment depends on the specific type of ILD � there are now 2 treatments available for IPF 10
Lung Transplant for IPF Lung Transplant � Should be � ECLS / ECMO supports patients considered in all to LT patients < 70 years � Ex vivo perfusion systems support donor lungs to LT � 50-60% 5 year survival after transplant Bjoraker et al, AJRCCM ‘98, Neurohr et al, Transplant International, ‘10 Case of Recurrent Pneumonia � 31 year old man originally from Mexico Case of Recurrent prevents for evaluation. Pneumonia � history of recurrent pneumonias from a young age. � hospitalized 4 times in the past 3 years � His chief complaint is dyspnea and cough productive of brown mucous, and chest pain. He can walk about 3 blocks. He's limited by dyspnea, fatigue and palpations. 11
Case of Recurrent Pneumonia CXR � VS: BP 129/79 HR 90 T 36.7° C RR 20 SpO2 97% BMI 18.6 kg/m2 � Chronically ill appearing, cachectic, and frail � No sinus tenderness, No LAD � RRR, S1 and S2 present � Lungs with rhonchi bilaterally, prolonged expiratory phase, reduced air movement � Digital clubbing What is next best step to Spirometry establish the diagnosis? 11/19/14 Sweat Chloride Test A. Sputum : CF PCR for common mutations BMI 18.6 B. -AFB Culture negative x 3 C. A1AT genotype 37% FVC 1.69 D. Bronchoscopy 30% (36%) Echocardiogram 20% E. FEV1 0.98 13% (25%) FEV1/FVC 57.76 0% t e y m s e . . p p . y o a T a r t t c g e u o s d n o o m h i i e d o r g c r n n a l o T h o c C m A r o 1 B h m a t A c E e o w c r S o f R C P F C 12
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