Disclosures Nothing to Disclose Interstitial Lung Disease: - - PowerPoint PPT Presentation
6/22/2018 Disclosures Nothing to Disclose Interstitial Lung Disease: Decoding the Alphabet Soup Rupal J. Shah, MD MS Director, Interstitial Lung Disease Clinic University of California, San Francisco Outline What is ILD?
Farrand, E et al The hospitalized patient with interstitial lung disease: A hospitalist primer J Hosp Med 2017
Lederer DJ, et al Idiopathic pulmonary fibrosis NEJM 2018
67 yo M with progressive dyspnea and
40 pack year smoker, quit 6 years ago. On exam, late inspiratory crackles, +clubbing. CXR shows increased basilar reticulation. What additional historical information is most likely to assist in establishing a diagnosis? A. Allergy History B. Family History C. Occupational History D. Travel History
A l l e r g y H i s t
y F a m i l y H i s t
y O c c u p a t i
a l H i s t
y T r a v e l H i s t
y
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Elements Examples Demographics
Age, IPF > 50
Time course
Acute, sub-acute, chronic
Extra-pulmonary symptoms of CTD
Raynauds, rash, inflammatory arthritis, proximal muscle weakness, dry eyes/mouth
Smoking history
DIP, RB-ILD, LCH, AEP
Medications/Radiation
Nitrofurantoin, Amiodarone, methotrexate, chemotherapy, radiation 77 medications (pneumotox.com)
HP exposures (home, work, hobbies)
Avian (birds, down), molds (water damage, swamp cooler), mycobacteria (indoor hot tub, metal working fluid)
Occupational exposures
Asbestos, beryllium, metal dusts
Family history of ILD
Early graying, cryptogenic cirrhosis, bone marrow disorders Travis, WD et al An official ATS/ERS statement: Update of the international multidisciplinary classification of IIP AJRCCM 2013
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Elements Examples Face and mucus membranes
Scleritis, saliva pool & dentition, oral ulcers, leukoplakia, mouth
Lungs
Dry crackles (listen at bases or may miss early disease!) Inspiratory squeaks (bronchiolitis)
Cardiac
Signs of pulmonary hypertension
Hands
Clubbing, mechanics hands, sclerodactyly, nail bed capillaries, palmar telangectasias, Gottrons papules, synovitis and deformities, dystrophic nails
Neuro
Proximal muscle weakness Travis, WD et al An official ATS/ERS statement: Update of the international multidisciplinary classification of IIP AJRCCM 2013
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Fibrotic
Cystic
Nodular
carcinomatosis
Consolidation/GGO
Order full PFT’s
Spirometry Predicted Observed %Pred FVC 3.72 2.24 60% FEV1 3.06 1.78 58% FEV1/FVC 82 79 96% Plethysmography TLC 5.26 3.38 64% Diffusion Diffusing Capacity 29.01 8.01 28%
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Elements Comment CBC with differential Macrocytosis (telomeropathy) Eosinophilia (CEP) Autoimmune serologies Next slide HP precipitans Poor sensitivity and specificity, limited range of antigens tested Genetic testing Selected cases (e.g. BHD), emerging for FPF Telomere length measurement Emerging VEGF-D Lymphangioleiomyomatosis
Travis, WD et al An official ATS/ERS statement: Update of the international multidisciplinary classification of IIP AJRCCM 2013
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Meyer KC, Raghu G. Bronchoalveolar lavage for the evaluation of interstitial lung disease: is it clinically useful? Eur Respir J. 2011;38:761-769.
32 Hutchinson,JP et al In-Hospital Mortality after SLB for ILD in the US 2000-2011 AJRCCM 2015
History, Physical Exam, CT scan, labs Diagnosis!
IPF HP (some) LCH, AP, LAM Some occupational lung diseases
Is bronchoscopy safe and indicated?
Sarcoid Malignancy Eosinophilic pneumonia COP
No
Surgical Lung Biopsy
Bronchoscopy Diagnostic
Confident diagnosis Diagnosis of highest probability
Procedures are unsafe Non diagnostic Non diagnostic
Adapted from Wells, AU ILD guideline: the BTS with Thoracic society of Australia and New Zealand and Irish Thoracic Society Thorax 2008
Flaherty KR, King TE, Raghu G, et al. Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis? Am J Respir Crit Care Med. 2004;170:904-910.
clinicians, radiology, and pathology
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Idiopathic Pulmonary Fibrosis Connective Tissue Disease Hyper- sensitivity Pneumonitis Other Antifibrotics Immunomodulation Extrapulmonary dz / antigen avoidance
73 yo F with IPF presents to ER with increased dyspnea. SaO2 90% on 10LPM. Dry crackles at lung bases. Sputum gram stain is negative. CT shows new areas of alveolar consolidation superimposed on reticulation. Which is the most appropriate next test? A. Bronchoscopy with BAL B. Fungal serologies C. Right heart catheterization D. Swallow study
B r
c h
c
y w i t h B A L F u n g a l s e r
i e s R i g h t h e a r t c a t h e t e r i . . . S w a l l
s t u d y
progressive fibrotic interstitial lung disease that occurs in older adults and is characterized by radiographic or pathologic usual interstitial pneumonia without a secondary cause
reticulation, traction bronchiectasis and honeycombing without other features (i.e. ground glass, air trapping, etc)
– CTD, asbestosis, chronic HP, XRT
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Official ATS/ERS/JRS/ALAT Guidelines on Idiopathic Pulmonary Fibrosis. AJRCCM 2011;183:788
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Ryerson CJ et al Predicting survival across interstitial lung disease: the ILD-GAP model Chest 2014
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Nintedanib Pirfenidone
King, TE et al A phase 3 trial of pirfenidone in patients with IPF NEJM 2014 Richeldi, LR Efficacy and safety of nintedanib in IPF NEJM, 2014
6/22/2018 Presentation Title and/or Sub Brand Name Here 41 Nintedanib Pirfenidone Main Benefit
year, on average, compared to no treatment
year, on average, compared to no treatment
Other Possible Benefits
exacerbations
and risk of respiratory hospitalization
Administration
Tolerance/side effects
Safety
infarction (~1% increase)
Discontinuation (1 year)
Cost per year
history of dyspnea, dry cough, fever, chills, night sweats, and fatigue, which have not responded to treatment with antibiotics; she has lost 2.2 kg (5 lb) during that time. The patient had a thorough examination 6 months ago while she was asymptomatic that included routine laboratory studies, age- and sex-appropriate cancer screening, and a chest radiograph; all results were normal. Nonsmoker, no known environmental exposures, no recent travel or sick contacts. Meds: aspirin and a multivitamin.
examination is normal. There are scattered crackles in the mid-lung zones with associated rare expiratory wheezes. There is no digital clubbing. Musculoskeletal and skin examinations are normal. Chest radiograph is shown.
C r y p t
e n i c
g a n i z i . . I d i
a t h i c p u l m
a r y . . . N
s p e c i f i c i n t e r s t i t i . . L y m p h
y t i c i n t e r s t i t i . .
pattern of lung injury in multiple processes (infection, CTD, HP, etc)
Overlap with NSIP suggests autoimmune disease, especially myositis-related
months), relapses common, rare cases progressive/refractory
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Travis, WD et al An official ATS/ERS statement: Update of the international multidisciplinary classification of IIP AJRCCM 2013
– Autoimmune disease – Hypersensitivity pneumonitis – Medication toxicity – Can be idiopathic
Travis, WD et al An official ATS/ERS statement: Update of the international multidisciplinary classification of IIP AJRCCM 2013
– Classic imaging + good exposure – Lung biopsy
– Avian: feathers and droppings – Molds, mycobacteria, thermophilic actinomycetes – Some chemicals – Hot TUB
– Remove exposure – Prednisone +/- mycophenolate or azathioprine
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48 Systemic Sclerosis Rheumatoid Arthritis Sjogren’s Syndrome Mixed Connective Tissue Disease Dermatomyositis/ Polymyositis/ Antisynthetase SLE ILD Occurrence Likely Common Possible Common Likely Unusual ILD Type NSIP (80-90%), UIP (10-20%) UIP (50-60%), NSIP, OP, DIP NSIP (28-60%), LIP (20%) NSIP, OP NSIP, OP, NSIP/OP
NSIP, OP DAH Disease Pearl Esophgeal dysfunction PH screening Drug-induced (anti-TNF, MTX) Anti-Ssa/SSb Cysts PH screening Anti-Jo1, MDA-5 Consider
RNP
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Travis, WD et al An official ATS/ERS statement: Update of the international multidisciplinary classification of IIP AJRCCM 2013
– Fever, EN, polyathralgias, hilar lymphadenopathy – NSAID’s – 80% resolve without tx
Putman, RK et al Association between ILA and All-cause mortality JAMA 2016