CLEARING THE AIR ON DIFFUSE PARENCHYMAL (INTERSTITIAL) LUNG DISEASE (ILD)

Transcription

1 CLEARING THE AIR ON DIFFUSE PARENCHYMAL (INTERSTITIAL) LUNG DISEASE (ILD) David Northrop MBA, RRT Assistant Director of Respiratory Therapy Services The University of Kansas Health System Clinical Assistant Professor The University of Kansas Medical Center

2 INSPIRE to Change the World The Kansas Respiratory Care Society is formed to: Educate, advocate, and promote the profession and practice of Respiratory Care

3 Diffuse Parenchymal (Interstitial) Lung Diseases ILD: classified as a restrictive lung disease. Restrictive lung diseases restrict lung expansion. Therefore ILD results in the inability to.

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5 Objectives: INSPIRE you to learn about interstitial lung disease (ILD) Describe IDL Define the prevalence of ILD List many of the 200+ disorders that can be linked to ILD Review the three phases of ILD Understand the anatomical alterations of ILD Discuss diagnosis and treatment of ILD

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7 Diffuse Parenchymal (Interstitial) Lung Diseases 5B4VSSX0Aj1RXNyoA?p=Interstitial+Lung+Diseases&fr= yfp-t-900&fr2=piv-web youtube.co m/watch?fe ature=playe r_embedde d&v=w_jbkj tzf0i

8 Diffuse Parenchymal Interstitial Lung Disease

9 Diffuse Parenchymal Interstitial Lung Disease

10 Diffuse Parenchymal Interstitial Lung Disease Parenchyma= Functional tissue Interstitium= Includes: alveolar epithelium, pulmonary capillary endothelium, basement membrane, perivascular and perilymphatic tissues

11 Introduction As the Alveolar/Capillary membrane is both functional and interstitial, the phrase diffuse parenchymal interstitial lung disease will be is used interchangeably as Interstitial Lung Disease (ILD).

12 1. Interstitium supports the delicate relation between the alveoli and capillaries, allowing efficient gas exchange Introduction 1. Interstitium is the tissue which divides the spaces of the lung

13 Phase 1: Trigger Three phases of ILD Causative (or unknown) reason for ILD Phase 2: Chronic inflammation Eosinophils, mast cells, macrophages, neutrophils Secrete chemical mediators Fibroblast begins re-model Phase 3: Granuloma formation/ fibrosis formation (PULMONARY FIBROSIS)

14 Phase 2: Fibrogenesis Neutrophil, LPA and Fibroblasts play a critical role in wound healing LPA: signaling molecule to stimulate cell proliferation. Fibroblast: type of cell that synthesizes the extracellular matrix and collagen.

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16 Interstitial lung disease ILD occurs when the body s repair system (Fibrogenesis) results in imperfect re-modeling AKA: Scar formation/fibrosis

17 Clinical course Acute: Hours to days Subacute: Weeks to months Chronic: Months to years

18 Acute: Clinical course Occurs suddenly/rapidly great intensity then resolves Subacute: Occurrence with poor resolution (leading to insidious presentation) relapse not as intense poor resolution Chronic: Little resolution, poor resolution Slow, progressive

19 Interstitial lung disease (ILD) Injury inflammation repair process remodeling fibrosis Clinical pattern of ILD is decline, stabilization, slight recovery, decline, stabilization, slight recovery Worsening ILD can progress slowly or quickly.

20 Interstitial lung disease (ILD) Once lung scarring occurs, it's generally irreversible. (Pulmonary Fibrosis) Medications can slow the continuing damage of interstitial lung disease, but patients don t regain the full use of their lungs.

21 Interstitial lung disease (ILD) More than 200 different disorders can lead to interstitial lung disease

22 Causes of ILD: Mayo Clinic Web Alveolar proteinosis Asbestosis Berylliosis Black lung disease BOOP (bronchiolitis obliterans organizing pneumonia) CEP(Chronic eosinophilic pneumonia) Coal worker's pneumoconiosis AKA: black lung disease Connective-tissue diseaseassociated interstitial lung disease Desquamative interstitial pneumonia Farmer's lung Hamman-Rich syndrome Hard-metal pneumoconiosis Hypersensitivity pneumonitis Interstitial lung disease Lymphangiomyomatosis, lymphangioleiomyomatosis Lymphocytic interstitial pneumonia

23 Mayo Clinic Mixed connective tissue disease Nonspecific interstitial pneumonia Organizing pneumonia, cryptogenic organizing pneumonia Pigeon breeder's disease Pneumoconiosis Polymyositis, dermatomyositis Pulmonary alveolar microlithiasis Pulmonary eosinophilic granuloma (EG), histiocytosis X (HX), or Langerhans cell granulomatosis Pulmonary phospholipoproteinosis Usual interstitial pneumonia Respiratory bronchiolitis and respiratory bronchiolitisassociated interstitial lung disease Rheumatoid Sarcoidosis Scleroderma Silicosis Lupus erythematosus

24 Causes of ILD These 200+ different illnesses and disorders fall into one of five categories 5 categories 1. Occupational/environmental 2. Treatment related a.radiation: lung/breast cancer treatment b.drugs: antibiotics, chemotherapy, etc. 3. Connective Tissue 4. Immunologic 5. Idiopathic

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26 Occupational/Environmental Long-term exposure to a number of organic and inorganic materials and agents can lead to ILD. These include: Asbestos fibers Bird protein (live pets and feather-containing products) Coal dust Grain dust Mold from indoor hot tubs, showers and prior water damage Silica dust

27 Occupational/Environmental Four factors that increase a person s susceptibility to ILD 1. Pollutant density Amount of pollutant in air Size of pollutant (0.5-5 um) 2. Duration of exposure 3. Chemical properties of the pollutant 4. The individual s susceptibility to the pollutant

28 Medications Antibiotics Treatment related Nitrofurantoin (Macrobid, Macrodantin, others) and sulfasalazine (Azulfidine) Heart medications Eg. amiodarone and propranolol Chemotherapy Eg. methotrexate and cyclophosphamide

29 Treatment related Radiation: Lung or breast cancer treatment most commonly Signs of lung damage may not show up for months to years The severity of ILD depends on: How much of the lung was exposed to radiation The total amount of radiation administered Whether chemotherapy was also used The presence of underlying lung disease

30 Connective Tissue Disease Systemic lupus erythematosus Connective tissue disease Sarcoidosis Scleroderma Rheumatoid arthritis Sjogren's syndrome Undifferentiated connective tissue disease Dermatomyositis/polymyositis Pulmonary vasculitis Many others

31 Idiopathic Interstitial Lung Disease Idiopathic Interstitial Lung Disease refers to a the scarring and fibrosis of the interstitum where a primary disorder is not identified. AKA: idiopathic interstitial pneumonia (IIP) AKA: noninfectious pneumonia AKA: Idiopathic Pulmonary Fibrosis (IPF)

32 Idiopathic Pulmonary Fibrosis (IPF) Patients with idiopathic pulmonary fibrosis are generally elderly or middle-aged Approximately two-thirds of patients are older than 60 years at diagnosis. Idiopathic pulmonary fibrosis has no specific geographical distribution; it is found in equal proportions in urban and rural environments.

33 Clinical presentation of ILD

34 Clinical presentation of ILD Breathlessness and non-productive cough are the main clinical complaint Chronic Clubbing Cyanosis Insidious presentation (gradual) onset Pulmonary function testing Restrictive disorder Decreased diffusion capacity

35 Clinical presentation of ILD Pulmonary function testing

36 Clinical presentation of ILD PFT: No airflow obstruction: typically

37 Interstitial lung disease : diagnosis Pulmonary Function Testing Decreased diffusing capacity D lco Testing measures the transfer of gas from air in the alveoli to the red blood cells carbon monoxide

38 Imaging ILD - Diagnostic Workup Abnormal CXR (most) Peripheral reticular opacities at lung bases Honeycomb (coarse reticular pattern) & volume loss

39 Bilateral lower lobe reticular opacities

40 High Resolution CT Gold standard More sensitive than CXR in identifying the characteristic features of IPF Normally, the interstitium is so thin it can't be seen on chest X-rays or CT scans. ILD presentation: Diffuse, patch, reticular opacities with irregularly thickened interlobular fissures and honeycomb changes especially in bases

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42 Classic subpleural honeycombing (red circle) in a patient with a diagnosis of IPF (<5 mm round translucencies with a density equal to air)

43 Varies due to underlying factor leading to interstitial lung disease Mean survival ILD Morbidity/Mortality Varies: 1 month to 12 years Pulmonary fibrosis: Poor prognosis Pulmonary fibrosis diagnosis 2-6 yrs from time of dx: 3 years median 60% that die from IPF: acute exacerbation Other 40% infection, lung CA, CHF, ACS, venous thromboembolic disease

44 Treatment Prevent further damage Treat the primary cause Supportive treatment Oxygen Anti-inflammatory Immunosuppressive agent Anti-fibrotic Ofev (Nintedanib) Pirfenidone (Esbriet) Lung transplantation ATS/European Respiratory Society

45 Ofev and Esbriet medication Ofev is a kinase inhibitor that works by blocking several pathways that may be involved in the thickening or scarring of lung tissue Esbriet also works on multiple pathways that may be involved in the scarring of lung tissue.

46 Esbriet medication Lost lung function cannot be restored. However, Esbriet may help preserve more of your lung function by slowing the progression of the disease : Esbriet website

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48 Future research

49 THE END I hope you are INSPIRED Understand interstitial lung disease List many of the 200+ disorders that can be linked to interstitial lung disease Describe the five categories of interstitial lung disease Explain the anatomical alterations of interstitial lung disease Explain the diagnosis and treatment of ILD

50 Questions