Interstitial lung disease

detail description of interstitial lung disease

1. RAMAIAH INSTITUTE OF NURSING EDUCATION AND RESEARCH. “SEMINAR ON INTERSTITIAL LUNG DISEASE” SUBMITTED TO: PREPARED BY Mrs. Malathi K Ms . Saheli Chakraborty . Lecturur of II year M.SC Nursing. Dept . of Medical Surgical Nursing. RINER. RINER

2. OBJECTIVES: From today’s class learners will learn about- Introduction and Definition of Interstitial lung disease. Etiology . Pathophysiology . Clinical Manifestation. Diagnostic evaluation Managemnet and nursing diagnosis of interstitial lung disease. Common interstitial lung diseases and their management.

3. INTRODUCTION : Interstitial (in- tur - Stish - ul ) lung disease describes a large group of disorders, most of which cause progressive scarring of the lung. The scarring affects the ability to breath and get enough oxygen into the blood stream. It can caused by long term exposure to the hazardous substance like asbestos. Some types of autoimmune diseases such as rheumatoid arthritis, also can cause interstitial lung disease. Once lung scarring occurs it is generally irreversible.

4. DEFINITION: Interstitial lung disease is a group of disorders that cause progressive scarring of the lung tissue. Also called as diffuse parenchymal lung disease. INCIDENCE: Fewer than 1 million cases per India. Ages affected babies, children, teenagers and young adults.

5. ETIOLOGY Exposure to hazardous material such as asbestos or coal dust. Auto immune disorder such as rheumatoid arthritis. Occupational and environmental factors: Silica dust exposure Asbestos fibre exposure Grain dust Radiation treatment

6. Use of certain medications such as- Chemotherapy drug: eg Methotrexate , Cyclophosphamide . Heart medication eg . Amiodarone , Propranolol . Antibiotics: Nitrofurantoin , ethumbutol . Anti inflammatory drugs: Sulfasalazine , rituximab . 5. Medical conditions such as- Rheumatoid arthritis. Scleroderma

7. Mixed connective tissue disease Polymyositis Sjogren’s syndrome. Saroidosis Dermatomyositis .

8. RISK FACTORS : Factors that may make interstitial lung disease includes: Age: More likely to occur in adults, although infants and children sometimes develop the disorder. Exposure to occupational and environmental toxins: Exposure to hazardous substance in the mining, farming, or exposed to pollutants. Smoking Radiation and chemotherapy Gastro esophageal reflux diseases: Uncontrolled acid reflux or indigestion may cause increase risk of interstitial lung disease

9. PATHOPHYSIOLOGY Drugs, disease and Drug mold and other Unknown sources and radiations allergens genetic factors Lung injury Inflammation Interstitial and alveolar inflammatory cells Alveolar epithelial disruption Endothelial cell damage Activation of coagulation cascade.

10. Endothelin 1 Activation of interstitial fibroblasts Cytokines Growth factors Lung epithelium damage and inflammation Lung tissue thickening and fibrosis Chronic stage of interstitial lung disease

11. CLINICAL MANIFESTATIONS Shortness of breath at rest or aggravated by exertion. Dry cough and usually non-productive. Weight loss, most often in people with cryptogenic organizing pneumonia. Breathlessness, which may get worse over time. Fatigue and inability to exercise Tachypnoea. Clubbing of fingers Basal bilateral crackles: can be mistaken for infection.

12. DIAGNOSTIC EVALUATION History collection: Occupational history, medical history, habit of smoking, medication use. Physical Examinations. Blood tests- to rule out protein, antibodies and other markers. CT scan: It can show details of fibrosis. Echocardiogram: to evaluate the amount of pressure occurring in the right side of the heart.

13. Spirometry. Oximetry. Bronchoscopy Broncho alveolar lavage . Surgical biopsy.

14. COMPLICATIONS Pulmonary hypertension Right sided heart failure (corpulmonale) Respiratory failure.

15. MANAGEMENT: Corticosteroids : Prednisone H2 receptor antagonists. Proton pump inhibitor: Omeprazole , Pantoprazole . Oxygen therapy. Pulmonary rehabilitation: It includes- Physical exercise to improve the endurance Breathing techniques that improve lung efficiency. Emotional support Nutritional counselling.

16. SURGICAL MANAGEMENT Lung transplantation: It is the last option of the treatment of interstitial lung disease. LIFESTYLE AND HOME REMEDIES: Stop smoking. Eat well balanced diet Get vaccinated for pneumonia and annual flu shot

17. Nursing management Limiting exposure of the patient to cigarette smoke and other inhaled irritants. Oxygen therapy for anaphylaxis. Supervised exercise Monitor respiratory and heart rate for any changes. Assess for changes in respiratory status such as cyanosis, pallor, changes in the level of consciousness, laboured breathing, tachypnea . Monitor ABG analysis.

18. Assess the client’s comfort level. Auscultate the lung for biphasic crackle sound. Monitor oxygen saturation level. Provide adequate rest between activities during the day. Place the client semi-fowler position.

19. Nursing Diagnosis Impaired gas exchange related to airway obstruction by alveolar inflammation and damage to the endothelium and damage to the alveolar epithelium as evidenced by shortness of breath. Ineffective airway clearance related to consolidation as evidenced by breathlessness. Imbalanced nutrition less than body requirement related to decrease intake of food as evidenced by weight loss

20. COMMON INTERSTITIAL LUNG DISEASES Sarcoidosis . Idiopathic pulmonary fibrosis Interstitial pneumonia Asbestosis. Acute interstitial pneumonitis .

21. 1. SARCOIDOSIS Sarcoidosis is a disease characterized by the growth of tiny collections of inflammatory cells ( granulomas ) in any part of the body ; most commonly in the lungs and lymph nodes. But it can also affect the eyes, skin, heart and other organs.

22. ETIOLOGY Idiopathic Family history of sarcoidosis Excessive immune response to an irritant or allergens such as- Viruses Bacteria Pollution Dust Chemicals

23. RISK FACTORS Race or ethnicity- more common in African American Age- Between 20 and 40 years of age. Gender- Women, especially over the age of 50 Family history.

24. STAGES OF SARCOIDOSIS Stage 0- No demonstrable radiographic abnormality. Stage 1- Hilar and mediastinal lymphnode enlargement without radiographic parenchymal abnormality. Stage 2- Hilar and mediastinal lymphnode enlargement and radiographic parenchymal abnormality. Stage 3- Parenchymal abnormality alone. Stage 4- Advance fibrosis.

25. PATHOPHYSIOLOGY Due to etiological factors i.e. exposure to one or more exogenous agents. Activation of macrophages. Activation of T lymphocytes Release of cytokines and other substances It promotes replication of fibroblasts

26. Granulomatous inflammation and granuloma formation. In the lungs granuloma infiltrates and fibrosis occurs. Results in low lung compliance, impaired diffusing capacity and reduced lung volume.

27. CLINICAL MANIFESTATION Hemoptysis Generalized symptoms includes anorexia, fatigue, weight loss Other signs and symptoms includes Uveitis Joint pain Granulomatous lesions of the skin, liver, spleen, kidney and CNS With multisystem involvement patient may also have fatigue, fever, anorexia, weight loss.

28. Lungs: Chronic dry cough, wheezing, shallow breath or dyspnoea, unidentifiable chest pain often radiating out. 2) Lymph node: Swelling or edema , excess build up of fluid, pain, redness, difficulty swallowing, sore throat, general exhaustion or discomfort. 3) Heart: Pain often radiating throughout the chest, fainting, palpitations, arrhythmia, dyspnea , shortness of breath. 4) Eyes: Pain or soreness, redness, sensitivity of light, blurred or cloudy vision.

29. 5) Nervous system: Facial paralysis, inflammation in the brain, seizures, meningitis, damage to small nerves. 6) Skin: Erythema , nodule formation, skin discoloration, lesions or sores. 7) Joints and connective tissue: Arthritis, loss of muscle control, loss of flexibility, generalized joint pain, generalized weakness and exhaustion.

30. DIAGNOSTIC EVALUATION: History collection. Physical examination. Chest X-rays. CT scan. Transbronchial biopsy Open lung biopsy Mediastinoscopy Biopsy Pulmonary function test: reduction in Total Lung Capacity (TLC) Arterial blood gas analysis: hypoxemia, hypercapnia .

31. MANAGEMENT :- Corticosteroids Anti-inflammatory medications Immunosuppressants . Hydrochloroquine . Tumor necrosis factor alpha (TNF- alpha ) inhibitors. Surgical management.

32. COMPLICATIONS Blindness. Kidney damage or failure Muscle paralysis Skin dysfunction and discoloration Heart conditions.

33. NURSING MANAGEMENT Assess for drug side effects , especially adverse responses to corticosteroids(such as weight gain, change in mood, and development of diabetes mellitus) Assess for manifestations of improvement , such as increased exercise tolerance, disappearance of initial assessment findings, improvement of pulmonary function studies and better oxygenation. If assessment findings worsen , document them and notify the physician.

34. 2) IDIOPATHIC PULMONARY FIBROSIS IPF is defined as a specific form of chronic, progressive, fibrosing interstitial pneumonia of unknown cause, primarily occurring in older adults, limited to the lungs. Idiopathic pulmonary fibrosis leads to irreversible decline in lung function. Also known as usual interstitial pneumonia.

35. ETIOLOGY Idiopathic Cigarette smoking Viral infections Family history Acid reflux from the stomach Environmental factors such as exposure to dusts

36. PATHOPHYSIOLOGY Environmental factors: Smoking, occupational exposure, other irritants, toxins, viral infection. Lung Epithelium at risk: age, genetics, telomerase mutations, surfactant mutations, MUC 5B variant. Persistent epithelial injury/ activation Innate and adaptive immune response Pro- fibrogenic factors eg . TNF alpha, TGF-B etc. Abnormal intracellular signaling . Proliferation, Collagen production. Fibrosis.

37. CLINICAL MANIFESTATIONS Dry non-productive cough on exertion. Progressive exertional dyspnoea Shortness of breath with exercise Crackles on auscultation. Clubbing of the fingers Impaired gas exchange Chronic hypoxemia

38. DIAGNOSTIC EVALUATION History collection Physical examination Chest X-ray. CT scan- to produce a very detailed image of the lungs. Breathing tests- to measure how well patient can breath in and out Bronchoscopy - to analyse the lung tissue. Pulmonary function test -may reveal decreased lung volumes Lung biopsy Thoracotomy Thoracoscopy Broncho alveolar lavage - may reveal malignancy , infections, eosinophilic pneumonia

39. MEDICAL MANAGEMENT: There is no effective therapy for IPF. Oxygen therapy Pulmonary rehabilitation: it includes exercise training, nutritional modulation, occupational therapy, education and psychosocial counselling Medication: N acetyl cysteine - helps break up mucus in the lungs Pirfenidone and Nintedanib - slows down the scarring into the lungs Morphine SURGICAL MANAGEMENT : It include Lung Transplant.

40. 3) INTERSTITIAL PNEUMONIA Interstitial pneumonia is a disease in which the mesh like wall of the alveoli become inflamed, the plura might become inflamed as well. Thus results in progressive scarring of both lungs. The scarring involves the supporting framework ( interstitium of the lung)

41. ETIOLOGY Idiopathic Systemic sclerosis/ Scleroderma Rheumatoid arthritis Asbestosis. Prolonged use of medications such as nitrofurantoin or amiodarone . Dermatomyositis Mixed connective tissue disease Chronic hypersensitivity pneumonitis . Radiation Drug toxicity Hermansky – Pudlak Syndrome (very rare)

42. CLINICAL MANIFESTATION Slowly progressive dyspnoea. Non productive cough for a period of months. Clubbing of fingers. End inspiratory crackles on auscultation. Hypoxemia at rest or with exercise. Shortness of breath

43. DIAGNOSTIC EVALUATION History collection Physical examination CT scan of the chest. Chest X-ray High resolution computed tomography Histology- Honey comb lung, lobar volume loss.

44. MANAGEMENT : Oxygen therapy Pirfenidone - helps in slow progression Lung transplant. PROGNOSIS: Progressive in nature. Leading to respiratory failure. Long term survival is poor. Death occurs without lung transplant.

45. 4) ASBESTOSIS Asbestosis is long term inflammation and scarring of the lungs due to heavy asbestos fibres inhalation over a long period of time. Asbestosis symptoms can range from mild to severe, and usually do not appear until many years of continued exposure. ETIOLOGY: Breathing in asbestos fibres over a long period of time.

46. RISK FACTORS: Asbestos miners Aircraft and auto mechanics. Boiler operator Electricians Railroad workers Building construction workers.

47. PATHOPHYSIOLOGY Inhalation of asbestos dust over a long period of time. Asbestos fibre lodge within the alveoli Partial obstruction of lung wall. Obstructive emphysema of the lobe. Infection and inflammation of the lobe. Abscess formation Involvement of pleura. Extension of chest wall. Activation of macrophages and phagocytosis

48. Release of mediators Interstitial fibrosis Lung injury Mesothelial cell Spreading of fibrous tissue and compress the underlying structure Stiffness of the lungs Mesothelioma (Cancer of the pleura) Metastatis Shortness of breath

49. CLINICAL MANIFESTATION: Shortness of breath Clubbing of fingers A persistent dry cough Loss of appetite with weight loss Chest tightness or pain. Late symptoms: Respiratory failure Inspiratory crackles. Alveolar wall thickening Right sided heart failure ( Corpulmonale )

50. DIAGNOSTIC EVALUATION History collection: Occupational history, smoking. Physical examination : Inspiratory crackles. Chest X ray. CT scan Pulmonary Function test Bronchoscopy .

51. MEDICAL MANAGEMENT: There is no known cure available for asbestosis. Oxygen therapy Nebulised bronchodilators Immunization against pneumococcal pneumonia, influenza vaccination. Quitting of smoke. Cancer screening. Supportive therapy includes- Physiotherapy SURGICAL MANAGEMENT : Lung transplant.

52. COMPLICATION: Lung cancer. PREVENTION: Reduce the exposure to asbestos exposure.

53. 5) ACUTE INTERSTITIAL PNEUMONITIS Acute interstitial pneumonitis , also known as Hamman Rich Syndrome, is a rapidly progressive non infectious interstitial lung disease of unknown etionlogy . It is considered the only acute process among the idiopathic interstitial pneumonia. There is no known cause or cure.

54. ETIOLOGY: Idiopathic acute respiratory distress syndrome. Neutrophil mediated lung injury. CLINICAL MAIFESTATION: Initial symptoms: Cough, fever, chills, malaise, arthalgia , myalgia , Highly productive cough with expectorant of thick mucus. Breathing difficulty. Respiratory failure in later stge.

55. PHASES OF ACUTE INTERSTITIAL PNEUMONITIS: There are 3 phases. Acute exudative phase : Peak at 4 days. Interstitial and air space become edematous , haemorrhage occurs, fibrinous exudate forms. Organizing phase : Peak at 2-3 weeks. Proliferation of fibroblasts, vessel become thickened. Fibrotic stage : after 3-4 weeks. Extensive, dense fibrotic remodelling, possible honeycombing.

56. DIAGNOSTIC EVALUATION: History collection. Physical examination. Chest X ray. Biopsy- diffuse alveolar damage. Blood test and blood cultures Bronchoalveolar lavage .

57. MANAGEMENT Supportive care is the mainstay of treatment. Mechanical ventilation. Steroids. Antibiotics PROGNOSIS: > 60 % Mortality. Most death occurs within the first 6 month.