Interstitial lung disease (ILD) ppt slideshare

Interstitial lung diseases (ILDs) are a group of more than 200 different disorders that cause scarring in the lungs. Scar tissue in the lungs can make it harder for you to breathe normally. In ILDs, scarring damages tissues in or around the lungs’ air sacs and airways.

1. Presented by : Sonam

2. Interstitial lung disease Interstitial lung disease (ILD) or DPLD (diffuse parenchymal lung disease ) is a large group of diseases that cause scarring (fibrosis) of the lungs. The scarring causes stiffness in the lungs which makes it difficult to breathe and get oxygen to the bloodstream. It mainly affects the interstitium (the tissue and space around the alveoli) of lungs. Lung damage from ILD’s is often irreversible and gets worse over time.

3. EPIDEMIOLOGY The exact incidence and prevalence of disease is not unknown. Earlier it was considered a rare disease in India but now it’s not rare according to a number of studies. It is estimated that 10-15% of the patients coming to a pulmonary physician are diagnosed with ILD. Incidence is higher in men due to occupational exposure. The average rate of survival for someone with this disease is between three and five years.

4. Sarcoidosis G rowth of tiny collections of inflammatory cells in different parts of th e body. Hypersensitivity pneumonitis   (extrinsic allergic alveolitis): inflammation of airspaces and small airways within the lungs, caused by hypersensitivity to inhaled organic dusts and moulds.

5. Idiopathic interstitial pneumonia   : R epresent the majority of cases of interstitial lung diseases (up to two-thirds of cases).- Idiopathic pulmonary fibrosis   (IPF) is t he most common subgroup. Nonspecific interstitial pneumonia  (NSIP) Respiratory bronchiolitis-associated interstitial lung disease  (RB-ILD ) Desquamative interstitial pneumonia  (DIP): characterised by mononuclear cell infiltration of the airspaces Cryptogenic organizing pneumonia  (COP) Acute interstitial pneumonia  (AIP) (Hamman-rich syndrome) Lymphoid interstitial pneumonia  (LIP): I t is a syndrome of fever, cough and dyspnea , with bibasilar pulmonar y infiltrates consisting of dense interstitial accumulations of lymphocytes and plasma cells

6. Lymangioleiomyomatosis :  abnormal muscle-like cells begin to grow out of control in certain organs or tissues, especially the lungs, lymph nodes, and kidneys. Over time, these LAM cells can destroy the healthy lung tissue. Pulmonary alveolar proteinases : rare lung disorder characterized by an abnormal accumulation of surfactant derived lipoprotein compounds within the alveoli of the lungs Langerhans cell histiocytosis : abnormal clonal proliferation of Langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. Pleural parenchymal fibroelastosis : rare condition characterized by predominantly upper lobe pleural and subjacent parenchymal fibrosis, the latter being intra alveolar with accompanying elastosis of the alveolar walls

7. CAUSES Inhalation of inorganic dust (such as crystalline silica, asbestos, and coal dust) or Inhalation of organic dust from organisms encountered in farming, Use of air conditioning and Animal husbandry Radiation damage, and Infectious agents

8. Exogenous or endogenous stimuli Dust fumes, cigarette smoke, autoimmune conditions Drugs, infection, radiation, & others Microscopic lung injury Intact Wound healing aberrant lung homeostasis Pulmonary fibrosis Genetic predisposition Auto immune conditions Dyspnea Dry cough Fatigue Pleuritic chest pain

9. CLINICAL MANIFESTATION Breathlessness (most common) Non-productive cough Fatigue Pleuritic chest pain Hemoptysis – infrequent

10. DIAGNOSTIC INVESTIGATIONS HISTORY Occupational history Personal history Medical history and Surgical history Prolonged medication therapy Radiation exposure F amily history

11. Systemic symptoms Connective tissue disease is a frequent cause of ILD Nonspecific symptoms such as night sweats, fever, fatigue or weight loss suggest an underlying inflammatory condition

12. Dermatologic symptoms Heliotrope rash Periorbital erythema with or without edema of eyelids and periorbital tissue Highly characteristic of DM

13. Dermatomyositis Gottron’s papules mechanic’s hands Systemic sclerosis: calcium deposits, puffy fingers, raynaud’s , digital ulcers and scars Systemic lupus erythematosus: malar rash, photosensitivity skin reaction, hair loss

14. Gastrointestinal symptoms Esophageal motility problems Chronic, intermittent aspiration can lead to progressive fibrotic lung disease Bloating and diarrhea

15. Musculoskeletal complaints Connective tissue disease Raynaud’s phenomenon – scleroderma, SLE and antisynthetase syndrome Swollen fingers ( sausage digits ) may be observed in systemic sclerosis and polymyositis

16. Ophthalmologic symptoms Dry eyes – S jogren syndrome Increasing edema, syncopal events, or exertional chest discomfort may indicate severe pulmonary hypertension Presence of palpitations or syncope in a patient with sarcoidosis – cardiac sarcoidosis Pleuritic chest pain, leg swelling and increasing dyspnea – consideration of acute pulmonary embolism

17. Past medical history Prior diagnosis of connective tissue disease Case of HIV disease – lymphocytic interstitial pneumonia (LIP) are common H/o acute or chronic kidney disease might suggest underlying vasculitis, pulmonary renal syndromes H/o liver disease could suggest sarcoidosis, primary biliary cirrhosis

18. Occupational history Inorganic exposure Organic exposure Medication history Nitrofurantoin Amiodarone NSAID’s H/o recent chemotherapy H/o immune-modulating drug use

19. Physical examination Pulmonary signs: tachypnea and tachycardia, even at rest Bilateral, basilar, Velcro like rales Signs of pulmonary hypertension Extrapulmonary signs: Clubbing (e.g. IPF) Skin abnormalities, peripheral lymphadenopathy, hepatosplenomegaly (sarcoidosis) Subcutaneous nodules (rheumatoid arthritis)

20. Muscle tenderness and proximal weakness (polymyositis) Inspiratory squeaks Clubbing Skin involvement- vasculitis, tuberous sclerosis Arthritis Eye changes ( uveitis, conjunctivitis ) Muscle weakness Neuropathy Lymphadenopathy

21. INVESTIGATIONS Chest radiography Nodules, linear (reticular) infiltrates, or a combination of the two (reticulonodular infiltrates) Diffuse ground glass pattern- early Cystic areas (honeycomb pattern) – late T he first indication of underlying ILD High resolution CT scan : More sensitive than chest radiograph

22. Pulmonary function test : reduced lung volumes, reduced diffusing capacity, a normal or supernormal ratio of FEV1 to FVC, static lung compliance is decreased M aximal transpulmonary pressure is increased (a very high negative pressure must be generated to open the fibrotic alveoli)

23. Laboratory testing Elevated liver enzymes or hypercalcemia – sarcoidosis Renal insufficiency – pulmonary renal syndromes Peripheral eosinophilia – chronic eosinophilic pneumonia, churg-strauss syndrome, drug reaction Bronchoscopy Useful in the diagnosis of DPLD Inspection of the upper and lower airways, bronchoalveolar lavage (BAL), and the performance of transbronchial lung biopsy.

24. BAL: Bloody lavage specimens – diffuse alveolar hemorrhage Milky white BAL fluid – pulmonary alveolar proteinosis BAL eosinophilia (>25%) – acute eosinophilic pneumonia BAL lymphocytosis- granulomatous ILD, suggestive of hypersensitivity pneumonitis, drug reaction or cellular NSIP Positive lymphocyte proliferation assay in chronic beryllium disease Asbestos bodies in asbestosis CD1A positive cells on flow cytometry may lead to a diagnosis of LCH In the immunocompromised host, BAL fluid is highly sensitive for the diagnosis of bacterial, viral, fungal, and mycobacterial disease.

25. Surgical lung biopsy Despite a high yield in certain forms of lung disease, the utility of transbronchial biopsy for most of these is low and surgical biopsy is often required for accurate diagnosis The usual technique is video assisted thoracoscopic surgery (VATS) that has a low morbidity and mortality in selected populations.

26. TREATMENT Provide symptom- relief Improve quality of life Prevent complications End of life care and palliative treatment Removal from exposures Treatment of comorbidities Palliative care Lung transplantation

27. Removal from exposures Drug reaction is suspected Mold growth, removal of birds from the home, extensive cleaning of upholstery, window coverings, and ventilation systems Occupational exposures- avoided Antifibrotic drugs Useful in progressive fibrotic lung disease Pirfenidone Stabilize lung function

28. Corticosteroids: Mainstay of therapy Prednisone , 1 mg/kg for 1 month, followed by 40 mg/day given for 2 months Gradually tapered (5 mg/week) over several months to a maintenance dose of 15 to 20 mg/day Corticosteroids are continued until pulmonary function is stable for 1 year Relapses require returning to high dose steroids

29. Immunosuppressive therapy Some forms of ILD, including COP, CTD – associated ILD, and sarcoidosis, shows favorable response to steroids and other immunosuppressive agents When a more prolonged course of therapy is anticipated, azathioprine or cyclophosphamide, permit low dose of steroids If no clinical improvement is seen after 3 to 6 months of therapy, discontinuation of immunosuppressive therapy should be strongly considered

30. Lung transplant Most patients with ILD referred for lung transplantation have IPF – advanced stage. A severely impaired DLCO (<39%) as well as survival and are considered to be triggers for active listing. Early referral to a lung transplant center is useful.

31. prognosis Some forms of ILD resolve completely, while others lead to long term and irreversible scarring and lung damage with accompanying respiratory failure. Pulmonary hypertension can develop in cases of long standing ILD and can lead to cor pulmonale. The prognosis is dependent upon the type and severity of ILD as well as the underlying health status of the patient.

32. prevention Avoid or limit exposure to toxins or treatments that can lead to ILD. Proper diet and exercise reduces chances of developing ILD. Quitting smoking and avoiding exposure to substances known to cause ILD can prevent the disorder from developing or worsening. People who are employed in jobs where they may be heavily exposed to known causes of lung disease in the workplace typically should undergo routine screening for lung disease.

33. NURSING MANAGEMENT Ineffective breathing pattern related to decreased lung compliance, decreased energy as characterized by dyspnea, abnormal ABG, cyanosis and use of accessory muscles Monitor respiratory rate, depth, pattern, pulse oximetry, and arterial blood gas. Elevate the head of the bed to at least 30 degrees . Encourage deep breathing exercise, coughing, and the use of incentive spirometry. Administer medications(sedatives) with caution that may lead to respiratory failure Observe for evidence of coughing, increasing dyspnea or pulse rate. Maintain patent airway through suction and ventilatory support .

34. Risk for decreased cardiac output related to positive pressure ventilation Assess heart rate, rhythm, level of consciousness, and hemodynamic parameters Provide bed rest to the patient to relieve symptoms Monitor ECG changes. Auscultate breath sounds and heart sounds, listen for abnormal heart sounds, murmurs, gallop sounds. Assess for stiffness of chest and consolidation.

35. Risk for impaired skin integrity related to prolonged bed rest, prolonged intubation and immobility Assess integrity of skin, risk of pressure ulcers by braden’s scale Change the position of the patient every two hourly Provide nutritious diet to the patient(high calorie, high protein diet). Provide T PN in case the patient is not able to take orally or is advised npo Provide bed bath and keep the skin dry Apply emollients and reassess the skin integrity

36. Knowledge deficit related to health condition, new equipment and hospitalization as characterized by increased frequency of questions posed by patient and significant others. Assess the knowledge level of the patient and relatives about the disease condition, the equipment used in treatment and the medication and hospitalization need of the patient. Clarify the doubts of the family members and allow them to ventilate their feelings Provide therapeutic environment and counsel the family members Encourage the family members to stay positive and ask if any query persists. Provide information on how to promote recovery with healthy diet and exercise. Teach the patient deep breathing exercise and coughing etiquettes and range of motion exercises

37. Nursing alert : nursing assessment is essential to minimize the complications related to neuromuscular blockade. The patient may have discomfort or pain but cannot communicate these sensations. In addition, frequent oral care and suctioning may be needed.

38. CONCLUSION Interstitial lung disease is a group of disease that causes progressive scarring of lung tissues. People with interstitial lung disease may experience chronic or dry cough, fatigue or inability to exercise, shortness of breath or weight loss, etc. And the treatment of patients with interstitial lung disease depends on severity and underlying cause but often includes steroids, oxygen therapy, and pulmonary rehabilitation.

39. REFERENCES Brunner and suddharths . Textbook of medical and surgical nursing. 13th edition vol. I. .New delhi : reed elsevier india pvt. Ltd.; 2014. Pg. No. 360- 395 Lewis. Medical surgical nursing. Assessment and management of clinical problems. 2015. New delhi . Elsevier vol. I. Pg. No. 461-493 Joyce M. Black and jane hokanson ; medical surgical nursing; volume 2, 8 th edition, reed elsevier , india pvt. Pg. No. 1630 Kasper, fauci , hauser , lingo, harrison’s principles of internal medicine, 19 th edition, 2015. Noida, mcgraw hill india pvt ltd. Research hyperlinks: Suzuki, atsushi et al. “The impact of high-flow nasal cannula oxygen therapy on exercise capacity in fibrotic interstitial lung disease: a proof-of-concept randomized controlled crossover trial.” BMC pulmonary medicine vol. 20,1 51. 24 feb. 2020, doi:10.1186/s12890-020-1093-2 Kreuter , michael et al. “Health-related quality of life and symptoms in patients with IPF treated with nintedanib : analyses of patient-reported outcomes from the INPULSIS® trials.” Respiratory research vol. 21,1 36. 30 jan. 2020, doi:10.1186/s12931-020-1298-1

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