INTERSTITIAL LUNG DISEASE & SARCOIDOSIS

1. INTERSTITIAL LUNG DISEASE & SARCOIDOSIS Dr. Ishraq Elshamli Respiratory Unit Tripoli Medical Center

2. What is interstitial lung disease : Accounts for : • 15% of the cases seen by pulmonologists . • Includes more than 130 disorders Similar symptoms, signs, radiological changes and pulmonary function tests.

3. Different Causes and Pathological process Involving interstitial alveolar structures thus Therapy and Prognosis

4. Interstitial Lung Diseases are Characterized by : • Abnormal accumulation of inflammatory cells and/or non cellular material (exudate) within the walls of the alveoli of the lungs results in • Thickening and stiffness of the normally elastic tissues of the lung, ( stiff lung)

5. Loss of normal alveolar capillary gas exchange • ( Ventilation / Perfusion mismatch) • Interferes with normal breathing • DYSPNEA

6. Normal Alveoli. Normal lung tissue showing multiple alveoli, with only a small amount of connective tissue separating the air from the capillaries containing red blood cells

7. Diffuse interstitial fibrosis

8. Causes of interstitial lung disease 1. Idiopathic Pulmonary Fibrosis 1.FIBROSIS 2.Collagen vascular disease (RA, SLE, Scl) 3.Infection (post TB) 2.GRANULOMA 4. Radiation 5. Drugs (nitrofurantoin, Bleomycin, amiodarone) 8. Sarcoidosis 6.Pneumoconiosis ( Asbestosis) 7.Extrinsic allergic alveolitis (Farmer’s lung, Bird fancier lung)

9. 3.Unclassified diseases may include: Pulmonary histiocytosis X LAM. Pulmonary vasculitis. Alveolar proteinosis. Eosinophilic pneumonia. BOOP.

10. Conditions mimic interstitial lung disease Viral Pneumonia Pneumocystis Pneumonia Infection Leukemia, Lymphoma, Radiotherapy, Lymphangitis carcinomatosa Malignancy Exudates (Edema) ARDS Hemorrhage Goodpasteur’s

12. Diffuse interstitial pulmonary edema Pulmonary edema

13. The variable underlying etiology imply different therapy and prognosis

14. Idiopathic pulmonary fibrosis(Cryptogenic Fibrosing Alveolitis)

15. Idiopathic Pulmnary Fibrosis(Cryptogenic fibrosing alveolitis) • Unknown cause. • A combination of fibrosis and alveolitis . • Isolated or associated with connective tissue disease (Rheumatoid arthritis , systemic sclerosis) • Typically Sub pleural fibrosis and honeycomb appearance in the lower lobes.

16. Damage (Initiating stimulus is unknown) . Inflammation ( walls of the alveoli and small blood vessels in the lungs) Scarring (fibrosis) (the interstitium.) How does interstitial lung disease occur? The lung is affected in three ways:

17. Inflammation and scarring of the interstitium disrupts this tissue and leads to a decrease in the ability of the lungs to extract oxygen from the air.

18. Gross pathology of small and firm lungs due to advanced pulmonary fibrosis.

19. Scarred Alveoli. In interstitial lung diseases, the alveoli are distorted, scarred or fibrosis thickens their walls

20. The course of ILDs is unpredictable The diseases may run a gradual or rapid course. Varied symptoms -- from very mild to moderate to very severe. The condition may remain the same for long periods of time or it may change quickly. 20 11/16/2014

21. Clinical Features: • Predominently a disease of the elderly >60 ys. • Progressive exertional dyspnea. • Dry cough, and digital clubbing. • Poor chest expansion, with bilateral end inspiratory crackles particularly lower zones posteriorly.

22. Bilateral fine late inspiratory crackles particularly lower zones posteriorly.

23. Central cyanosis and digital clubbing

24. Finger clubbing ½ of the patients with idiopathic pulmonary fibrosis develop clubbing

25. Investigations • Rheumatoid factor and ANA (+)ive in 30-50% of cases. • ESR is • Chest X-ray shows diffuse lower zone opacities, Honeycomb appearance in advanced lung disease. • Pulmonary function tests shows restrictive pulmonary defect.

26. Typical radiographic features of CFA with basal and peripheral reticulonodular shadowing

27. CXR 67-year-old man diagnosed with idiopathic pulmonary fibrosis, on open lung biopsy findings. Extensive bilateral reticulonodular opacities are seen in both lower lobes.

30. High resolution CT- scan CT scan of chest showing honeycombing of the lung typical for advanced IPF

31. A normal individual can expire : 75-85% of his FVC in 1 sec. 94% in 2 sec 97% in 3 sec.

32. FEV 1 The amount of air that can be blown in the first second of FVC. Normal Obstructive Restrictive

33. Pulmonary function tests shows restrictive pulmonary defect And reduced CO diffusion capacity.

34. Arterial Blood Gases • Type ( I )respiratory Failure (Early) PO2 , P CO2 • Type ( II ) Respiratory Failure (Late) PCO2 PO2 ,

35. Bronchoalveolar Lavage (BAL) cells mostly macrophages and neutrophils. BAL with high lymphocyte count better prognosis and response to treatment. Transbronchial biopsy is not recommended , small sample , patchy disease. • Open Lung Biopsy

36. Complications • Hypoxemia & Respiratory failure. • Pulmonary hypertension. • Right-sided heart failure (cor pulmonale).

37. Management of Interstitial (ILD)and Fibrotic Lung Disease

38. Disease Progression • 50% will die within 5 ys. • 75% die of respiratory problems. • Increased incidence of Bronchogenic carcinoma which is the cause of death in 10%of cases. Treatment should be started early and monitored carefully

39. The goals of treatment are: • Decrease inflammation and prevent further lung scarring. • Remove the source of the problem, when possible. • Minimize and manage potential complications of ILD. • Improve or prevent deterioration in a patients quality of life

40. Treatment Corticosteroids • 50% improvement in symptoms • 25% improvement in lung function tests

41. Indications for corticosteroids in CFA • Symptomatic patients . • Rapidly progressive disease. • Sustained fall of FVC >15% over 6 months Start combined therapy with prednisolone 0.5mg/kg and azathioprine 2-3mg /kgor cyclophosphamide

42. OTHER: • Antifibroticscolchicine and D-penicillamine have failed to show significant benefit !!. • Pirfenidone, a unique, investigational antifibrotic agent, was recently shown to have some potential benefit ?? • IFN-gamma ???

43. Other therapies? Oxygen Therapy • Oxygen therapy help relieve strain on the heart and lungs and improve symptoms of shortness of breath and fatigue. Pulmonary Rehabilitation • Helps patients to achieve their highest possible level of functioning includes: education, exercise, breathing techniques, energy saving techniques, nutritional counseling and psychosocial support.

44. Lung Transplantation Lung transplantation in selected patients is an option for some advanced cases to improve quality of life and prolong survival . • Indicated in: • Previously fit , < 60ys, • Rapidly progressive disease. unresponsive to therapy.

45. SARCOIDOSIS

46. SarcoidosisIntroduction: • A chronic non caseating granulomatous disease of unknown etiology that affects many organs and tissues, most commonly the lungs • The etiology is unknown. • High mortality in patients with extensive organ involvment.

47. Race: • The disease affects all races. • High incidence among american black than white persons 10-20:1. Sex: • Females are affected more often than males. Age: • Most commonly affect 20- 40ys old

48. Lungs (90% with abnormal chest x-ray findings at some point) Lymphatics (75% of pulmonary and 60% of peripheral) Liver (60-90%) Spleen (40%) Bone marrow (30%) Skin (25%) and Joint (25%) Eyes (25%) Upper respiratory tract (nose, tonsils in 20%) Central nervous system (5%) Heart (5%) Endocrine system Gastrointestinal system Sarcoidosis is a multisystem granulomatous disease, the organs mostly affected are :

49. Since Sarcoid lesions develop in any tissue, patient’s presentation is variable: • No symptoms; .(30%) diagnosed incidentally because of abnormal chest x-ray or abnormal liver function tests. • Respiratory & Constitutional symptoms (20-30%) • Fever/night sweats. • Malaise, Fatigue, Weight loss. • Dry cough • Dyspnea that worsens upon/with exertion.

50. Erythemanodosum and arthralgia(20-30%)