Interstitial Lung Disease ILD

1. Interstitial Lung DiseaseILD

2. General description • ILDs represent a large and heterogeneous group of lower respiratory tract disorders. • There are similar clinical signs and X-ray features.

3. The characteristic of clinical signs including: • Dyspnea after exercising • chest X-ray shows diffuse abnormality of pulmonary parenchymal,including nodules,linear(reticular) infiltrates • pulmonary function tests shows restrictive hypoventilation reduced diffusing capacity • tissue biopsy shows a variety pulmonary fibrosis and aveolar inflammation

4. Clinical Classification of ILD • known cause • unknown cause (IIP,ects)

5. Pathogenesis • The pathogenesis of ILDs is unknown. • But more and more facts have shown that immune cells and their cytokines play an important role in the course of ILDs.

6. Nowadays the major courses of the ILDs including: • Intra-alveolar inflammation • immune cells and their cytokines injure epithelial and endothelial cells • intra-alveolar fibrosis/alveolar collapse

7. In the course of ILDs, many cytokines, including TGF-, IGF-, prostaglandin E2, platelet-derived growth factor, ects, involve in.

8. Clinical manifestations • Breathlessness, Progressive respiratory insufficency • cough without sputum. • Some patients may have fatigue, weight loss, joint pain.

9. Physical examinations • Bilateral basilar, crepitant velcro-like rale are found in most patients • wheezing, rhonchi and coarse rales are occasionally heard • with advanced disease, patients may have tachypnea and tachycardia • clubbing of the fingers and toes is common • At last, pulmonary hypertention and cor pulmonale may be exist

10. Chest radiography It is important method to diagnose the ILDs. The majority of ILDs cause infiltrates in the lower lung zones.

11. A diffuse ground glass pattern is seen early in the disease • when the disease progresses, a chest radiography demonstrates nodules, linear(reticular) infiltrates, or a combination of the two • at last, the infiltrates become coarser and lung volume is lost • honeycomb pattern may appear at the end of the disease

12. nodular linear

13. nodular linear

14. honeycomb ground glass pattern

15. Pulmonary function tests Pulmonary function tests of ILDs shows restrictive hypoventilation.

16. It includes: • Reduced lung volumes(vital capacity, total lung capacity) • reduced diffusing capacity • static lung compliance is decreased

17. BALF examination • the cell counts in BALF of ILDs is twice than that of normal humans • cell complements of ILDs is difference from that of normal humans • for example, the percents of neutriphils in BALF of IPF is higher than that of normal humans

18. Blood examination

19. Lung biopsy For example, TBLB(transbronchial biopsy), an open-lung or thoracoscopic biopsy are used to diagnose the ILDs

20. Idiopathic pulmonary fibrosisIPF

21. IPF is an unknown chronic interstitial lung disease.Nowadays It has become a common disease. • the clinical manifestations, and some experimental examination including pulmonary function tests,chest radiography examinations and lung biopsy are coincide to that of ILDs introduced before.

22. Pathology: According to the pathologic classification, there are seven types of Idiopathic interstitial pneumonitis. • IPF-UIP(usual interstitial pneumonitis) • NSIP, nonspecific interstitial pneumonitis • DIP, desquamative interstitial pneumonitis • RBILD, respiratory brnchiolitis associated interstitial lung disease • LIP, lymphocytic interstitial pneumonitis • COP, concealed organizing pneumonia • AIP, acute interstitial pneumonitis

23. How to diagnose IPF • According to the clinical signs and some experimental examinations, we can diagnose the IPF except some known cause ILDs • lung biopsy is an only way to give a last diagnosis

24. Clinical Diagnostic Standard of IPF • Except known cause ILDS • Lung function • HRCT • TBLB and BAL • Age • Unexplained dyspnea after exercise • Period • Physical examination

25. Chest radiography

26. Chest radiography

27. Pathologic Diagnosis • The pathologic diagnosis of IPF is coincide with UIP

28. Treatment of IPF • Nowadays, the treatment ways of IPF are lack of effective ways • corticosteroids are the main therapy • the initial treatment of choice is prednisone 0.5mg/kg of ideal body weight per day. For 1 month, the dose is gradually tapered over several months to a maintenance dose of 0.125 mg/kg per day

29. Immunosuppressive agents,including CTX, MTX • lung transplantation

30. Treatment • Some common therapies, including oxygen therapy, antibiotic therapy when pulmonary infections exist.

31. prognosis

32. Sarcoidosis

33. Definition • Sarcoidosis is a disease of unknown cause and is characterized by the presence of non-caseating granulomas in one or more organ, system. It is considered a systemic disease • Usually lungs and the lymph nodes in the mediastinum and hilar regions are the most site of involvement • The clinical course is quite variable asymptomatic

34. The cause of sarcoidosis is unknown. But many researchers have suggested that immune mechanisms are important in disease pathogenesis.Genetic factor may also play an important role.

35. Other factors including infectious and environmental or occupational may also involve in the sarcoidosis.

36. Pathogenesis • Antigen processing by macrophages is believed to trigger an oligoclonal expansion of CD4(helper-inducer) lymphocytes of the Th1 phenotype with production of IL-2 and IFN-. • IL-2 cause proliferation of more CD4 cells, elaborate more cytokines. • Many cytokines, mainly IL-2,adhension molecules and growth factors are released from lymphocyte and macrophages.

37. The basic pathogenesis includes three main stages: • Pulmonary alveolus inflammation • formation of non-caseating granulomas • the stage of interstitial fibrosis

39. Clinical manifestations • The clinical course is variable • the respiratory system is the most commonly affected • approximately 90% of patients demonstrate intrathoracic involvement on a chest radiograph • sometime with or without extrathoracic disease

40. Clinical manifestations • Almost 30 to 60 per cent of patients have no symptoms at the time of presentation • sometimes the disease is identified because of abnormalities on a chest radiograph • some patients present with respiratory symptoms such as dyspnea and cough, which may or may not be accompanied by constitutional symptoms, such as fever and malaise

41. Specific signs and symptoms depend on the particular organ system(s) involved • Respiratory system disease • Intrathoracic nodal involvement and parenchymal lung disease are the most common ways in which sarcoidosis affeccts the respiratory system • Hilar and medistinal lymph nodes may be affected • The pulmonary parenchyma demonstrates well defined,non-caseating granulomas with the pulmonary interstitium • Usually upper lobes of the lung tend to be more involved

42. Extrapulmonary sarcoidosis Including: • skin disease: about 20-25% of patients are involved lesions include papules, plaques, nodules and lupus pernio, erythema nodosum • eye disease and neurologic disease, liver and spleen, peripheral lymph nodes, bone lesions and myopathy

44. Experimental examinations and some specific examinations • elevations in the level of angiotensin-converting enzyme(ACE) (the normal level is 17.6-34u/ml). The measurement of serum ACE might be a useful diagnostic and prognostic test in sarcoidosis • hypercalcemia: a potentially important complication of sarcoidosis

45. PPD test: about 2/3 patients with sarcoidosis has no reaction • Kveim antigen test: we can’t usually used the test we have not standard antigen • bronchial-alveolar lavage fluid examination(BALF): the lymphocyte percent of the BALF is elevated. Usually, the percent of lymphocyte of BALF is more than 28%.It demonstrates that the disease is active

46. tissue biopsy: • an affected organ or tissue are generally used to diagnostic biopsy, including skin, lymphy node,ects. • Flexible electric bronchoscopy with transbronchial lung biopsy(TBLB) • Mediastinoscopy is sometimes performed in the presence of isolated mediastinal adenopathy

47. X-ray examination • The plain chest radiography is an important way to diagnose sarcoidosis. • The major abnormalities seen on the chest radiograph include: lymphadenopathy,usually involving both hila and mediastinal,and involvement of the pulmonary parenchyma

49. Computed tomography(CT) of the chest is used to evaluate of suspected sarcoidosis, especially there is need for better definition of mediastinal lymph node involvement. • High-resolution CT is used to demonstrate that pulmonary parenchymal involvement is localized around bronchovascular structures, producing an appearance resembling budding branches on a tree.