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.
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3. Different Causes and Pathological process
Involving interstitial alveolar structures
thus
Therapy and Prognosis
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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)
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5. Interferes with normal breathing
Loss of normal alveolar capillary gas exchange
( Ventilation / Perfusion mismatch)
Interferes with normal breathing
DYSPNEA
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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
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7. Diffuse interstitial fibrosis
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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)
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9. 3.Unclassified diseases may include:
Pulmonary histiocytosis X
LAM.
Pulmonary vasculitis.
Alveolar proteinosis.
Eosinophilic pneumonia.
BOOP.
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10. Conditions mimic interstitial lung disease
Viral Pneumonia
Pneumocystis Pneumonia
Infection
Malignancy
Leukemia, Lymphoma, Radiotherapy,
Lymphangitis carcinomatosa
Exudates (Edema)
ARDS
Hemorrhage
Goodpasteur’s
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12. Diffuse interstitial pulmonary
edema
Pulmonary edema
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13. The variable underlying etiology imply different therapy and prognosis
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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.
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16. How does interstitial lung disease occur?
Damage (Initiating stimulus is unknown) .
Inflammation ( walls of the alveoli and small blood vessels in the lungs)
Scarring (fibrosis) (the interstitium.)
The lung is
affected in
three ways:
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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.
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19. Scarred Alveoli. In interstitial lung diseases, the alveoli are distorted, scarred or fibrosis thickens their walls
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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.
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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.
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22. particularly lower zones posteriorly.
Bilateral fine late inspiratory crackles
particularly lower zones posteriorly.
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23. Central cyanosis and
digital clubbing
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24. ½ of the patients with idiopathic pulmonary fibrosis develop clubbing
Finger clubbing
½ of the patients with idiopathic pulmonary fibrosis develop clubbing
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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.
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26. Typical radiographic features of CFA with basal and peripheral
reticulonodular shadowing
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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.
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30. High resolution CT- scan
CT scan of chest showing honeycombing of the lung typical for advanced IPF
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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.
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34. Arterial Blood Gases Type ( I )respiratory Failure (Early) PO2 , P CO2
Type ( II ) Respiratory Failure (Late)
PCO2
PO2 ,
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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
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36. Complications Hypoxemia & Respiratory failure. Pulmonary hypertension.
Right-sided heart failure (cor pulmonale).
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37. Management of Interstitial (ILD) and Fibrotic Lung Disease

38. Treatment should be started early and monitored carefully
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
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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
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40. Treatment 50% improvement in symptoms
Corticosteroids
50% improvement in symptoms
25% improvement in lung function tests
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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 /kg or cyclophosphamide
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42. OTHER:
Antifibrotics colchicine 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 ???
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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.
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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.
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45. SARCOIDOSIS

46. Sarcoidosis Introduction:
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.
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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 ys old
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48. Sarcoidosis is a multisystem granulomatous disease, the organs mostly affected are :
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
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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.
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50. Erythema nodosum and arthralgia (20-30%)
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51. Skin sarcoid ( lupus pernio). (5%)
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52. Ocular symptoms (ant .uveitis & sicca syndrome).
Superficial lymphadenopathy. (5%)
Other, ( Hypercalcemia, diabetes isipidus, cranial nerve palsies, cardiac arrhythmias, and nephrocalcinosis).
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53. 90% have an abnormal chest x-ray finding at some stage of the disease; 10% have pulmonary fibrosis.

54. Lab Studies: Complete blood count Leukocytosis
With or without eosinophilia
Elevated erythrocyte sedimentation rate (ESR)
Hypercalcemia is observed in 10-15% of cases
Serum angiotensin converting enzyme (ACE).
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55. Imaging Studies: 1.Chest x-ray For diagnosis and staging.
Stage O :
No findings
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56. Stage I : Stage I Hilar adenopathy Hilar adenopathy
Often asymptomatic,
may be associated with
Erythama Nodosum Or
Arthralgia
Spontaneous resolution within one year.
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57. Stage II: Hilar adenopathy and parenchymal involvement
Hilar adenopathy and parenchymal involvement as diffuse pulmonary opacities.
Patients have cough and are breathlessness.
Spontaneous
improvement in the majority.
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58. Diffuse pulmonary shadowing without adenopathy.
Stage III Pulmonary infiltrates
Diffuse pulmonary shadowing without adenopathy.
Disease less likely to resolve spontaneously
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59. A CXR of stage III sarcoidosis, pulmonary infiltrates without evidence of mediastinal lymphadenopathy.
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60. Stage IV: Pulmonary fibrosis
Can cause progressive ventillatory failure , Pulmonary hypertension and corpulmonale.
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61. Gallium 67 scanning Used for staging of disease and to detect
extrapulmonary sarcoidosis.
Gallium bound by inflammatory tissue and
not by fibrotic tissue
Distinguish areas of fibrosis from inflammation.
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62. Other Tests:
Skin anergy with PPD ( Tuberculin Test) is common in sarcoidosis patients but obviously not specific.
Kveim- test
A suspension from the spleen or a lymph node of a patient with a confirmed diagnosis of sarcoidosis is injected intradermaly into a patient suspected to be affected by the disease.
Test results are considered positive if a nodule appears within 2-7 weeks.
The nodule is then biopsied to find similarities to sarcoid granuloma
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63. Bronchoalveolar lavage
A bronchoalveolar lavage (BAL) shows increases in the CD4/CD8 ratio, lymphocytes, and cytokines.
Biopsy is an integral part of the diagnosis and is of very high yield.
The site of biopsy is dictated by clinical presentation of the organ involved..
Pulmonary function tests (PFTs) findings consistent with restrictive lung disease.
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64. TREATMENT

65. Resolve spontaneously ..usually no treatment required.
STAGE I&II
Resolve spontaneously ..usually no treatment required.
Persistent erythema nodosum, pyrexia, arthralgia
NSAID
Hypercalcemia and ant uveitis
short course corticosteroids.
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66. STAGE III
Symptomatic pulmonary and sarcoid involving the eyes or vital organ as heart or brain
Corticosteroids starting at mg /day tapered after 6 months by 5 mg per month.
and maintained on 10 mg /day for years.
Methotrexate and hydroxychloroquin are effective steroid sparing drugs. (2nd line)
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67. Extrapulmonary Sarcoidosis
The heart can manifest with mechanical (CHF) and/or conduction defects (arrhythmia).
Diuretics and inotropics
CNS or peripheral neuropathy
systemic steroids.
Eye involvement uveitis or conjunctivitis.
Topical steroids and or systemic steroids.
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68. Skin manifestations are treated with topical or systemic steroids.
Hypercalcemia is generally responsive to IV hydration and systemic steroids..
Skin manifestations are treated with topical or systemic steroids.
Arthritis generally is treated with NSAIDs. Steroids and occasionally colchicine are reserved for severe cases.
Asymptomatic elevation of LFTs does not require treatment
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69. Lung Disease Due To Organic Dust
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70. Extrinsic Allergic Alveolitis
Organic dust inhalation causes diffuse immune complex reaction in the wall of the alveoli and bronchiooles. e.g.
Disorder
Source
Antigen
Farmer’s lung
Mouldy hay
Aspergillus fumigatus
Bird fancier’s lung
Avian excreta
Avian serum protein
Byssinosis
Textile worker
Cotton, hemp dust
Humidifier Fever
Contamination of air conditioning
Thermophilic actinomycetes
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71. Clinical Features:
Flu like symptoms within few hours of exposure e.g. headache , malaise, muscle pain, dry cough, SOB, no wheeze.
Continuous exposure SOB no systemic
effects
Irriversible Pulmonary fibrosis
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72. Lung Disease Due To Inorganic Dust Exposure
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73. Prolonged exposure to inorganic dust
Diffuse Pulmonary Fibrosis e.g.
Cause
Occupation
Description
features
Coal dust
Coal mining
Coal worker pneumoconiosis
Focal and interstitial fibrosis
Silica
Pottery,boiler scaling
Silicosis
fibrosis
Asbestos
Insulating materials
Asbestos related disease
Fibrosis, pleural disease
CA lung,larynx
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74. Asbestos associated diseases:
Respiratory diseases:
Parenchymal asbestosis
Asbestos-related pleural abnormalities
Lung carcinoma
Pleural mesothelioma
Nonrespiratory diseases:
Peritoneal mesothelioma
Rarely, cor pulmonale or constrictive pericarditis

75. Resistant to breakdown by acid, alkali, water, heat, and flame
Blue asbestos
White asbestos
Brown asbestos
Asbestos is a naturally occurring mineral, Very strong,
Resistant to breakdown by acid, alkali, water, heat, and flame
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76. Asbestos bodies
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77. Chest X-ray shows: Irregular striped opacities, most pronounced in the
basal lung segments

78. Asbestos-Related Pleural Abnormalities:
Pleural plaques.
Pleural plaques with
calcification
Pleural effusions.
Diffuse pleural thickening.

79. General approach to a patient with interstitial lung disease
Medical history
Age and sex.
ys, the most common
Sarcoidosis,
Pulmonary fibrosis associated with connective tissue disease is more common among women.
Rarely:
Histiocytosis X.
Lymphangioleiomyomatosis (LAM), only in females.
Over 50 years old ­- idiopathic pulmonary fibrosis
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80. Family history: Smoking Certain types of ILD primarily affect smokers
This can provide very useful information.
Patients with IPF disease (familial pulmonary fibrosis).
Sarcoidosis.
Smoking
Certain types of ILD primarily affect smokers
e.g. desquamative intersitial pneumonia (DIP),
respiratory bronchiolitis-associated interstitial lung disease (RB-ILD).
Sarcoidosis and EAA, occur more frequently in nonsmokers
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81. Occupational and work history.
A complete occupational history should be recorded with
details of work activities and any possible exposure to inorganic
dust.
EAA is caused by exposure to organic agents, and
Pneumoconiosis by exposure to inorganic dust
Drug history.
all current and past medications taken by the patient should be
noted, including details of the dose and duration of treatment.
Radiotherapy.
A history of chest radiation therapy could be the cause of DILD.
Systemic diseases. Identify and investigate ( rash, joint pain…)
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82. Diagnostic Tests That May Be Used to Identify Pulmonary Fibrosis or Interstitial Lung Disease
Blood Tests
Pulmonary Function Tests
Chest X-ray
CT Scan
Bronchoscopy
Bronchoalveolar Lavage
Lung Biopsy
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83. Hope For An Effective And Safe Medications
CONCLUSION
Current therapy remains with uncertain outcome and is not thought to reverse scarring that has already taken place. Early diagnosis as well as identifying and removal of occupational or environmental exposure is essential
still
Hope For An Effective And Safe Medications
In The Future

84. THANK YOU