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1. Interstitial Lung Disease

2. A. General Characteristics
●1. ILD is defined as an inflammatory process involving
the alveolar wall (resulting in widespread fibroelastic
proliferation and collagen deposition) that can lead to
irreversible fibrosis, distortion of lung architecture, and
impaired gas exchange.
●2. The prognosis is highly variable and depends on the
diagnosis.
● 3. Patients with environmental or occupational lung
disease, especially those with asbestosis, are frequently
involved in lawsuits against their employers.

4. 2. Environmental lung disease
●a. Coal worker’s pneumoconiosis
●b. Silicosis
●c. Asbestosis
•d. Berylliosis

5. B. Classification
1. Classified based on
pathologic and
clinical
characteristics

6. 3. ILD associated with granulomas
a. Sarcoidosis—other organs in
addition to the lungs are
involved b. Langerhans cell
histiocytosis
c. Granulomatosis with
polyangiitis (GPA, previously
known as Wegener
granulomatosis)
d. Eosinophilic granulomatosis
with polyangiitis (EGPA,
previously known as Churg–
Strauss syndrome)

7. 4. Alveolar filling disease
a.Goodpasture disease (anti-
GBM antibody disease)
b.. Idiopathic pulmonary
hemosiderosis
c. Alveolar proteinosis

8. 5. Hypersensitivity lung disease
a.Hypersensitivity
pneumonitis
b.Eosinophilic pneumonia

9. 6. Drug-induced
amiodarone,
nitrofurantoin,
bleomycin,
phenytoin,
illicit drugs

10. 7. Miscellaneous
a.Idiopathic pulmonary
fibrosis
b.Cryptogenic organizing
pneumonia (COP)
c. ILD associated with
connective tissue disorders:
rheumatoid arthritis,
scleroderma, systemic lupus
erythematosus, mixed
connective tissue disease
d. Radiation pneumonitis

12. C. Clinical Features
Symptoms :
1.Dyspnea (at first with
exertion; later at rest)
2.Cough (nonproductive)
3.Fatigue
4.Other symptoms may be
present secondary to another
condition (such as a
connective tissue disorder)

13. Signs
a.Dry “velcro-like” rales at the
bases are common
b. Digital clubbing is common
(especially with idiopathic
pulmonary fibrosis)
c. Signs of pulmonary HTN and
cyanosis in advanced disease

15. D. Diagnosis
CXR :
a. Findings are usually nonspecific.
b. Typical diffuse changes are noted
(reticular, reticulonodular,
ground glass, honeycombing)

16. 2. High-resolution CT scan
shows the extent of fibrosis
better than other imaging
modalities.

17. 3. PFTs
A restrictive pattern is noted: FEV1 /FVC ratio is
normal or increased. All lung volumes are low.
Both FEV1 and FVC are reduced, but the ratio is
often preserved.
b. Low diffusing capacity (DLCO).
4. Oxygen desaturation during
exercise.
5.Bronchoalveolar lavage (fluid for
culture and cytology)—use is controversial
because results are quite variable.

18. 6. Tissue biopsy.
a. This is often required in patients with
ILD, though high-resolution CT can often
be used to make the diagnosis without the
need for biopsy.
b. This can be done via fiberoptic
bronchoscopy with transbronchial biopsy
(a limited amount of tissue can be
obtained, which limits its utility), open
lung biopsy, or video-assisted
thoracoscopic lung biopsy.
7. Urinalysis,
if there are signs of glomerular injury (for
Goodpasture syndrome and GPA)

19. Interstitial Lung Diseases Associated
With Granulomas
Sarcoidosis
General characteristics a. A chronic systemic granulomatous disease
characterized by noncaseating granulomas, often involving multiple organ
systems. Lungs are almost always involved. Etiology unknown.
b. Occurs most often in the African American population, especially
women. c. Seventy-five percentage of cases occur when the individual is below 40 year of
age
d. Sarcoidosis carries a good prognosis in majority of patients.

20. 2. Clinical features
Constitutional symptoms Malaise,
fever, anorexia, weight loss Symptoms
vary in severity and may be absent in
many patients
. b. Lungs:
dry cough, dyspnea (especially with
exercise), chest pain c.
Skin (25% of cases)
Erythema nodosum Plaques,
subcutaneous nodules, maculopapular
eruptions

21. Eyes (25% of cases)—
may result in significant visual impairment Anterior
uveitis (75%) Posterior uveitis (25%) Conjunctivitis e.
Heart (5% of cases) Arrhythmias Conduction
disturbances, such as heart block Sudden death f.
Musculoskeletal system (25% to 50%)
Arthralgias and arthritis Bone lesions
Nervous system (5%) Cranial nerve VII involvement
(Bell palsy)
Optic nerve dysfunction
Papilledema
Peripheral neuropathy

22. Up to two-thirds of patients with sarcoidosis experience resolution/
improvement of symptoms over several years. Approximately 20% of
patients develop chronic disease. From 10% to 20% of patients are
asymptomatic but have CXR findings
Cardiac disease is the most common cause of death, although it is not a common
finding.

23. 3. Diagnosis
Diagnosis is based on clinical,
radiographic, and histologic findings.
CXR
—Bilateral hilar adenopathy is the
hallmark of this disease but is not
specific; it is seen in 50% of cases
Four stages have been described based
on CXR findings
c. Skin anergy with tuberculin skin test
—typical finding but not diagnostic

24. Sarcoid. There are bilaterally enlarged hilar lymph
nodes (white arrows) with a clear-space noted
between the nodes and the cardiac contours.
 

25. Staging of sarcoidosis (on CXR)
a. Stage I: bilateral hilar adenopathy without
parenchymal infiltrates (highest rate of remission)
b. Stage II: hilar adenopathy with parenchymal
infiltrates
c. Stage III: diffuse parenchymal infiltrates
without hilar adenopathy (least favorable
prognosis)
d. Stage IV: pulmonary fibrosis with
honeycombing and fibrocystic parenchymal
change

26. d. Angiotensin-converting enzyme (ACE)
is elevated in serum in about 50% to 80% of patients. This test helps
support the diagnosis. However, other pulmonary diseases may cause an
elevation in this enzyme (lacks sensitivity and specificity).
Hypercalciuria and hypercalcemia are common.
transbronchial biopsy: Must see noncaseating
granulomas By itself is not diagnostic because noncaseating
granulomas are found in other diseases Must be used in the context of
clinical presentation g.
PFTs:
decreased lung volumes (VC and TLC), decreased DLCO (diffusing
capacity for carbon monoxide), decreased or normal FEV1 /FVC ratio

27. 5. Treatment
a. Most cases resolve or significantly improve spontaneously in 2 years and do not
require treatment.
b. Systemic corticosteroids are the treatment of choice. The indications for treatment
are unclear. However, patients who are symptomatic or have active lung disease,
pulmonary function deterioration, conduction disturbances, or severe skin or eye
involvement should be treated.
c. Methotrexate or other immunosuppressive agents can be used in patients with
progressive disease refractory to corticosteroids.

28. B. Pulmonary Langerhans Cell Histiocytosis
Chronic interstitial pneumonia caused by abnormal proliferation of histiocytes
(related to Langerhans cells of the skin).
2. Most patients (90%) are cigarette smokers.
3. Variants of disease include eosinophilic granuloma (localized to bone or lung),
and two systemic forms—Letterer–Siwe disease and Hand–Schüller–Christian
syndrome.
4. Common findings include dyspnea and nonproductive cough.
5. Other possible manifestations are spontaneous pneumothorax, lytic bone
lesions, and diabetes insipidus.
6. CXR has a honeycomb appearance, and CT scan shows cystic lesions.
7. The prognosis and course are highly variable. Corticosteroids are sometimes
effective. Lung transplantation may be necessary.

29. C. Granulomatosis With Polyangiitis
(Previously Wegener Granulomatosis)
Rare disease with unknown etiology.
2. Characterized by necrotizing granulomatous vasculitis.
3. Affects vessels of lungs, kidneys, upper airway, skin, and sometimes
other organs.
4. Manifestations of the disease include upper and lower respiratory
infections, glomerulonephritis, and pulmonary nodules.
5. The gold standard for diagnosis is tissue biopsy, but if the patient tests
positive for c-antineutrophil cytoplasmic antibodies, the likelihood of
having this condition is high.
6. Treatment usually includes immunosuppressive agents and
glucocorticoids.

30. D. Eosinophilic Granulomatosis With
Polyangiitis
(Previously Churg–Strauss Syndrome)
Granulomatous vasculitis is seen in patients with asthma.
2. Typically presents with pulmonary infiltrates, rash, and eosinophilia.
3. Systemic vasculitis may result in skin, muscle, and nerve lesions.
4. Diagnosis: Based on clinical findings in association with significant
eosinophilia.
5. Associated with perinuclear antineutrophil cytoplasmic antibody.
6. Treat with systemic glucocorticoids.
Antineutrophil cytoplasmic antibodies (ANCA) and associated ILD: c-ANCA: GPA or
Wegener granulomatosis; p-ANCA EGPA or Churg–Strauss syndrome; may also be
positive in anti-GBM antibody or Goodpasture disease

31. Environmental Lung Disease/
A. Coal Worker’s Pneumoconiosis
1. Most have simple coal worker’s pneumoconiosis, which usually causes no significant
respiratory disability.
2. Some patients may develop complicated pneumoconiosis, which is characterized by
fibrosis (restrictive lung disease).
3. Causes: inhalation of coal dust, which contains carbon and silica.
Pneumoconiosis is defined as the accumulation of dust in the lungs, and the tissue
reaction to its presence. Dusts that have been implicated: silica, beryllium, asbestos, coal
dust, graphite, carbon black, aluminum, talc.

32. B. Asbestosis
1. Characterized by diffuse interstitial fibrosis of the lung caused by
inhalation of asbestos fibers; predilection for lower lobes.
2. Develops insidiously many years (>15 to 20 years) after exposure.
3. Increased risk of bronchogenic carcinoma (smoking is synergistic) and
malignant mesothelioma.
4. Symptoms and physical findings are nonspecific
. 5. Diagnosis made based on clinical findings and history of exposure to
asbestos.
6. CXR shows hazy infiltrates with bilateral linear opacities and may show
pleural plaques (especially in lower lung regions).
7. No specific treatment is available.
Classic CXR findings in environmental lung disease: Asbestosis: pleural
plaques Silicosis: “eggshell” calcifications

34. C. Silicosis
Localized and nodular peribronchial fibrosis (upper lobes more common).
2. Can be acute (massive exposure leading to rapid onset and death), or
chronic (symptoms years after exposure—up to 15 years or longer).
3. Associated with an increased risk of TB.
4. Sources include mining, stone cutting, and glass manufacturing.
5. Exertional dyspnea is the main symptom; cough with sputum is also
seen.
6. There are restrictive pulmonary function abnormalities.
7. Treatment is supportive and involves removal from exposure to silica

35. cutting

36. D. Berylliosis
1. Like silicosis, berylliosis has acute and chronic forms.
2. Acute disease is a diffuse pneumonitis caused by massive exposure to beryllium.
3. Chronic disease is very similar to sarcoidosis: granulomas, skin lesions, and
hypercalcemia may be present.
4. The beryllium lymphocyte proliferation test is a useful diagnostic blood test.
. Give glucocorticoid therapy for both acute and chronic berylliosis.

37. Interstitial Lung Disease Associated With
Hypersensitivity
Hypersensitivity Pneumonitis (Extrinsic Allergic Alveolitis)
Inhalation of an antigenic agent to the alveolar level induces an immune-mediated
pneumonitis. Chronic exposure may lead to restrictive lung disease.
2. A variety of organic dusts and chemicals have been implicated.
3. The presence of serum IgG and IgA in the inhaled antigen is a hallmark finding,
although many may have these antibodies without developing disease.
4. The acute form has flu-like features (e.g., fever, chills, cough, dyspnea).
CXR during the acute phase shows pulmonary infiltrates.
5. The chronic form is more insidious and more difficult to diagnose.
6. Treatment involves removal of the offending agent and sometimes glucocorticoids.
causes of hypersensitivity pneumonitis:
Farmer’s lung (moldy hay)
Bird-breeder’s lung (avian droppings)
Air-conditioner lung Bagassosis (moldy
sugar cane)
Mushroom worker’s lung (compost)

38. B. Eosinophilic Pneumonia
Fever and peripheral eosinophilia are features.
2. Eosinophilic pneumonia may be acute or chronic.
3. CXR shows peripheral pulmonary infiltrates.
4. Treatment with glucocorticoids is usually very effective, but relapses may occur

39. Alveolar Filling Disease
A. Anti-GBM Antibody or Goodpasture Disease
1. Autoimmune disease caused by IgG antibodies directed against glomerular and
alveolar basement membranes (type II hypersensitivity reaction).
2. Results in hemorrhagic pneumonitis and glomerulonephritis.
3. Ultimately, renal failure is a complication of proliferative glomerulonephritis.
4. Usually presents with hemoptysis and dyspnea.
5. Diagnosis made by tissue biopsy, serologic evidence of antiglomerular
basement membrane antibodies.
6. Prognosis is poor; treat with plasmapheresis, cyclophosphamide, and
corticosteroids.

40. B. Pulmonary Alveolar Proteinosis
Rare condition caused by accumulation of surfactant-like protein and
phospholipids in the alveoli.
2. Usually presents with dry cough, dyspnea, hypoxia, and rales.
3. CXR typically has a ground-glass appearance with bilateral alveolar infiltrates
that resemble a bat wing shape.
4. Lung biopsy is required for definitive diagnosis.
5. Treatment is with lung lavage and granulocyte colony–stimulating factor.
6. Patients are at increased risk of infection, and corticosteroids should not be
given

41. Miscellaneous Interstitial Lung Diseases
Idiopathic Pulmonary Fibrosis
1. General characteristics a. Etiology unknown; more common in men
and smokers.
b. Presents with gradual onset of progressive dyspnea, nonproductive
cough.
c. This is a devastating and unrelenting disease.
Although the prognosis is variable, the mean survival is only 3
to 7 years after first diagnosis.

42. 2. Diagnosis
CXR: ground-glass or a honeycombed appearance; may be normal.
b. High-resolution CT of chest: bibasilar reticular opacities,
honeycombing, and traction bronchiectasis.
c. Definitive diagnosis requires open lung biopsy consistent with
usual interstitial pneumonia.
d. Other causes of ILD must be exclude

43. 3. Treatment.
Options are limited. The majority of patients (>70%) do not improve with therapy
and experience progressive and gradual respiratory failure.
The following can benefit some patients:
Supplemental oxygen.
b. New anti-fibrotic agents such as nintedanib or pirfenidone have been shown in
trials to slow progression of mild and moderate disease.
c. Corticosteroids have been used historically but with little or no benefit and
have significant side effects.
d. Lung transplantation.

44. B. Cryptogenic Organizing Pneumonitis (COP)
An inflammatory lung disease with similar clinical and radiographic features
to infectious pneumonia.
2. Associated with many entities (viral infections, medications, connective
tissue disease). Most cases are idiopathic.
3. Features: cough, dyspnea, and flu-like symptoms; bilateral patchy infiltrates
on CXR.
4. Antibiotics have not been found to be effective.
5. Spontaneous recovery may occur, but corticosteroids are used most
commonly (>60% of patients recover).
6. Relapse may occur after cessation of steroids (requiring resumption of
steroids).

45. C. Radiation Pneumonitis
Interstitial pulmonary inflammation—occurs in 5% to 15% of patients who
undergo thoracic irradiation for lung cancer, breast cancer, lymphoma, or
thymoma. Mortality and morbidity are related to the irradiated lung
volume, dose, patient status, and concurrent chemotherapy.
2. Acute form occurs 1 to 6 months after irradiation; chronic form develops
1 to 2 years later—characterized by alveolar thickening and pulmonary
fibrosis.
3. Features: low-grade fever, cough, chest fullness, dyspnea, pleuritic chest
pain, hemoptysis, acute respiratory distress.

46. 4. CXR is usually normal.
5. CT scan is the best study: diffuse infiltrates (hallmark)
with straight-line effect conforming to area of radiation,
ground-glass density, patchy/homogeneous consolidation,
pleural/pericardial pleural effusions. It is excellent for
detecting recurrent cancer in irradiated area.
6. The treatment of choice is corticosteroids for
symptomatic patients. Prophylactic treatment is not useful
in humans (useful only in mice).

48. DR.OSAMA ALNATSHEH