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MS-PA: Respiratory Diseases > RESTRICTIVE AND FIBROTIC PULMONARY DISEASES > Flashcards

RESTRICTIVE AND FIBROTIC PULMONARY DISEASES Flashcards

1
Q

What are the causes of restrictive lung diseases ?

A
  • Idiopathic pulmonary fibrosis and Sarcoidosis.
    *Pneumoconiosis such as silicosis, asbestosis, and anthracosis.
  • ARDS and NRDS
  • Granulomatosis with polyangiitis also known as Wegnner’s granulomatosis.
  • Eosinophilic granulomatosis with polyangiitis ( Churg- Strauss Syndrome).
  • Iatrogenic and mechanical restrictions.
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2
Q

What is the pathophysiology of IPF ?

A

The pathophysiological process consist of phases of injury, inflammation, and repair. Destruction of the alveolar-capillary basement membrane leads to platelet activation and fibrin-rich clot formation. This results in chemotaxis of neutrophils which augments inflammation. The macrophages released TGF-beta promotes fibroblast proliferation and myofibroblast formation by stimulating type II alveolar cells. This increases secretion of fibrous proteins and ground substance, which form the extracellular matrix. Chronicity of this process ultimately results in irreversible fibrosis and impaired gas exchange.

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3
Q

What are the risk factors for interstitial pulmonary fibrosis ?

A
  • smoking
  • Male sex and age >40
  • Medications and radiotherapy around the chest
  • Auto-immune disorders such as RA, PM, SLE, DM, Sarcoidosis etc.
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4
Q

What are the medications that can cause IPF ?

A
  • Chemotherapeutic agents.
  • Amiodarone
    *Nitrofurantoin
    *Rituximab
    *Sulfasalazine
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5
Q

What is the epidemiology of IPF ?

A

*Men > Women
* Rarely seen before age 50
* Ireland ~ approx. 1,000 living with IPF

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6
Q

What is the clinical Hx of IPF patient ?

A

Periodic “exacerbations” on top of persistent
symptoms such as non-productive cough, exertional dyspnea, reduced exercise tolerance, malaise and weight loss.

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7
Q

What are the physical examination findings in IPF ?

A

*Bibasilar inspiratory crackles and Clubbing.

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8
Q

What is the blood work up findings in IPF ?

A
  • ABG will show reduced PaO2 and increased PCO2.
  • > CRP, +ve ANA (30%), +ve RF (10%).
    *
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9
Q

What is the classical imaging finding in PF ?

A

reticular honeycombing defined as clustered, air-filled cysts that are often of similar diameters on the order of 3-10 mm or occasionally larger. They exhibit well-defined walls that are shared by ≥ 2 cysts.

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10
Q

What are the PFT findings in IPF ?

A

restrictive pattern consists of normal FEV1/FVC ratio,Low FVC, Low TLC, and low DLCO.

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11
Q

What is the histopathology of IPF?

A

usual interstitial pneumonia

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12
Q

What is the management of IPF ?

A
  • Reduce risk factors.
  • Supplemental oxygen if hypoxia.
  • Drugs such as Pirfenidone and Nitedanib both helps to suppress immune mediated over-production of alveolar cells and collagen.
    *Pulmonary rehabilitation
  • Lung transplant
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13
Q

What is the prognosis of IPF ?

A

5 year survival rate is 50%.

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14
Q

what is the pathophysiology of sarcoidosis ?

A

Unknown antigens and genetic factors activates T helper cells and Macrophages causing inflammation due to immune cells mediated chemotaxis which results in noncaseating granulomas, the pathologic hallmark of sarcoidosis.

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15
Q

What is the structure of noncaseating granulomas seen in sarcoidosis ?

A

They are are collections of mononuclear cells and macrophages that differentiate into epithelioid and multinucleated giant cells and are surrounded by lymphocytes, plasma cells, fibroblasts, and collagen.

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16
Q

What is the cause of hypercalcemia in sarcoidosis ?

A

It may occur because of increased conversion of vitamin D to the activated form (1,25 hydroxy vitamin D) by macrophages producing 25-Hydroxyvitamin D 1-alpha-hydroxylase.

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17
Q

What are the most common locations of noncassiating granulomas of sarcoidosis ?

A

-Lungs (90%)
– Eyes
– Skin
– Lymph nodes

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18
Q

What are the systemic symptoms of sarcoidosis ?

A

fever, wt loss, fatigue

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19
Q

What are the dermatological findings in sarcoidosis ?

A

erythema nodosum, lupus pernio

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20
Q

What is dacrocystitis seen in sarcoidosis ?

A

It is the inflammation of the palpebral lobes of the lacremal glands located on the upper eyelids.

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21
Q

What are the ophthalmological symptoms in sarcoidosis ?

A

anterior uveitis and keratoconjunctivitis
sicca

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22
Q

What are the pulmonary and lymphatic manifestations of sarcoidosis ?

A

Lung: dry cough, wheezing , SoB.
Lymphatic: Splenomegaly, generalized and bilateral hillar lymphadenopathy.

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23
Q

What are the neurologic and MSK manifestations of sarcoidosis ?

A

cranial neuropathy, aseptic meningitis, mass lesions, encephalopathy, vasculopathy, seizures, hypothalamic-pituitary disorders, hydrocephalus, myelopathy, peripheral neuropathy, and myopathy and arthritis (MSK)

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24
Q

What are the cardiac manifestations of sarcoidosis ?

A

arrhythmias, conduction
abnormalities, cardiomyopathy, Sudden cardiac death.

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25
Q

What is the Chest radiographic classification of sarcoidosis ?

A

Stage 00: No abnormalities 5 to 10%
Stage 01: Lymphadenopathy 50%
Stage 02: Lympahdenopathy + pulmonary infiltration 25 to 30%
Stage 03: Pulmonary infiltration 10 to 12%
stage 04: fibrosis 5% ( upto 25% during the course of the disease.

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26
Q

What are the Ddx of hillar and mediastinal lymphadenopathy?

A

Tumors: Lung CA, lymphangitic spread of carcinoma, and lymphoma
Inhalation: Sarcoidosis, silicosis, and anthracosis.
Infections: TB or mycobacterium species.
Connective tissue disorders such as Scleroderma.

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27
Q

What is LÖFGREN SYNDROME ?

A

It is an acute sarcoidosis syndrome consisting of Erythema nodosum, Bilateral hilar lymphadenopathy, Bilateral ankle arthritis or pain, fever and malaise. It is more common in Ireland and scandinavia. It is associated with HLA-DRB1 mutation and it spontaneously resolves.

28
Q

What are the blood work up findings in sarcoidosis ?

A
  • normochromic, normocytic anaemia.
  • Elevated ESR, Ca2+, and ACE
29
Q

What are the CT chest and C-XR findings in sarcoidosis ?

A
  • Bilateral hilar lymphadenopathy and Fibrosis in CXR.
  • CT chest will show Nodules up to 10 mm in diameter, strung together
    giving “beading” appearance along airways.
  • Coalescing nodules forming mass of up to 3 cm and Fibrotic honeycombing in severe cases.
30
Q

What are the PFT findings in sarcoidosis ?

A
  • Normal if isolated hilar lymphadenopathy.
  • Restrictive pattern in severe disease
  • Mixed pattern if endobronchial involvement.
  • Reduced DLCO if fibrosis present.
31
Q

What are the Bronchoalveolar lavage findings in sarcoidosis ?

A

increased CD4+ T Lymphocytes and the Biopsy showing non-caseating granuloma.

32
Q

What is the management of pulmonary sarcoidosis ?

A

Often self-limited, good prognosis
– Can use corticosteroids for significant symptoms (dyspnea), up to one year of low-dose therapy may be needed.

33
Q

What is the management of Extrapulmonary sarcoidosis ?

A

It is commonly seen in black or asian individuals and has poor prognosis and high incidence of recurrence. The Tx is corticosteroids, methotrexate, azathioprine and/or hydroxychloroquine.

34
Q

What is pneuomoconiosis class of diseases ?

A

These are a group of diseases caused by Inhalation of mineral dust,
organic/inorganic particles,
vapor, and fumes. This results in insidiously progressive pulmonary fibrosis and the severity of disease dependent on dose, particle size and type of dust.

35
Q

What are the longterm complications of Pneumoconiosis ?

A

-Progressive respiratory failure
– Lung cancer
– Tuberculosis
– Cor pulmonale

36
Q

What are the pathological changes seen in Coal workers pneumoconiosis or black lung disease ?

A

Diffusely distributed, small focal anthracosilicosis. The fibrosis is initially centriacinar and peribronchiolar with many carbon-laden macrophages and perifocal emphysema. The extent of fibrosis depends on admixture of quartz.

37
Q

What are the different types of CWP?

A

1) Pulmonary anthracosis: Asymptomatic pigment deposits with no cellular reaction.
2) Simple CWP: macrophages accumulate with little to no pulmonary dysfunction, diffuse small nodules (1-5 mm).
3) Complicated CWP – extensive fibrosis and compromised lung function, larger nodules (> 5 mm).

38
Q

What is Caplan syndrome or rheumatoid pneumoconiosis?

A

It is the Rapid progression of CWP in patients with a history of
rheumatoid arthritis.

39
Q

What percentage of CWP progress from simple to complex CWP ?

A

< 10%

40
Q

What is the symptomatology of CWP ?

A

Symptoms range from none to mild and severe.
Mild : Dyspnea, cough, rales on auscultation.
Severe: Pulmonary dysfunction, pulmonary hypertension, and cor pulmonale.

41
Q

What are the CXR and CT findings in CWP ?

A

nodules with poorly defined
margins, “granular” appearance, and most commonly located in the upper lungs. Fibrosis in severe disease, best seen on CT.

42
Q

What is the clinical utility of MRI in CWP ?

A

It is helpful to distinguish CWP from lung CA.

43
Q

What are the preventive measures in CWP ?

A
  • Eliminate exposure
  • Respiratory masks in the field
    provide limited protection.
  • Smoking cessation
  • Pneumococcal, influenza and
    COVID vaccines
44
Q

what should be Tx approach in CWP ?

A
  • Simple CWP rarely requires
    treatment
  • Pulmonary rehabilitation
  • Supplemental oxygen may be
    required
  • Monitor for
    – Pulmonary hypertension
    – Cor pulmonale
45
Q

What is silicosis ?

A

The most prevalent chronic disease in the world due to Inhalation of crystalline silica. It causes macrophage mediated pulmonary fibrosis.

46
Q

What are the pathological changes seen in acute silicosis ( Uncommon)?

A

Alveolar lipoproteniosis and progressive diffuse interstitial fibrosis secondary to inhalation of small particulate silica crystals.

47
Q

What are the pathological changes seen in nodular silicosis ( most common type)?

A

Multiple growing silicosis nodules of 2mm to 1 cm. Fibrosing granulomoas consist of concentric fibrous layers, anthracotic pigments, small slit like spaces, and needle-shaped crystalline specules on polarization.

48
Q

What are the pathological changes in progressive massive silicosis ?

A

Multiple silicotic granulomas of > 10 cm in size seen in both lungs.

49
Q

How does silicotic nodules form?

A

. Dead macrophages release
silica into interstitial tissue around small bronchioles.

50
Q

What are the two forms of silicosis commonly seen ?

A

Simple chronic silicosis: discrete silicotic nodules that do not compromise pulmonary function
– Complicated chronic silicosis: coalescing silicotic nodules causing progressive fibrosis and reduced lung
volumes

51
Q

What is the complication of silicosis ?

A

Increased susceptibility to TB.

52
Q

What is the symptomatology of silicosis ?

A

Dyspnea with exertion progressing to dyspnea at rest with productive cough. Breath sounds will be diminished as the disease progress.

53
Q

What are the radiological findings in silicosis ?

A
  • Multiple small (1-3 mm) round nodules usually in the upper lung fields, in miliary pattern.
  • Eggshell calcifications on the hilar and mediastinal lymph nodes.
  • Nodular conglomerate masses
    indicating severe disease.
54
Q

What are the preventive measures in silicosis ?

A
  • Respiratory masks are inadequate
  • Dust suppression and proper
    ventilation
  • Surveillance:
    – Respiratory questionnaires
    – Spirometry
    – Chest x-rays
  • Smoking cessation
  • Pneumococcal, influenza and COVID
    vaccines
  • TB testing
55
Q

What is the Tx approach in silicosis ?

A

*Symptomatic tx with
bronchodilators and inhaled
corticosteroids especially if evidence of obstructive lung disease on PFT.
* Acute silicosis – oral
corticosteroids and whole lung
lavage.
* Pulmonary rehabilitation
* Lung transplant rarely needed

56
Q

What is asbestosis ?

A

It is pulmonary fibrosis mediated by macrophages as a result of inhaling asbestos fibers. It is commonly seen in nsulator, shipyard worker, construction worker approximately after 10 years of initial exposure.

57
Q

What is the risk associated to asbestosis ?

A

MALIGNANT MESOTHELIOMA

58
Q

What is the clinical presentation of asbestosis ?

A

SOB and DOE progressing to Dyspnea at rest. Bibasilar crackles can be heard early in the disease. The disease progress to significant hypoxia and core pulmonale. Pleural involvement due to mesothelioma can cause pleural effusion.

59
Q

What are the CXR findings in Asbestosis ?

A

CXR may show pleural plaques,
starting in lower lung fields and
moving superiorly as disease
progresses

60
Q

What are the CT chest and PFT findings in Asbestosis ?

A

Chest CT may show fibrosis
(honeycombing) with restrictive pattern.

61
Q

What are ferruginous bodies ?

A

These are linear asbestos bodies seen in the histology of asbastotic lung.

62
Q

What are the preventive measures in asbestosis ?

A
  • Special asbestos removal
    protocols
  • Surveillance
    – Respiratory questionnaires
    – Spirometry
    – Chest x-rays
  • Smoking cessation
  • Pneumococcal, influenza and
    COVID vaccines
  • TB testing
63
Q

What is the Tx approach in asbestosis ?

A
  • Symptomatic treatment oxygen,
    bronchodilators
  • Pulmonary rehabilitation
  • Thoracentesis for pleural
    effusions
  • Lung transplant rarely
    recommended
  • Monitor for mesothelioma
64
Q

What are malignant mesotheliomas ?

A

These are Tumour of mesothelial cells seen in pleura and 90% of the cases are reported in patients with asbestosis exposure. The exposure to onset time is 45 years ~. It has only a 4 to 18 months survival rate.

65
Q

How is malignant mesotheliomas treated ?

A

Cancer: chemotherapy ± radiation ± surgery
– Chronic / recurrent pleural effusions : pleurodesis ± intra-pleural drain

66
Q
A

MS-PA: Respiratory Diseases (15 decks)

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