Restrictive Lung Disorders Flashcards by Richhpal Singh

Spirometry findings in Restrictive lung disorders

TLC decreases
FVC decreases
FEV1 - Normal or slightly decreased
FEV1/FVC Increases
Fibrosis - decreased lung compliance
Diffusion capacity decreases

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Causes of Restrictive lung disorders can be classified into

Extra parenchymal causes
Parenchymal cause

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Extra parenchymal causes in RLD includes

Chest wall disorders - Obese/ Kyphosis/ Ankylosis spondylitis
Neuromuscular disorders - Diaphragmatic palsy, Myasthenia gravis, Gullian Barre syndrome
Muscular dystrophy

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Parenchymal cause of RLD can be classified to

Acute
Chronic

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Acute parenchymal causes of RLD

ARDS

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Chronic parenchymal causes of RLD can be divided to

Fibrosing
Granulomatous
Eosinophilic
Smoking related

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Fibrosing parenchymal causes include

Idiopathic pulmonary fibrosis (IPF)
Non specific interstitial pneumonia (NSIP)
Cryptogenic organising pneumonia (COP)
Pneumoconiosis

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Granulomatous parenchymal causes includes

Hypersensitivity pneumonitis
Sarcoidosis

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Eosinophilic parenchymal causes includes

Loffler syndrome
Drug allergy

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Smoking related parenchymal causes includes

DIP
Resp. Bronchiolitis

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Idiopathic pulmonary fibrosis also termed as

Cryptogenic fibrosing alveolitis

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Pathogenesis of Idiopathic pulmonary Fibrosis (IPF)

Environmental factors (GERD/Smoking etc) - alveolar epithelial cell injury (Alveolitis) - TGF- beta secretion - Fibrous tissue deposition

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Lobes involved in IPF

Bilateral Lower lobes
Subpleural location
Along interlobular septum

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Commonly affected age group in IPF

Elderly patients

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Clinical features of IPF

M > F
Dyspnea on exertion
Dry cough
Dry inspiratory crackles

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Diagnosis of IPF

Clinical Presentation
Surgical biopsy
Radiology - HRCT Scan

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Findings of surgical biopsy in case of IPF

Usual interstitial pneumonia
Patchy interstitial fibrosis
Architecture distortion
Honeycombing pattern microscopically

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Treatment of IPF

Lung transplant
Pirfenidone - inhibits TGF-beta activity
Nintedanib

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In case of massive fibrosis in IPF

Leads to death in 3 years

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Cause of Non specific Interstitial pneumonia (NSIP)

Idiopathic

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NSIP is associated with connective disorders except

Rheumatoid arthritis

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Clinical features of NSIP

Dry cough
Progressively increasing dyspnea
Age - 50-60 years

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NSIP commonly seen in

Non smoker females

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Heterogenous fibroplastic foci is seen in

IPF

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Diagnosis of NSIP

HRCT Scan Surgical biopsy

Most common lobe involved in NSIP

Lower lobe

Surgical biopsy findings in NSIP

Uniform/homogeneous involvement - same pattern

Cellular variant of NSIP includes

Chronic Inflammatory cells +ve Fibrous tissue -ve

Fibrosing NSIP includes

Chronic Inflammatory cells + fibrosis +ve

Clinical features of Cryptogenic Organising pneumonia (COP)

Cough Dyspnea

Diagnosis methods used in COP

Surgical biopsy

Surgical biopsy findings in case of COP

Masson body - loose C.T

Treatment of COP

Steroids

Pigmented or Smokers macrophages are seen in

Desquamative Interstitial pneumonia

Treatment of Desquamative Interstitial pneumonia

Quit smoking Steroids

In Respiratory bronchiolitis

Pigmented intraluminal macrophages +ve Resp. Bronchioles involvement

Pneumoconiosis means

Diseases due to occupational hazards

Pneumoconiosis pathogenesis

Ingestion of dust particles with breathing air - activates alveolar Macrophages - can leads to Fibrosis

Size of dust particles leading to Pneumoconiosis

1-5 micron in size

Most common pneumoconiotic disease

Silicosis

Inhalation of silica occurs in which occupations

Sand mining Stone crushing

Which lobe is commonly involved in Silicosis

Upper lobe

Findings on polarised microscopy in case of Silicosis

Shiny birefringent crystals

Chest X ray finding in case of Silicosis

Egg shell calcification

Which industry is commonly involved in Asbestosis

Insulation industry

Asbestos is

Tumor initiator or tumor promoter Dangerous chemical

Multiple type of lesions seen in Asbestosis

Pleural plaque (MC) Interstitial fibrosis

Body seen in Asbestosis

Ferruginous or Asbestos body - dumble like structure

Calcium levels in case of Asbestosis

Increased

Exposure of asbestos particle for 15-25 years can leads to

Bronchogenic carcinoma

Exposure of asbestos particles for 25-40 years can leads to

Malignant mesothelioma

Stages of Coal dust Pneumoconiosis

Stage 1 - Anthracosis Stage 2 - Simple coal worker Pneumoconiosis Stage 3 - progressive massive fibrosis

Anthracosis is

Asymptomatic coal worker disease Microscopically deposition of coal pigment in alveolar Macrophages

Simple coal worker Pneumoconiosis

Small nodules in lungs Centriacinar emphysema

Progressive massive Fibrosis in coal dust Pneumoconiosis is reversible or irreversible

Irreversible

Coal worker Pneumoconiosis+ Rheumatoid arthritis

CAPLAN Syndrome

Inhalation of cotton fibers is termed as

Byssinosis

Inhalation of sugarcane dust is termed as

Baggassosis

Berryliosis is more common in

Aerospace industries

Sarcoidosis is a

Multi system disorder Immune dysregulation

Genes associated with Sarcoidosis

HLA A1/B8

Sarcoidosis is most commonly seen in which gender

F >M Non smokers most frequently

Pathophysiology of Sarcoidosis

Unknown antigen - activates CD4 T cells - Cytokines secretion - TNF-alpha - Bronchoalveolar lavage fluid

Granuloma seen in Sarcoidosis

Non caseating granuloma

Clinical features of Sarcoidosis

MC involves lungs and lymph nodes - Bilateral hilar lymphadenopathy (Potato nodes) Dyspnea Cough Low grade fever Weight loss Also involves skin/eye/lacrimal gland/spleen/liver/BM

Skin related findings in Sarcoidosis

Lupus pernio - pinkish or purplish rash on cheek or tip of nose Erythema nodusum - painful nodes on lower limbs

Lofgren Syndrom

Includs triad of Erythema nodusum Arthralgia Bilateral hilar lymphadenopathy

Eye related clinical finding in Sarcoidosis

Uveitis

Lacrimal and salivary glands in Sarcoidosis

Mickulics syndrome - dry eyes, dry mouth

Granuloma formation is seen in which organs in case of Sarcoidosis

Spleen Liver BM

Endocrine gland most commonly affected in Sarcoidosis

Pituitary gland

Diagnosis of Sarcoidosis

Diagnosis of exclusion S. ACE elevated Increased activity of 1alpha hydroxylase - increased production of active form of vit D - Hypercalcemia Bronchioalveolar lavage fluid(BAL) - CD4 : CD 8 ratio - 5-15:1

Findings of lymph node biopsy in Sarcoidosis

Non caseating granuloma Intracellular inclusion bodies Residual body Asteroid body - Star shaped body Schaumann body - Bluish color material deposited in giant cells

Kveim test procedure

Extract tissue of spleen from known case of Sarcoidosis - intradermally injected into suspected patient - after 4-6 weeks - finds non - caseating granuloma

Treatment of Sarcoidosis

Spontaneous remission Steroids