Interstitial Lung Disease Flashcards

Question

What does honeycomb pattern on lungs indicate?

Answer 1

idiopathic pulmonary fibrosis

Answer 2

extrnsinc lung disease

Answer 3

Reduced DLCO indicates intrinsic lung disease so assess for drug related or occupation related exposures. No exposures means that antibiotic testing should be assessed with ANA, RF, ANCA or anti-GBM

Answer 4

hypersensitivity pneumonitis

Answer 5

sarcoidosis

Answer 6

A chronic immune-related inflammatory condition caused by a non-caseating granuloma. It typically affects the lungs and causes mediastinal lymphadenopathy, pulmonary fibrosis and pulmonary nodules but has multisystem effects. It tyupocally affects black women in their 20s40s. ## Footnote Typically affects the lungs and has multisystem effects

Answer 7

Aetiology is linked to occupational/environmental exposures to beryllium and dust or mutation of chromosome 6 MHC gene. It is more common in black populations and women.

Answer 8

interaction with unknown antigen induces CD4+ T helper cells tor ecruit macrophages to form the non-caesatng granuloma which contain epitheioid histiocytes and giant cells. The macrophages synthesise 1-a-hydroxylase which activates vitamin D, increasing calcium absorption and causing hypercalcaemia, increasing risk of kidney stones. *It causes hilar lymphadenoapthy and these hilar lymph nodes drink into the mediastinal lymph nodes and cause mediastinal lymphadenoapthy. * There may also be hypergammaglobulinaemia and raised ACE due to elevated activity of macrophages. High T cell activity causes B cell stimulation, leading to raised serum immunoglobulins and immune complexes. There is a reduction in delayed-type hypersensitivity reactions

Answer 9

cirrhosis and choelstasis and most common cause of non infectious hepatitis

Answer 10

erythema nodosum (tender red nodules on the shins due to subcutaneous fat inflammation which has a good prognosis and is also associated with ) and lupus pernio (raised purple lesions of skin nose and cheeks that has a bad prognosis)

Answer 11

causing bundle branch block and heart block and cardiomyopathies if depositied into myocardium and may cause restrictive cardiomyoapthy

Answer 12

* Stage 0: Normal * Stage 1: Hilar lymphadenopathy * Stage 2: Hilar lymphadenopathy and parenchyma infiltrate * Stage 3: Parenchyma infiltrate * Stage 4: Fibrosis ## Footnote Staging helps in assessing disease progression

Answer 13

* Dry cough * Dyspnoea * Blurry vision * Palpable lymph nodes * Painful swollen joints * Proximal myopathy In more severe cases, heart block and sudden death may occur, therefore it is reccomended that an implantable ICD ## Footnote Other symptoms may include fever and psychiatric overtone

Answer 14

A mild acute form of sarcoidosis characterised by fever, erythema nodosum, bilateral hilar lymphadenopathy, and polyarthritis. On biopsy, there are naked granuloma with little surrounding lymphocytic inflammation. Treatment is typically supportive with NSAIDs or corticosteroids because the condition generally self-resolves. ## Footnote Typically presents acutely in younger patients

Answer 15

Erythema nodosum is painful bright red subcutaneous bilateral nodules on the anterior shins, along with a fever.

Answer 16

tachypnoea, Inspiratory crackles, friction rub due to pleurisy or pericarditis. Triphasic with pericarditis and biphasic with pleurisy. Opthalmolgocal signs include corneal opacitieisand gluaucoma.

Answer 17

Cardiac symptoms may include palpitations, syncope and dizziness, shown through echocardiogram that shows restrictive cardiomyoapthy. Treatment is with systemic steroids, prednisolone or immunosuppressants.

Answer 18

Systemic symptoms include fever, sweats, loss of appetite and weight loss.

Answer 19

Macrophages synthesise 1-alpha-hydroxylase, increasing calcium absorption ## Footnote This can lead to kidney stones

Answer 20

* Interstitial pulmonary fibrosis * Cor pulmonale * Increased risk of aspergillosis in lung cavities ## Footnote Cor pulmonale is caused by pulmonary artery vasoconstriction

Answer 21

Serum ACE is raised and can be used to monitor disease progression Hypercalcaemia Bronchoalveolar lavage showing increased CD4:CD8+ ratio Soluble iL-2 receptors CXR showing bilateral hilar lymphadenopathy Bronchoscopy showing an increased CD4:CD8 ratio

Answer 22

Histology where biopsy of granuloma area showing non-caesating granuloma with epithelioid cells

Answer 23

Initial treatment with high dose 40mg Oral steroids such as prednisolonefor 6-24 months with bisphosphonates to prevent osteoporosis. Can later reduce dose to 5-20mg for maintenance. If this fails, IgG dose intravenous methylprednisolone is useful, especially for neurosarcoidosis. Immunosuppressants like methotrexate and azathriopine

Answer 24

-> methotrexate should be avoided with hepatic or renal failure and can cause GI upset, stomatitis, pneumonia and is teratogenic

Answer 25

myelosuppression and fever

Answer 26

cyclophosphamide should be used for neurosarcoidosis unresponsive to steroids Pulmonary transplantation for severe disease

Answer 27

-> 60% of patients have spontaneous resolving of symptoms ->Pulmonary fibrosis and dhypertension can mean transplantation is required ->Death is usually due to heart involvement causing arrythmias and CNS involvement ->Hypercalcaemia should be managed by limiting dietary calcium intake and provide steroids and increasing fluid intake ->Skin lesions can be treated with topical steroids, otherwise lupus pernio should be treated with systemic steroids. -> Treatment of eye inflammation is with local steroids ->Renal involvement is treated with steroids and hyodrxychloroquine

Answer 28

* Serpentine: curly and flexible, associated with interstitial pulmonary fibrosis and lung cancer * Amphibole: straight and rigid, associated with interstitial pulmonary fibrosis, lung cancer, and mesothelioma ## Footnote Different types of asbestos have different health impacts

Answer 29

Inhalation of asbestos fibres causes interaction with epithelial cells and macrophages, leading to interstitial fibrosis. Deposition of fibres typically occurs in the lower lobes, related to intensity and duration of asbestos exposure as a result of radial oxygen species. The severity and rate of progression is influenced by cigarette smoking. It begins centrally and moves peripherally. deposition of fibres typically occurs in the lower lobes, related to intensity and duration of asbestos exposure as a result of radial oxygen species. The severity and rate of progression is influenced by cigarette smoking. It begins centrally and moves peripherally. ## Footnote Affects the lower lobes

Answer 30

Asbestosis can cause lower lobe bronchi dysplasia and lead to bronchoalveolar carcinoma , or pleural dysplasia and mesothelioma.

Answer 31

* Reticulonodular opacities on CXR due to visceral pleura thickening and leads to a restrictive lung disease, with reduced chest expansion and clubbing. The severity is dependent on exposure levels. * Honeycomb pattern on high resolution CT ## Footnote Imaging is critical for diagnosis

Answer 32

Normal DLCO indicates extrinsic lung disease; reduced DLCO indicates intrinsic lung disease ## Footnote Helps assess for drug-related or occupational exposures

Answer 33

* High-dose oral steroids (prednisolone) * Immunosuppressants (methotrexate, azathioprine) ## Footnote Treatment may depend on the severity of symptoms

Answer 34

Inhalation of asbestos fibres ## Footnote Asbestosis is characterized by diffuse interstitial pulmonary fibrosis due to asbestos exposure.

Answer 35

Lower lobes. They can become coated with iron and haem to form a ferruginous bodies, that can better be visualised with Prussian blue stain they appear golden brown ## Footnote The severity and progression of asbestosis are related to the intensity and duration of exposure.

Answer 36

* TNF * Oxygen derived free radicals ## Footnote These mediators induce interstitial fibrosis.

Answer 37

Restrictive lung disease ## Footnote It is characterized by reduced chest expansion and clubbing.

Answer 38

It influences the severity and rate of progression ## Footnote Cigarette smoking exacerbates the effects of asbestos exposure.

Answer 39

* Small linear opacities * Ground glass opacities * Dumbbell shaped ferruginous bodies ## Footnote These findings are typically visible on a Chest X-ray.

Answer 40

More sensitive for identification of honeycomb lung ## Footnote Both HRCT and CXR are not very sensitive for detecting asbestosis.

Answer 41

There may be signs of cor pulmonale such as pedal oedema as a result of pulmonary vascular remodelling. It has a restrictive lung disease pattern with reduced Kyle volumeDLCO is reduced with maintained FEV1 and FVC. ABG may show hypoxia and respiratory alkalosis. Treatment is with corticosteroids and immunosuppressive drugs can be used as an alternative or oxygen therapy for patients with hypoxaemia.

Answer 42

* Benign pleural plaques * Primary bronchogenic carcinoma * Malignant mesothelioma ## Footnote These conditions are linked to occupational asbestos exposure.

Answer 43

20-40 years ## Footnote This latency period follows exposure to asbestos.

Answer 44

Benign pleural plaques -> MULTIPLE white/yellow calcified plaques on the parietal pleura or diaphragm dome and increase the risk of pleural effusion, but no effect on risk for mesothelioma. This can be better viewed radiologically, showing ivory white supradiaphragmatic and pleural plaques. It develops 20-30 years after exposure and high resolution CT scan are more sensitive than CXR for detecting pleural plaques ## Footnote These plaques are typically multiple white/yellow calcified plaques on the parietal pleura.

Answer 45

Diffuse pleural thickening where fibrosis of the visceral pleural may occur, with ill-defined margins and follows reccurrent asbestos pleurisy. CXR may show pleural opacity over at least a quarter of the chest wall and presents commonly with symptoms like Exertional breathlessness and chest pain.

Answer 46

Rounded atelctasis/Blesovsky syndrome develops as contracting visceral pleural fibrosis, creating a mass 2.5-5cm in diameter, typically diagnostic with CT. It is typically asymptomatic and stable and slowly progressive, with no specific treatment/

Answer 47

Malignant mesothelioma is a malignant tumour that invades the subpleural lung tissue from a latency period following asbestos exposure 25-40 years. This can lead to haemorrhagic pleural effusion and pleural thickening. It arises form the serosa cells of the pleura and is unrelated to dose-exposure. There will be psammoma bodies and calretinin positive. Non-asbestos related causes include Eriodonite, found in rocks in cappadocia.

Answer 48

Rheumatoid arthritis with rheumatoid nodules in longs nand coal worker pneumonicosis

Answer 49

Poor prognosis for mesotheiolima is transdiaphramatic muscle invasion, male gender, sarcamatoid histology no involvement of mediastinal lymph nodes.

Answer 50

* Epitheliod: better prognosis * Sarcomatoid: poor prognosis * Mixed ## Footnote Each subtype has different prognostic implications.

Answer 51

Nodules form and originate from the parietal pleura, which typically spreads to the visceral pleura and invades into adjacent structures such as the chest wall diaphragm and mediastinum. Lymph node involvement is typically the bronchopulmonary/hilar lymph nodes and nodal spread is direct and local but is typically uncommon.

Answer 52

* Dull diffuse chest pain * Shortness of breath * Haemoptysis * Unintentional weight loss * Cough * Fatigue ## Footnote Symptoms often indicate advanced disease.

Answer 53

Pneumothorax can occur due to damage of the bronchial tree in pleural space. Complications include SVC obstruction, cardiac tamponade and spinal cord compression and distant metastases is common. There is a poor prognosis with survival of 1 year and average age of diagnosis is 72 and the disease typically appears around 50 to 60s at a later stage and treatment dependents on disease stage

Answer 54

Risk factors for mesothelioma include asbestos exposure with a latency period of 20 to 440 years, ionising radiation or genetic predisposition with genetic mutation in the BRCA1 gene.Professions with high asbestos exposure include mining, shipbuilding, ceramics, insulation and road repair.

Answer 55

*Sputum sample with bronchoalveolar lavage showing ferruginous bodies *pleural fluid analysis showing straw. Coloured or bloody effusion *High resolution CT is best and shows opacities in lower lung fields CXR will show pleural effusion with encasement of the lung *Radiology showing asbestosis in the lungs *Pleura thickening or calcification in lower long fields and white plaques *Pleural fluid analysis showing exudative straw-coloured or serous or bloody exudate *Pulmonary function test showing restrictive pattern *Reduced DLCO

Answer 56

72 years ## Footnote The disease typically appears in individuals around their 50s to 60s.

Answer 57

Average survival is 9-12 months post diagnosis ## Footnote Prognosis is poor, especially with advanced disease.

Answer 58

Systemic therapy with chemotherapy with Pemetrexel and Cisplatin -> this is in combination with folic acid and b12 supplementation Immunotherapy with PD-1 inhibitor and CTLA-4 inhibitor Radiotherapy, adjuvant used post-operative LH or palliative for symptom management for chest wall diaphragm pain or dyspnoea Pleural intervention with pleurodesis Symptom management with pain control, nutrition and psychosocial care

Answer 59

Supportive care Talc pleurodesis to reduce the occurrence of pleural effusion Mesotheliom: surgery, radiation, chemotherap with cisplatin and paclitaxel -> paclitaxel works by inhibiting DNA synthesis proteins like thymidylate synthase -> cisplatin works by inducting DNA alkylation Median survival with mesothelioma is 9-12 months post diagnosis, with involvement of the contralteral lung, SVCO syndrome and cardiac tamponade.

Answer 60

Reduces the occurrence of pleural effusion ## Footnote It is a supportive care measure.

Answer 61

* Surgery * Radiation * Chemotherapy with cisplatin and pemetrexed ## Footnote Treatment options vary based on disease stage.

Answer 62

* Ground glass appearance * Nodular opacities * Egg shell calcifications in hilar lymph nodes ## Footnote These findings are indicative of silicosis, especially with chronic exposure.

Answer 63

Coal worker’s Pneumonicosis involves inhalation of coal dust from either coal mines or tobacco smoke that can result in te formation of anthracotic pigments which can become consumed by alveolar macrophages and become dust cells. causing inflammation of the intersistial tissue of the lung and the hilar lymph nodes. Patients with coal wor’ers pneumonicosis are typically asymptomatic.

Answer 64

fibrotic opacity is less than 1cm, typically affecting the upper lobes and upper portion of the lower lobes and progression is dependent on the amount of coal dust consumed. It typically causes obstructive lung disease and results in emphysema.

Answer 65

fibrotic opacity is over 1-2cm and occurs at the lung apex, with no correlation to exposure. It causes a restrictive lung disease pattern with risk of cor pulmonale, carcinoma and there is no treatment available. It forms part of Caplan syndrome, where there is pneumonicosis with pulmonary nodules and rheumatoid arthritis.

Answer 66

In Caplan syndrome, there is a necrobiosis rheumatoid nodule containing a central necrotic core of black coal dust and necrotic tissue, with an active zone of inflammation in the lungs or concentric rings of coal dust. There will be rapid development of multiple nodules which obstruct airflow but majority of patients are asymptomatic but may experinece dry cough, pleuritic chest pain, SOB and fatigue.

Answer 67

Serum markers ifor diagnosis include ANAA (antinuclear antibody) rheumatoid factor and ANCA (antineutrophil cyptoplasmic antibodies). Management is to reduce exposure, administer corticosteroids to slow progression and provide supportive care.

Answer 68

Silicosis is caused by inhalation of silica quarts from dust, which are small enough to bypass mucociliary apparatus activating alveolar macrophages, causing cytokine release for surfactant cells to increase production, resulting in build-up of protein Aeolus debris in alveoli called silicoproteinosis and leading to irreversible lung fibrosis in the upper lungs. Within the macrophages, the silica dust is resistant to degradation and causes the production of reactive oxygen species and cellular destruction and contents are released. There is a concentrically arranged zone with a peripheral poorly organised zone.

Answer 69

Simple: small lung nodules due to chronic exposure within 10 to 30 years and have an asymptomatic presentation or dry cough/dyspnoea. Complicated: large lung nodules which can coalesce to cause a progressive large fibrosis Silicoproteinosis: rare, caused by overwhelming exposure within 5 years of exposure to silica dust causes eosinophilic materials to fill the alveoli, causing alveolitis and may be mistakenly identified as pulmonary oedema.

Answer 70

Patients with silicosis present with cough, dyspnoea and are at risk of progressive massive fibrosis, cor pulmonale due to pulmonary artery vasculature remodelling, Caplan syndrome, TB and lung cancer.

Answer 71

Patients with silicosis present with cough, dyspnoea and are at risk of progressive massive fibrosis, cor pulmonale due to pulmonary artery vasculature remodelling, Caplan syndrome, TB and lung cancer. CXR will show multiple bilateral nodules in the upper and middle lobes and enlargement of hilar and mediastinal lymph nodes.

Answer 72

treatment is prevention of fibrosis through exposure avoidance and whole lung lavage to slow progression or anti-fibrotic therapy. Steroids may alleviate inflammation with acute silicosis and lung transplantation is a last resort. Supportive treatment may be offered with supplemental oxygen.

Answer 73

Berylliosis is a granulomatous lung disease associated with inhalation of beryllium with risk from working in aerospace industry, dentistry, metal alloys.

Answer 74

Type 4 hypersensitivity reaction driven by sensitised T cells that results in non-Necrotising granuloma formation of macrophages and CD4+ T cells in the upper lobes that causes chronic interstitial pneumonitis -> fibrosis and presents clinically with cough and SOB, fever, night sweats and fatigue. There is a latency period from 3 months to 30 years. ## Footnote This reaction results in non-necrotizing granuloma formation.

Answer 75

bronchoalveolar lavage, beryllium lymphocyte proliferation test and granulomatous inflammation on lung biopsy. *CXR and high resolution CT will show nodules along septal lines, ground glass opacities and parenchyma nodules *Beryllium lymphocyte proliferation test: acquiring peripheral blood or fluid from bronchial alveolar lavage and culturing lymphocytes with beryllium sulphate, where elevated count is abnormal. *Bronchoscopy and bronchoalveolar lavage is performed to obtain cell count

Answer 76

Acute pneumonitis Pulmoanry fibrosis Chronic granulomatous disease

Answer 77

Management is with steroids, methotrexate immunosuppressant as an lternative with low dose with folic acid. Prognosis is good in the absence of chronic exposure, but has a highly variable clinical course and can impair lung fucntion due to nodule formation

Answer 78

* Breathlessness * Dry cough * Systemic symptoms (fever, chills, arthralgia) ## Footnote Symptoms may resolve within 1-3 days after exposure.

Answer 79

Exposure to mouldy hay ## Footnote This condition is a type of hypersensitivity pneumonitis.

Answer 80

Rheumatoid arthritis with rheumatoid nodules in lungs and coal worker pneumoconiosis ## Footnote It features necrobiosis and rapid development of multiple nodules.

Answer 81

Silicosis ## Footnote It results from inhalation of silica dust.

Answer 82

Hypersensitivity pneumonitiis is an interstitial lung disease characterised by immunological reaction of the lung parenchyma in response to repetitive inhalation of a sensitised allergen that causes immune complex-mediated 4 hypersensitivity and mediated by IgA and IgG after initial sensitisation.

Answer 83

Acute hypersensitivity pneumonitis following a short period of exposure (4-6hrs) to antigen which is reversible and affects the airways and bronchi, due to the inhalational nature with poorly-formed non-caesating granuloma and inflammatory cell infiltrates. Clinical features of hypersensitivity pneumonitiis includes breathlessness, dry cough and systemic psymptoms with fever, chills, arthralgia, myalgia and headache. There is crackles and squeaks on auscultation, and symptoms may resolve in 1-3 days. There are diffuse small nodules with ground glass change and increased lucency due to air trapping from bronchiolar involvement.

Answer 84

Chronic hypersensitivity pneumonitis following a chronic exposure to antigen and is less reversible. There is fibrosis and and absence of granulomata involvement. Clinical features include progressive exertional breathlessness, finger clubbing, dyspnoea, productive cough, systemic symptoms like weight loss, with crackles and squeaks on auscultation. There is typically upper and mid-zone reticulation with centrilobular nodules.and there is a restrictive pattern with PFTs

Answer 85

Exposure to mouldy hay ## Footnote Farmer’s lung is a type of hypersensitivity pneumonitis resulting from inhalation of organic antigens found in mouldy hay.

Answer 86

Delayed IV hypersensitivity ## Footnote This reaction activates CD8+ cytotoxic cells in response to chronic antigen exposure.

Answer 87

Progressive exertional breathlessness, finger clubbing, dyspnoea, productive cough, systemic symptoms like weight loss, crackles and squeaks on auscultation ## Footnote These symptoms indicate lung involvement and systemic effects due to chronic inflammation.

Answer 88

Upper and mid-zone reticulation with centrilobular nodules ## Footnote This imaging pattern indicates interstitial lung disease associated with chronic exposure.

Answer 89

It is associated with neutrophilia and diagnosis with onset of symptoms 4-8 hours after exposure, presence of weight loss and finding of crackles on examination. Management is based on antigen avoidance, but if necessary, corticosteroids are required

Answer 90

Restrictive pattern ## Footnote This pattern reflects decreased lung volume and capacity due to fibrosis.

Answer 91

Elevated ESR, CRP, and IgG positive for a specific antigen ## Footnote These findings indicate inflammation and an immune response to the inhaled antigen.

Answer 92

4-8 hours ## Footnote This delayed response is characteristic of hypersensitivity pneumonitis.

Answer 93

Antigen avoidance, corticosteroids if necessary ## Footnote Avoiding exposure to the causative antigen is crucial, while corticosteroids can help reduce inflammation.

Answer 94

Malt worker’s lung ## Footnote This condition is similar to Farmer’s lung but is specific to workers handling barley.

Answer 95

Pet birds, bird droppings ## Footnote This condition results from inhaling organic dust and proteins from birds.

Answer 96

Chemical pneumonitis ## Footnote This condition arises from exposure to harmful chemicals, leading to lung inflammation.

Answer 97

Grain exposure ## Footnote Similar to Farmer’s lung, this condition is related to inhaling organic dust from grain.