DPLD Flashcards

Question

What are the cause of OP?

Answer 1

* Cryptogenic * Organising pneumonia secondary to (ABCIII):- - Airway obstruction - (Post) Bone marrow transplant - CTD (myositis, RA, Sjorgen’s), Cancer (lung and haematological malignancy, radiotherapy) - Drugs, DAD (diffuse alveolar damage) - Infection (lung abscess, pneumonia, bronchiectasis), IBD, pulmonary infarction)

Answer 2

* Short history (<3 months) * Dyspnoea (often mild) + dry cough - Some develop severe dyspnoea with rapid onset respiratory failure -> “fulminant COP” * Often systemic features – malaise, fever, weight loss, myalgia

Answer 3

* Bloods – neutrophilia, raised CRP, raised ESR * Lung function – mild-mod restrictive pattern * CXR – patchy consolidation, sometimes nodular shadowing * HRCT - Consolidation with air bronchograms - Sometimes associated with GGOs or small nodules - Location – often basal, subpleural and peribronchial - Reticulation – suggests poor response to treatment - Solitary mass (rare) – can lead to cavity * TBB – can be diagnostic for patients with typical features * BAL – lymphocytosis, neutrophila and eosinophilia

Answer 4

* Alveolar spaces ‘plugged’ with granulation tissue (fibrin, collagen-containing fibroblasts, inflammatory cells) – can extend up to bronchiolar lumen * Patchy * Lack of architectural distortion * Evidence of underlying cause – infection, vasculitis

Answer 5

* Made on the basis of clinical, HRCT and biopsy findings * Histological findings often non-specific – should search for cause of OP

Answer 6

* Normal management: prednisolone - 0.5-1.5mg/kg for 1-3 months - Taper over 6-12 months * Fulminant disease: methylprednisolone then prednisolone - pulsed IV methylpred, 0.75-1mg x3 - then prednisolone maintenance 0.5-1mg/kg * If poor response to steroids - Azathioprine or - Cyclophosphamide (can be pulsed pulsed in critically ill patients after 5-7 days with no steroid response

Answer 7

* Often preceded by ‘viral’-type illness * Systemic symptoms: fevers, fatigue, myalgia, arthralgia * Rapid onset (days) of dyspnoea

Answer 8

* CXR: - Diffuse airspace shadowing with air bronchograms - Bilateral and diffuse - Often spares CP-angles/heart borders/hila * HRCT - Early: bilateral/diffuse/patchy GGOs and airspace shadowing - Late: reticulations, traction bronchiectasis, cystic change * PFTs - Restrictive spirometry - Reduced DLCO - Also profound hypoxia and respiratory failure * BAL - Increased total cells, red blood cells and haemosiderin - Non-diagnostic, helps to exclude infection * Lung biopsy - Required for diagnosis

Answer 9

* Diffuse alveolar damage * Hyaline membranes * Oedema * Interstitial inflammation * Alveolar septal thickening * Advanced disease: organising fibrosis and honeycombing

Answer 10

* Supportive * High dose steroids (pulsed methylpred then pred) may be of help – although more likely fulminant COP rather than AIP.

Answer 11

* RB - Accumulation of bronchiolar pigmented macrophages in cigarette smokers - Nearly always asymptomatic * RB-ILD - An ILD that develops in a minority of cases * DIP - Felt to be a more extensive accumulation of pigmented macrophages causing more advanced ILD - Macrophages located more diffusely throughout alveolar airspaces

Answer 12

* Mild dyspnoea and cough - Small proportion have severe dyspnoea and respiratory failure * Crackles

Answer 13

* PFTS - Restrictive or combined restrictive and obstructive disease - Mildly impaired DLCO * CXR - Thick walled bronchi - Reticular change - GGOs - May be normal * HRCT - Centrilobular nodules - GGOs - Thick-walled airways - Often centrilobular emphysema present * BAL - Pigmented alveolar macrophages

Answer 14

* Pigmented brown macrophages in terminal bronchioles * Patchy bronchiolocentric distribution * Findings frequently found in healthy smokers

Answer 15

* Smoking cessation * Steroids occasionally used

Answer 16

* Cough and dyspnoea – onset over weeks-months * Clubbing present (50%)

Answer 17

* PFTs - Mild restrictive pattern - Sometimes reduced DLCO * CXR - Normal often - Reticulations - GGOs - Particularly affects lower zones * HRCT - GGOs - Reticulations and honeycombing can be present (often mild) - Lower or peripheral zones dominant * BAL – pigmented macrophages

Answer 18

* Lung biopsy often required * Histology – diffuse accumulation of pigmented macrophages in alveolar airspaces, changes are uniform

Answer 19

* Smoking cessation * Steroids often used – responds well

Answer 20

Lymphoid Interstitial Pneumonia (LIP) * Diffuse lymphoid infiltrates and lymphoid hyperplasia causing interstitial pneumonia * Minority of cases idiopathic, most have an association/cause

Answer 21

* Autoimmune Disease - Haemolytic anaemia - Hashimoto’s thyroiditis - Pernicious anaemia - Chronic active hepatitis - PBC - MG * Connective Tissue Disease - Sjorgen’s syndrome - Rheumatoid arthritis - SLE * Drugs - Phenytoin * Infection - PCP - HBV * Immunodeficiencies - HIV - Common variable immunodeficiency * Idiopathic

Answer 22

* Gradual-onset dyspnoea and cough (years) * Systemic features – fever, weight loss * Crackles

Answer 23

* Bloods - Mild anaemia may occur - Poly- or monoclonal increase in serum immunoglobulins * CXR - Alveolar shadowing (lower zone) - Diffuse honeycombing * HRCT - GGOs - Reticulations - Cysts (differentiates from NSIP) - Honeycombing and nodules (occasionally) * BAL – non-clonal lymphocytosis

Answer 24

* Diffuse interstitial lymphoid infiltrates * Predominantly involving alveolar septa * Occasional lymphoid hyperplasia or honeycombing * Similar appearance to: cellular NSIP, follicular bronchiolitis and lymphoma

Answer 25

Disease Source Antigen Farmer’s Lung Mouldy hay Aspergillus species Thermophillic actinomycetes (thermoactinomyces vulgaris, micropolyspora faeni) Bagossosis Mouldy bagasse (pressed sugarcane) As above Compost lung Compost As above Mushroom workers Lung Mushrooms As above Humidifier lung Contaminated water in humidifiers and A/C units As above Malt Worker’s Lung Mouldy barley Aspergillus clavatus Summer-house HP (Japan) House dust Trichosporon cutaneum Hot Tub Lung Hot tub mist, ceiling mould M. Avium Complex Bird Fancier’s Lung Bloom and bed feathers and droppings Bird/avian proteins Rat Lung Rat droppings Rat proteins Isocyanate HP Paints Toluene dissocyanate

Answer 26

* Not fully understood * Likely Type IV hypersensitivity reaction (T-Cell mediated and granuloma formation) * And/or Type IV hypersensitivity reaction (antibody-antigen immune complex formation) * Not an atopic disease – not Type I hypersensitivity (rise in tissue eosinophils or IgE)

Answer 27

Acute HP * Symptoms (4-8hrs after antigen exposure) - Dry cough - Dyspnoea - Systemic symptoms: fevers, chills, arthralgia, myalgia, headache - Settle within 1-3 days spontaneously (if exposure stopped) * Examination - Crackles - Squeeks - Wheeze Chronic HP * Symptoms (months-years) - Exertional dyspnoea - Dry cough - Sometimes systemic symptoms (weight loss) - May have history of acute episodes * Examination - Crackles - Squeeks - Features of cor pulmonale may be present

Answer 28

CXR * Nodules (small (1-3mm) and diffuse) * Infiltrates * GGOs * Apical sparing * Often normal (20%) HRCT * GGOs (patchy or diffuse) * Poorly-defined micronodules * Mosaic attenuation (enhanced on expiratory HRCT) - Occur due to air trapping from bronchiolar involvement) PFTs * Restrictive pattern * Reduced gas transfer * Reduced lung volumes * Mild obstruction can occur * May be normal Bloods * Neutrophilia * Raised inflammatory markers * Serum antibody (IgG) Precipiten - Found in 90% of patients, but also in significant numbers of asymptomatic patients with antigen exposure - Levels often fall in absence of ongoing antigen exposure BAL * Lymphocytosis (often >50%) = characteristic finding * Not diagnostic, also may be found in asymptomatic exposed individuals Transbronchial or surgical lung biopsy * May be required if diagnosis unclear * Histo - bronchiolocentric granulomatous lymphocytic pneumonitis

Answer 29

CXR * Reticulations (upper and mid zone) * Reduced lung volumes HRCT * Diffuse centrilobular nodules * GGOs * Mosaic attenuation * Honeycombing * Traction bronchiectasis * Upper lobe predominance typical (differs from UIP) PFTs * Restrictive pattern * Reduced gas transfer * Reduced lung volumes * Mild obstruction can occur * May be normal Bloods * Raised inflammatory markers * Serum antibody (IgG) Precipiten - Found in 90% of patients, but also in significant numbers of asymptomatic patients with antigen exposure - Levels often fall in absence of ongoing antigen exposure BAL * Lymphocytosis (often >50%) = characteristic finding * Not diagnostic, also may be found in asymptomatic exposed individuals Transbronchial or surgical lung biopsy * May be required if diagnosis unclear * Histo - bronchiolocentric granulomatous lymphocytic pneumonitis

Answer 30

* Typical presentation is diagnostic - History of antigen exposure - Typical clinical features - Typical HRCT features * Atypical presentation requires further investigations - BAL lymphocytosis - Histology (bronchiolocentric granulomatous lymphocytic pneumonitis)

DPLD Flashcards

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