-13- Interstitial Lung diseases (ILD)

Thursday, September 18, 2008 9:11 AM

Notes By:

Interstitial Lung diseases

Includes over 200 diseases They all share similar clinical, roentgenographic, physiologic or pathologic manifestation There is initially diffuse lung injury which progresses to Diffuse fibrosis of lung parenchyma Fibrosis of alveolar wall Fibrosis of interstitium Thickening and destruction of pulmonary vessels Resultant impairment of gas transfer and ventilation-perfusion mismatching. Etiological Classification: Unknown causes: Idiopathic Pulmonary fibrosis Desquamative interstitial pneumonia Acute interstitial pneumonia (Hamman Rich syndrome) Non-specific interstitial pneumonia Cryptogenic organizing pneumonia Pulmonary lymphangiomyomatosis Pulmonary hemorrhage syndromes e.g. Goodpastures syndrome, pulmonary hemosiderosis Pulmonary Langerhans cell Histiocytosis Sarcoidosis Wegner Granulamotsis BOOP Chemical or dust inhalation: Organic dusts e.g. moulds, avian proteins Mineral dusts e.g. silica, coal dust, asbestos Chemicals e.g. chlorine, nitrogen dioxide Iatrogenic and toxicities: Radiation pneumonitis Oxygen toxicity Toxic fumes and gases Drugs: Bleomycin Cyclophosphamide Methotrexate Hydralazine Busulphan Amiodarone Nitrofurantoin Pulmonary involvement in other disease: Rheumatoid arthritis Ankylosing spondylitis Sjogren's syndrome Systemic sclerosis Dermatomyositis Congenital causes: Neurofibromatosis Gaucher's disease Niemann-Pick disease Tuberous sclerosis Pathogenesis: Two major types of histopathologies 1. Those associated with predominant granulomatous reaction in interstitial or vascular areas 2. Those associated with predominant inflammation and Fibrosis Interstitial Lung diseases Clinical Features: Exertional dyspnea Persistent dry cough Hemoptysis Chest pain Clubbing Tachypnea Crackles Signs of cor-pulmonale Asymptomatic with identification of opacities on chest X-ray Investigations: Lung function tests: Restrictive pattern decreased lung capacity Total lung capacity (TLC), functional residual capacity and residual volume are decreased FEV1/FVC normal or increased Normal flow rates Impaired diffusion of CO (DLCO) due to reduced surface area for gas exchange Arterial blood gases: Hypoxemia Normal or reduced PaCO2 CXR: Bibasilar Reticular pattern or nodular opacities Honeycomb like features Hilar/mediastinal lymphadenopathy You can have the granulomatous reaction or fibrosis. How do these people present? The single most common thing they present with is exertional dyspnea. You will get a 35 year old or 45 year old. They cant walk to the mailbox without stopping anymore. Very common scenario for IPF. They may or may not have a cough with it. It will be dry? They may have hemoptysis, will complain of chest pain.

We are going to look at PFTs, we will have decreased lung capacity. Decreased functional residual capacity. Residual volume will be decreased. The ratios will usually be about the same. The flow rates will stay pretty good. The diffusion will be off though. Its restrictive and the diffusion capacity eventually will go way way down. Thats the ventilation perfusion mismatch.

Check blood gases: Will be hypoxic depending on where they are in the disease process. If its sarcoid then they will be normal. If its IPF, then they will be eventu hypoxic.

Chest xray: Whenever you hear ground glass, think about interstitial disease. Alveolar disease is the fluffy cloudy stuff we talked about

Fine reticulonodular, kind looks like looking through ground glass at it (patchy opacities). Very full hilums (hilar shadows).

Investigations High resolution CT scan (HRCT) gives a better assessment of ILD than CXR Gallium-67 or TC99 scan may be able to identify the inflammatory zones Fibroptic bronchoscopy and Bronchoalveolar lavage Lung Biopsy helps in confirming the diagnosis Treatment: Avoidance of further exposure to offending agents If hypoxemia is severe supplemental oxygen Anti-inflammatory and immuno-modulators Steroids, Cyclophosphamide, azothioprine Management of cor pulmonale if present

We are going to get a CT scan. Probably dont do gallium scans as much as we used to, because of the highres CT's, we will do a bronchoscopy and BALs, and transbronchial biopsies. If you dont get a diagnosis with CT scan bronchospy, then you may have to get a lung biopsy?

Treatment is to avoid it, give them oxygen, and

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Specific Types of ILD

Idiopathic Pulmonary Fibrosis (IPF)

Also known as cryptogenic fibrosing alveolitis is characterized by: Unknown etiology. Diffuse interstitial fibrosis. Severe hypoxia and cyanosis (in advanced cases). Sex & age: Male more than female after 60 years of age. Idiopathic Pulmonary Fibrosis

Treatment is to avoid it, give them oxygen, and then antiinflammatories, and manage rightsided failures.

This si just in here for noting the fibroblasts moving in after fibrosis. Depending on when you find these people, you will have different stages. The stages of IPF are thought about being different names. In the beginning its Usual interstitial pneumonia, its not infectious, its idiopathic.

Early stages interstitial pneumonia. Usual interstitial pneumonia (UIP) is associated with IPF. The hallmark of UIP is a heterogenous appearance of the lung parenchyma at low magnification. Interstitial inflammation (patchy - lymphocytes, plasma cells, & type II pneumocyte hyperplasia). Alternating areas of normal lung Later stages Interstitial fibrosis (dense collagen). Honeycomb lung cystic spaces with bronchiolar epithelium and filled with mucin Clinical Features: Presentation is insidious with Fatigue, Anorexia, weight loss Gradual onset of non-productive cough and progressive dyspnea. Dry crackle during inspiration. Advanced stages cyanosis, clubbing, cor pulmonale

You have inflammation in this IPF, then You get fibrosis later on. And honeycomb lung.

These people present with dyspnea on exertion. They may have weightloss because they get tired eating!!

Investigations: CXR: Bilateral, diffuse reticular or reticulonodular pattern More common in lower lobes, dont get pleural involvement. Lung biopsy is the gold standard. You are usually safe in saying lung biopsy is the gold standard in these diseases. More common in lower lobes No pleural involvement Biopsy: Diagnosis is mainly by surgical lung biopsy (gold standard) Presence of Usual interstitial pneumonia is characteristic Honey comb lung and fibrosis in late stages

Idiopathic Pulmonary Fibrosis: This is what it looks like on chest xray. This is big fibrotic holes

Idiopathic Pulmonary Fibrosis: This is what it looks like on CT Scan. Imagine how hard it was to get oxygen through that long.

Treatment: Not very effective Steroids Immunosuppression - azothioprine, cyclophosphamide Anti-fibrotic agents colchicine, pirfenidone, Interferon gamma 1b Lung transplantaion in end stage disease Prognosis remain grim with survival averaging 2-4 years

Steroids is the corner stone, cyclophosphamide, interferon, all these things have been tried. Usually they start then add/add/add. They may, depending on whether or not they have other diseases, may be a candidate for transplant. Generally this is the treatment. Usually these people were dead in 2-3 years, some in 4. These people developed pulmonary hypertension, rightsided failure.

Desquamative interstitial Pneumonia

Exclusively seen in smokers Peak incidence 4th - 5th decade Extensive accumulation of macrophages in intraalveolar spaces with minimal interstitial fibrosis Lung functions show restrictive pattern with reduced DLCO and arterial hypoxemia X-ray and HRCT show diffuse hazy opacities Good response to cessation of smoking and corticosteroids 10 year survival rate ~ 70%

This is called DIP. You get a diffusion defect on your lung xrays, you have hazy opacities on your scans. The difference with this is look at the response rate, 10yr survival of 70%. So you can put them on steroids until they do well.

Acute Interstitial Pneumonia (Hamman-Rich syndrome)

Fulminant lung injury with diffuse alveolar damage Probably constitutes a subset of cases of idiopathic ARDS Most patients older than 40 yrs Abrupt onset in previously healthy individuals Fever, cough, dyspnea frequently present Diagnosis and prognosis Diffuse bilateral opacification on CXR HRCT shows bilateral patchy ground-glass attenuation Lung biopsy shows diffuse alveolar damage Most patients develop respiratory failure More than 60% die in 6-months, but those who recover have substantial improvement in lung function

The Hamman Rich syndrome, usually is in older patients, comes on suddenly, looks JUST LIKE ARDS. For all intents and purposes, it is. They dont respond well to treatment!

They have diffuse opacities. They have leakage of cells and protein into alveoli. More than 60% DIE IN 6 MONTHS. But if you can keep them alive through the acute, then they may regain function!

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Cells filling up with proteinaceous material -> then fibrosis

This is early. On chest xray.

You can see them resolving over time. This is over months. But you can see it slowly resolving.

Treatment (Hamman Rich) Supportive Oxygen and ventilator support is often needed Role of corticosteroids is controversial

Most of the patients will be on steroids their entire life, except DIP. IPF will be on high dose until the day they die. This is endstage. Honeycomb lung

Pulmonary lymphangioleiomyomatosis (LAM)

It afflicts premenopausal women Should be suspected in young women with -, recurrent pneumothorax (50%) or chylous pleural effusion (30%) or chylous ascites(10%) Caucasians affected more commonly There is proliferation of atypical pulmonary interstitial smooth muscle cells in lymphatics and venules resulting in cyst formation LAM clinical features Disease accelerates in pregnancy and decreases after oophrectomy Patient presents with hemoptysis, dyspnea, cough, chest pain, pneumothorax and or chylothorax Progression is common and median survival is 8-10 yrs after diagnosis LAM diagnosis and treatment Pulmonary function may reveal - Obstructive or restrictive pattern HRCT shows thin walled cysts surrounded by normal lung and maybe diagnostic , FOB in some cases for conformation Treatment includes Oophrectomy Progestrone 10mg/day Tamoxifen 20 mg/day LHRH Lung transplant in end stages

They love this disease **ON BOARDS**, even though its rare. They get a recurrent pneumothorax, chylous pleural effusion or chylous ascities. The interstitial smooth muscle cells inside the lungs grow, and you get these cysts in the lymphatics/venules. So you get the uncontrolled smooth muscle growth.

It accelerates in pregnancy, driven by hormones, It decreases after oophrectomy Boards: 24 year ol woman comes in, 3 months pregnant, hemoptysis, shortness of breath, and pneumothoraces. This would be a sample board question presentation -0r- women in second pregnancy,and has pneumothorax for a sceond time Recurrent pneumothorax!! Has pretty good survival with treatment.

On PFTs - you get mixed results.

LHRH - leutenizing hormone replacements.

Professor has never seen a lung transplant given fort his, but guesses it happens.

On Chest xray/

CT scan- thickening and cysts are visible.

Syndromes of ILD with diffuse alveolar hemorrhage

This is a patient who did not do well. This is an arteriogram done after death after autopsy, look at all the cysts.

In vasculitis -injury to arterioles, venules, and alveolar septal capillaries can result in bleeding in alveolar space Recurrent episodes of alveolar hemorrhage leads to fibrosis ILD with diffuse alveolar Hemorrhage - New set of diseases You are allowing blood to get into the alveoli, they present with cough, may be hemoptysis. It depends Clinical features include on where they are at. They will have fever, if blood gets where its not supposed to be, they will have cough, fever, dyspnea dyspnea. Hemoptysis may be present CXR may show alveolar opacities Bronchio-alveolar lavage fluid shows presence of blood Falling Hematocrit and hemoglobin levels Goodpasture syndrome is the prototype of this group. Other examples include Wegners granulomatosis, isolated pulmonary capillaritis, connective tissue disorders Idiopathic pulmonary hemosiderosis Will have diffuse alveolar opacities. If you do a BAL, it will come back with hemoglobin. They may become anemic. Goodpastures is the prototype for this, but others include Wegeners Granulomatosis. So Study Goodpastuers, get it down. Then study wegeners, learn how they are same and different. Then make them your prototypical diseases and learn how the others are different. Do this for studies and for boards!!

Goodpasture Syndrome (GPS) Renal and pulmonary involvement Rapidly progressive glomerulonephritis and hemorrhagic interstitial pneumonitis Caused by antibodies to 3 chain of type IV Collagen in the glomerular and pulmonary basement membranes. Clinical Features Typically seen in males 5-40 yrs age Some cases occur in 6th and 7th decade Male: female ratio 6:1 Patient may present with hematuria and proteinuria and progressive renal failure Followed by dyspnea, hempotysis

You get a hemorrhagic pneumonitis and progressive glomerulonephritis. He doesnt care about pathophys, just here for completeness, cares about presentation,diagnosis, treatment.etc.

Mostly young males!! Board q: 14 year old comes in with hemoptysis and hematuria, thats what they might give in presentation.

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Followed by dyspnea, hempotysis

Chest xray, predominantly lower lobe. You see it fibrosing, then alveolitis. Presentation on stain Treatment: Plasmapheresis remove the offending antibody (plasma exchange). corticosteroids (e.g. methyl prednisolone) Immunosuppressive therapy with azothioprine or cyclophosphamide is used in severe cases Renal transplantation is sometimes needed (in severe cases).

Plasmapharesis is the treatment of choice.Cyclo Phosphamide has been used, but its only for reserve.

ILD with granulomatous response Sarcoidosis

A multi-system disorder of unknown etiology characterized by non-caseating granulomas. The histological diagnosis of sarcoidosis is one of exclusion Most common presentation. Bilateral hilar lymphadenopathy, or parenchymal lung involvement or both. Eye and skin involvement each can occur in about 25% of cases. Prevalence in US 10-40/100,000 More prevalence in African Americans Mostly presents between 20-40 yrs. Of age but can occur in children and elderly Multi-organ involvement: Lung (most common) Kidney Bone Heart CNS Lymph nodes Etiology and Pathogenesis. Immunologic factors. Intra-alveolar and interstitial accumulation of CD4+ T helper-I cells and macrophages. cytokines locally (IL-8, IL-1, TNF, MIP) recruitment of other T cells & monocytes noncaseating granulomas. Granulomas destroy surrounding tissue Chronic inflammation fibrosis Systemic immunologic abnormalities. Anergy to common skin test antigen (PPD, candida antigen). Thought to result from pulmonary recruitment of CD4+ T cells and consequent peripheral depletion. Polyclonal hypergammaglobulinemia. Manifestation of helper T cell dysregulation. Genetic Features: Familial and racial clustering. Association with certain HLA genotypes (HLA-A1 & HLA-B8). Clinical Features: due to lung involvement Asymptomatic (most common) Fever Cough Dyspnea Chest pain

In big city hospital with large black population, may see Sarcoid, in other places you may not. The key is non-caseating granulomas! How do they present? Usually they will have bilateral, hilar lymphadenopathy. And/Or lung disease. They may have one/the other/both. Eye and skin are the big ones involved, but other things can be involved.

They may be totally asymptomatic, it may be multiorgan involvemnt. Key to remember **SKIN AND EYE**

Remember Non-caseating granulomas, that granulomas destroy surrounding tissue. And that chronic inflammation leads to fibrosis.

They may be anergic. They can get a polyclonal hypergammaglobulinemia. Professor hasnt seen it but you will find it in writings.

May be present in familes!

It will usually be found on routine chest xray generally!! The symptoms might look like they have TB Fever, cough, weight loss, chest pain, making you think of some kind of chronic thing.

Clinical Features: Eyes: Anterior uveitis Heart: Tachyarrhythmias & cardiomyopathy Kidney: Glomerulonephritis, renal failure Bone Marrow: Leukopenia CNS: Cranial nerve palsy, seizures, headache, Skin changes: Papular lesions Lymphadenopathy: Mediastinal involvement, peripheral lymphnodes may get enlarged Hepatosplenomegaly Arthritis: First ankles, then knees, spares axial joints

Professor has seen CNS and Seizures. Arthritis is another thing that you will find. It is Ascending when they have it. Starts in ankles and goes up! But most common complaint professor has seen is knees!

Typical rash - erythema nodosum Sarcoidosis Clinical syndromes Lfgreen syndrome: Erythema nodosum, arthralgia and hilar lymphadenopathy Heerfordts syndrome: (uveioparotid fever) - Uveitis, parotitis and facial nerve palsy

Lupus pernio. <- These are board syndromes, probably not on test, but for boards.

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Sarcoidosis - diagnosis Diagnosis is based on clinical data and histology Investigations: Blood : Lymphopenia Elevated ESR Elevated ACE - Serum Angiotensin Converting Enzyme Hypergamaglobulinemia Hypercalcemia, Hypercalciuria (increased conversion of vitamin D to its active form in granulomas) is uncommon Lung biopsy is mandatory for the diagnosis non caseating granulomas and may contain inclusions like Schaumann bodies (conch like), asteroid bodies (stellate like) and residual bodies (refractile calcium containing) Other Investigations: CXR: Stage I: Bilateral hilar lymphadenopathy alone - Most common type Stage IIa: Adenopathy and diffuse Infiltrates Stage IIb: Infiltrates alone: Least common Stage III: Honey comb appearance Gallium-67 scan increased uptake

This is one of the few places where the sed rate will help (ESR?) Lymphopenia Elevated ESR Elevated ACE - Serum Angiotensin Converting Enzyme Hypergamaglobulinemia Profressor does not know why the ACE levels are elevated, it just says the noncaseating granulomas, the cells secrete a lot of it and they go up. May see hypercalcemia

Need tissue for diagnosis. All of this stuff is supportive, but to be 100% you need a lung biopsy. You will see this also in males and caucasians. What else is in differential for hilar lymphadenopathy? TB, Cancer! We have to know what it is! We have to make a diagnosis.

We need to know what it is, if we have bilateral hilar lymphadenopathy, and this is what we see on the chest xray, then its stage 1. Stage2a is when we start getting infiltrates. 2b is getting infiltrates and adenopathy goes away! This ones is least common. Stage 3 is honeycomb of lung. These people dont do very well.

Left: Stage 1 (lymphadenopathy), Right: Stage IIa. Hilum =Not normal!

Stage 3: Honeycombing, Patchy infiltrates.

This one is an inbetween (honeycombinb). May be some overlap, wont be entirely clear.

CT Scan of involvement!

Most of these do well and it will Sarcoidosis - Prognosis go away almost completely. As The disease has a unpredictable course. far as lung damage, may be as Progressive chronicity or high as 20%. But theres 10% that Remitting and relapsing. die from progressive pulmonary Prognosis: fibrosis and cor pulmonale. 65-70% of patients recover almost completely. 20% of patients develop permanent lung damage 10-15% of patients die from progressive pulmonary fibrosis and cor pulmonale.

Sarcoidosis Treatment: First-line: Systemic corticosteroids - prednisone Second line: Methotrexate Other drugs include chloroquine, indomethacin, oxyphenbutazone, thalodomide, levamisol, cyclophospamide, Professor used cyclophosphamide the most.

Wegener Granulomatosis
Wegener Granulomatosis is characterized by granulomatous vasculitis of upper and lower respiratory tract with glomerulonephritis There is necrotizing vasculitis - Pulmonary vessels may also show necrotizing granulomas. Necrotizing granulomatous inflammation in lung parenchyma Wegener Granulomatosis Clinical presentation: URT Epistaxis, nasal perforation, and chronic sinusitis.T LRT Cough, hemoptysis, and chest pain. Glomerulonephritis in 77% Eye involvement is seen in 52% Skin, cardiac and nervous system involvement may also occur in some cases Investigations: ESR is markedly elevated Leukocytosis Hypergammaglobulinemia 90% patients have antiproteinase 3 ANCA (antineutrophil cytoplasmic antibody) Biopsy necrotizing granulomatous vasculitis is diagnostic Treatment Glucocorticoids have improved the prognosis Cyclophosphamide and azothiprine can be used Wegeners they love to talk about on Boards, and Rounds. Necrotizing vasculitis and necrotizing granulomas

If its upper, it Will have nosebleeds, chronic sinusitis If its lower, it will be hemoptysis. Majority will have a nephritis with it What they might give you is young patient with nosebleeds, and hematuria .**

They should have a leukocytosis. But big thing is 90%+ will have positive cANCA. Need a tissue biopsy. But if they dont have renal involvement, then do a lung involvement. But with renal involvement, then go ahead and do renal biopsy, because they are not as involved, you can do those ultrassound guided. But open lung biopsy is MAJOR surgery! They respond very well to steroids generally. Cyclophosphamide, our friend, is the fallbakc agent.

Someone who did not erspond to therapy!! Lung looks like this because of blood! Patient Drowned in his own blood

Examples of what the necrotizing granulomas look like. Some of them have holes in the middle of them. Thats where they are becoming necrotic from the inside out

Langerhans Cell Histiocytosis

Smoking related diffuse lung disease Primarily affects men between 20-40 yrs Proliferation of Langerhans cell (Dendritic cells) that are CD1a positive Presence of HX bodies (Birbeck granules) in cytoplasm is characterstic Clinical spectrum is variable from asymptomatic to rapid progress Fever, cough, dyspnea, chest pain , weight loss are common Pneumothorax occurs in 25% Hemoptysis can occasionally occur

What to call it????? Langerhans Cell Ganulomatosis Langerhans Cell Histiocytosis Histiocytosis X Pulmonary Eosinophilic Granuloma

Langerhans Cell Histocytosis Disease has many names. All same thing!

These people are affected even younger than with DIP. Theres thing thats called berbeck granules. Thats where the X in histiocytosis X coems form. How will they present? With cough, generally dont have a lot of productive cough. This might look something like TB. You might have some fever, dyspnea, weight loss, often will have pneumos, and hemoptysis will occur.

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What it looks like with a stain. Diagnosis: CXR nodular or reticular opacities, upper zone cysts with sparing of costophrenic angles HRCT: combination of nodules with thin wall cysts are fairly diagnostic Lung functions show DLCO and evidence of restrictive disease Treatment: Discontinuance of smoking is key and results in improvement in 1/3 of patients Death due to respiratory failure occurs in ~ 10%

Everybody will get a chest xray, not diagnostic. May have nodular /reticular opacities. Will get a highres CT, showing thinwalled cysts. Will do the lung functions, and will see restriction and diffusion going down too!! Eventually will probably get a tissue diagnosis. You may be able to do it by bronchoscopy? If they stop smoking, most will get better on their own. But a small percentage (10%) wil have progression. Key is to stop smoking!!

Little nodules with small fibery looking reticular part

Fine lines is the reticular part, the little bitty nodules, as opposed to alveolar filling processes which are white, large fluffy (like CHF, ARDs). This is classic reticular nodular. Have to look at a few thousand xrays and they will get easier.Like EKGs.

Reticular nodules with little bit of honeycombing there too.

Bronchiolitis
Not all things call pneumonia are infectious 2 Types of Bronchiolitis Proliferative Bronchiolitis- ex: BOOP, SLE, PCP, CMV, CEP, Wegeners, drugs (gold, amiodarone) Constrictive Bronchiolitis- ex: RA, bone marrow rejection, viruses, inhaled toxins (phosgene), silica, coal, asbestosis

There are two broad categories of bronchiolitis. Theres a proliferative bronchiolitis: BOOP, SLE, PCP, Gold. Different infectious diseases.. Another kind, constrictive Bronchiolitis: RA, Bone marrow rejections.. Proliferative and Constrictive types!

BOOP (Bronchchiolitis Obliterans Organizing Pneummonia)

Cryptogenic Organizing Pneumonia Nonspecific reparative reaction to bronchiolar injury Organizing intraluminal exudate mostly in alveolar ducts Inflammatory changes in alveolar walls and foamy macrophages CXR-bilat patchy airspace dz with interstitial opacities PFTs- restrictive defect ( or mixed ) Steroid responsive and usually reversible

BOOP:Also called bronchiolitis Obliterans Organizing Pneumonia and cryptogenic. It is a repair mechanism that goes haywire ON CT or xray you see bilateral patchy airspace disease with interstitial opacitis. The PFTs are generally restrictive! Key here is proliferative bronchiolitis, restrictive defect. You would tihnk it would be the other way around. But if it is obliterative, it has a restrictive pattern.

Reticulonodular with alveolar filling Chest xray. See how they have alveolar filling, and a reticulonodular pattern. Look how fluffy white it looks on the upleft pic, but in other areas of pic it looks like reticulonodular. And bottom left is what it looks like on CT

Uncommon, you have fibrosis, diffuse pancronchiolitis, but the constrictive has an obstructive pattern. Constrictive Bronchiolitis Constrictive bronchiolitis has an obstructive pattern - Counterintuitive!!! Uncommon finding One of the most confusing disease processes! So dont get confused dragos and deepa. Inflammation to fibrosis Diffuse Panbronchiolitis CXR- progressive increase in lung vols PFTs- Obstruction with hyperinflation Obstructive pattern and unresponsive. Relatively corticosteroid unresponsive and progressive with irreversible obstruction and air trapping

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Pretty nonspecific imaging. You need to do biopsies. A lot of times these patients would come into professor, if they didnt have an infection, often it was constrictive bronchiolitis Last patient with BOOP professor saw was young 32 year old trust lawyer. Developed lymphoma, got treated, did well, went into remission, reflare up, came back to hospital. Then started having SOB. Was on bleomycin. Came back as BOOP from CT scan and biopsy. Put him on Steroids and he looked better for a few days then started getting worse again. The patient said he felt like he was going to die and died from a 4 inch saddle embolus. But these were not related. 3 separate events going on.

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